2nd hong kong neurological congress 24th annual … committee of the 2nd hong kong neurological...

Hong Kong Med J Vol 17 No 6 # Supplement 5 # December 2011 1

2nd Hong Kong Neurological Congress cum24th Annual Scientific Meeting of The Hong Kong Neurological Society

Council of The Hong Kong Neurological Society 5

Organising Committee 5

List of Speakers 6

Scientific Programme 7

SESSION ABSTRACT PAGE

FREE PAPER PRESENTATIONS3D Rotational Angiography as a Screening Imaging Test FP 1 10for Intracranial StentingFlorence SY Fan

Intravenous Alteplase for Ischaemic Stroke Patients with FP 2 10Borderline Eligibility in Hong Kong—How Selective Should We Be? Alexander Lau, Edward Wong, Yannie Soo, Deyond Siu, Venus Hui, Edward Shum, Cecilia Leung, Colin Graham, Thomas Leung, Lawrence Wong

Subthalamic Deep Brain Stimulation for Advanced FP 3 11Parkinson’s Disease: Efficacy and ComplicationsMovement Disorder Group, Prince of Wales Hospital, The Chinese University of Hong Kong; Anne Chan, Jonas HM Yeung, Danny TM Chan, XL Zhu, Edith Wong, KY Lau, Rosanna Wong, Christine Lau, Vincent CT Mok, Wayne WS Poon

Guillain-Barré Syndrome: a Retrospective Study of Clinical FP 4 11Profiles and Outcomes in a Tertiary CentreAnna HY Wong, YF Cheung, WC Fong, KW Fong, HF Chan, YW Ng, LT Chan, YC Chan, WT Lo, M Ismail, HM Chan, P Li

Stroke in Tuberculous Meningitis FP 5 12YP Chu, MK Fong, B Sheng, YH Chan, WT Wong, KK Lau

DISSERTATION HIGHLIGHTSTransient Axonal Glycoprotein-1 (TAG-1) Polymorphism DH 1 13and Its Correlation with Clinical Features and Prognosis in Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)Shirley YY Pang

Volume 17 # Number 6 # DECEMBER 2011

S U P P L E M E N T 5

Editor-in-ChiefIgnatius TS Yu 余德新

Senior EditorsPT Cheung 張璧濤CB Chow 周鎮邦

Albert KK Chui 徐家強Michael G Irwin

EditorsKL Chan 陳廣亮KS Chan 陳健生

Henry LY Chan 陳力元David VK Chao 周偉強

TW Chiu 趙多和Stanley ST Choi 蔡兆堂

LW Chu 朱亮榮WK Hung 熊維嘉TL Kwan 關添樂

Alvin KH Kwok 郭坤豪Paul BS Lai 賴寶山Eric CH Lai 賴俊雄

Stephen TS Lam 林德深WY Lam 林永賢

Patrick CP Lau 劉志斌Arthur CW Lau 劉俊穎Nelson LS Lee 李禮舜

Danny WH Lee 李偉雄KY Leung 梁國賢

Danny TN Leung 梁子昂Thomas WH Leung 梁慧康

WK Leung 梁惠強Kenneth KW Li 李啟煌

David TL Liu 劉大立Janice YC Lo 羅懿之

Herbert HF Loong 龍浩鋒James KH Luk 陸嘉熙Ronald CW Ma 馬青雲

Ada TW Ma 馬天慧 Jacobus KF Ng 吳國夫

Hextan YS Ngan 顏婉嫦Martin W Pak 白威

PC Tam 談寶雛SW Tang 鄧兆華

William YM Tang 鄧旭明Clement CY Tham 譚智勇

Martin CS Wong 黃至生Kenneth KY Wong 黃格元

TW Wong 黃大偉Patrick CY Woo 胡釗逸

TK Yau 游子覺

Advisors on BiostatisticsWilliam B Goggins

Eddy KF Lam 林國輝

Advisor on Clinical Epidemiology Shelly LA Tse 謝立亞

2 Hong Kong Med J Vol 17 No 6 # Supplement 5 # December 2011

SESSION ABSTRACT PAGEUsefulness of ABCD2 Score in Prediction of 90-day Risk DH 2 14of Stroke in Patients with Transient Ischaemic Attack in a Chinese, Non-tertiary Care Community HospitalKK Wong

Clinical Profile and Prognosis of Patients with Myasthenia DH 3 15Gravis in Hong KongEric SW Yeung

Factors Associated with the Risk of Thymoma at the DH 4 16Presentation of Myasthenia GravisKY Cheung

Predictors of Early Neurological Deterioration During Acute DH 5 17Phase of Ischaemic Stroke: Experience in Acute Stroke Unit at a Regional Hospital in Hong KongGrace LY Cheung

SYMPOSIUM ON PERIPHERAL NEUROPATHYFocal Neuropathies S 1 17Windsor Mak

Guillain-Barré Syndrome: the Immunopathogenesis and S 2 18Relationship between the Different SubtypesNobuhiro Yuki

Common Pitfalls in the Electrodiagnosis of Entrapment S 3 18NeuropathyAlex CP Chow

SYMPOSIUM ON NEURO-OPHTHALMOLOGYIdiopathic Intracranial Hypertension S 4 19BL Man

Atypical Optic Neuritis S 5 19Carmen KM Chan, Andy CO Cheng

Using Eye Movements for Topological Diagnosis of Central S 6 20DisordersDavid S Zee

NOVARTIS SYMPOSIUM ON MOVEMENT DISORDERS JOINT SYMPOSIUM WITH THE HONG KONG MOVEMENT DISORDER SOCIETYNew Targets for Treatments of Parkinson’s Disease S 7 21Ken KL Yung, John MT Chu, Cathy NP Lui, Olivia TW Ng

Levodopa/carbidopa/entacapone as First-line Levodopa S 8 21Formulation: Pharmacokinetic ConsiderationsThomas Müller

Homocysteine and Entacapone Treatment S 10 22Thomas Müller

Deep Brain Stimulation for the Treatment of Parkinson’s S 11 22DiseaseEugene C Lai

INTERNATIONAL EDITORIAL ADVISORY BOARD

Sabaratnam Arulkumaran United Kingdom

Robert AtkinsAustralia

Peter CameronAustralia

James DickinsonCanada

Adrian DixonUnited Kingdom

Willard Fee, JrUnited States

Robert HoffmanUnited States

Sean HughesUnited Kingdom

Arthur KleinmanUnited States

Xiaoping LuoChina

Jonathan SametUnited States

Rainer SchmelzeisenGermany

David WeatherallUnited Kingdom

Homer YangCanada

EXECUTIVE EDITOR

Cyrus R Kumana

MANAGING EDITOR

Yvonne Kwok 郭佩賢

ASSISTANT MANAGING EDITORS

Warren Chan 陳俊華

Betty Lau 劉薇薇



ORIENT EUROPHARMA SYMPOSIUM ON EPILEPSY JOINT SYMPOSIUM WITH HONG KONG EPILEPSY SOCIETYPsychogenic Non-epileptic Seizures S 12 23David G Vossler

Upcoming Antiepileptic Drugs: the United States Experience S 13 23David G Vossler

GLAXOSMITHKLINE SYMPOSIUM ON EPILEPSY JOINT SYMPOSIUM WITH HONG KONG EPILEPSY SOCIETYSleep and Epilepsy S 14 24Carl Bazil

Psychiatric Issues in Epileptic Disorders S 15 25Joyce Lam

SYMPOSIUM ON NEUROGENETICSBasic Neurogenetics: What Clinical Neurologists Need to Know S 16 25KY Mok

Molecular Neurogenetics: Tools and Methodsh S 17 26Liz YP Yuen

Advanced Neurogenetics: Frontiers in Research S 18 26KY Mok

ALLERGAN SYMPOSIUM ON HEADACHE & PAINCurrent Approach for Chronic Migraine Management S 19 27SJ Wang

Updated Guidelines for Treatment of Neuropathic Pain S 20 28PP Chen

Trigeminal Autonomic Cephalalgias S 21 29TH Tsoi

BAYER LUNCH SYMPOSIUMAnticoagulation for Prevention of Stroke in Patients with S 22 30Atrial FibrillationJaseong Koo

SYMPOSIUM ON MOYAMOYA DISEASE JOINT SYMPOSIUM WITH THE HONG KONG STROKE SOCIETY Surgical Outcomes from Revascularisation Surgery for S 23 31Moyamoya Disease: Experience in Tuen Mun Hospital, Hong KongKF Fok, WL Poon, YC Wong, Florence Kwok, Dawson Fong

Surgical Management of Moyamoya Disease S 24 32KC Wang

SYMPOSIUM ON ISCHAEMIC STROKE JOINT SYMPOSIUM WITH THE HONG KONG STROKE SOCIETY Mechanical Thrombectomy in Ischaemic Stroke: a Look at S 25 33the ToolboxFerdinand Hui

Medical Management of Intracranial Stenosis S 26 34Kazunori Toyoda



POSTERS Off-label Use of Rituximab in Refractory Neurological P 1 35Autoimmune Diseases—Experiences of a Regional Teaching Hospital in Hong KongVincent HL Ip, Alexander YL Lau, Anne YY Chan, Lisa WC Au, Florence SY Fan, Nick NC Ng, Edward HC Wong, Yannie OY Soo, Vincent CT Mok, Thomas WH Leung, Lawrence KS Wong

Cisplatin Neuropathy: a Prospective Study on Chinese Patients P 2 35KF Ko, WY Lau, WK Cheng, MC Kwan, LK Yip, KP Yiu

A Lady with Autoimmune Lymphocytic Hypophysitis P 3 36Presenting with Right 3rd, 4th and 6th PalsiesSH Li

Long-term Outcome of Stroke Thrombolysis in Hong Kong P 4 37Chinese PatientsEdward Wong

Do Cerebral Microbleeds Increase Intracranial Haemorrhage P 5 37Risk in Hong Kong Stroke Patients Receiving Thrombolytic Therapy?Edward Wong, Alexander Lau, Yannie Soo, Deyond Siu, Jill Abrigo, Tom Cheung, Venus Hui, Lawrence Wong

Intravenous Thrombolysis Versus Intra-arterial Recanalisation P 6 38Therapy for Acute Ischaemic Stroke Due to Large Artery Occlusion—Comparison of Outcomes from a Hong Kong HospitalEdward Wong, Simon Yu, Alexander Lau, Joyce Hui, Deyond Siu, Colin Graham, Cecilia Leung, Venus Hui, Lawrence Wong, Thomas Leung

The Efficacy of Deep Brain Stimulation for Advanced P 7 38Parkinson’s DiseaseYF Cheung, HF Chan, TL Poon, PMP Choi, WS Chan, FC Cheung, HM Chan, P Li

A Case with Atypical Neuroleptic Malignant Syndrome P 8 39OC Lau, BH Fung, PW Ng, CH Lo, CK Chan, KY Cheung

Headache Associated with Chest Pain and ECG P 9 39Abnormalities: What Can It Be?Raymond CK Chan, CH Lo, PW Ng

AUTHOR INDEX 40


Council of The Hong Kong Neurological Society

President DrLeonardSheung-waiLi李常威醫生

Vice-President DrJonasHon-mingYeung楊漢明醫生

HonSecretary DrKwok-kwongLau劉國光醫生

HonTreasurer DrWing-chiFong方榮志醫生

CouncilMembers DrEricLok-yiuChan陳樂耀醫生

DrNelsonYuk-faiCheung張煜暉醫生

DrGardianChung-yanFong方頌恩醫生

DrSonnyFong-kwongHon韓方光醫生

DrKwok-faiHui許國輝醫生

DrThomasWai-hongLeung梁慧康醫生

DrColinHiu-tungLui呂曉東醫生

ProfVincentChung-tongMok莫仲棠教授

DrBunSheng盛斌醫生

DrKin-lunTsang曾建倫醫生

DrWinnieWing-yinWong黃詠妍醫生

AdHocMember DrTak-hongTsoi蔡德康醫生

PastPresident DrPing-wingNg吳炳榮醫生

HonLegalAdvisor MrTsang-hoiKoo顧增海律師

HonAuditor MrEricLi李家祥先生

Organising Committee of the 2nd Hong Kong Neurological Congress cum 24th Annual

Scientific Meeting of The Hong Kong Neurological Society

Chairmen DrJonasHon-mingYeung楊漢明醫生

DrLeonardSheung-waiLi李常威醫生(Co-Chair)

ScientificCommittee DrWing-chiFong方榮志醫生

DrGardianChung-yanFong方頌恩醫生

DrSonnyFong-kwongHon韓方光醫生

DrKwok-faiHui許國輝醫生

DrThomasWai-hongLeung梁慧康醫生

DrColinHiu-tungLui呂曉東醫生

ProfVincentChung-tongMok莫仲棠教授

PublicationCommittee DrWinnieWing-yinWong黃詠妍醫生

DrEricLok-yiuChan陳樂耀醫生

Website DrNelsonYuk-faiCheung張煜暉醫生

DrKwok-kwongLau劉國光醫生

DrBunSheng盛斌醫生

DrKin-lunTsang曾建倫醫生


List of Speakers

Name AffiliationProfCarlBazil CollegeofPhysiciansandSurgeonsatColumbia

University,UnitedStatesDrCarmenKar-munChan HongKongEyeHospital,HongKongSARDrPhoon-pingChen AliceHoMiuLingNethersoleHospital/NorthDistrict

Hospital,HongKongSARDrAndyChi-onCheng HongKongEyeHospital,HongKongSARDrAlexChi-pingChow HongKongSanatorium&Hospital,HongKongSARDrKam-fukFok TuenMunHospital,HongKongSARDrFerdinandHui ClevelandClinicFoundation,UnitedStatesProfJaseongKoo CatholicUniversityofKorea,KoreaProfEugeneCLai TheMethodistNeurologicalInstitute,Houston,Texas,

UnitedStatesDrJoyceLam TheChineseUniversityofHongKong,HongKongSARDrWindsorMak QueenMaryHospital,HongKongSARDrBik-lingMan TuenMunHospital,HongKongSARDrKin-yingMok UCLInstituteofNeurology,UnitedKingdomProfThomasMüller StJosephHospitalBerlin-Weissensee,GermanyDrKazunoriToyoda NationalCerebralandCardiovascularCenter,Osaka,

JapanDrTak-hongTsoi PamelaYoudeNethersoleEasternHospital,HongKong

SARProfDavidGVossler UniversityofWashington,Seattle,UnitedStatesProfKyu-changWang SeoulNationalUniversityHospital,KoreaProfShuu-jiunWang NationalYang-MingUniversitySchoolofMedicine,

TaiwanProfRuey-meeiRobinWu NationalTaiwanUniversity,TaiwanDrLizYuet-pingYuen TheChineseUniversityofHongKong,HongKongSARProfNobuhiroYuki NationalUniversityofSingapore,SingaporeProfKenKLYung HongKongBaptistUniversity,HongKongSARDrDavidSZee JohnsHopkinsSchoolofMedicine,UnitedStates


SCIENTIFIC PROGRAMME

Venue: Grand Ballroom, leVel ll1, Kowloon ShanGri-la hotel

28 octoBer 2011, Friday

09:00 – 09:30 Registration FunctionRoom

09:30 – 11:00 FREE PAPER PRESENTATIONChairpersons: Thomas Leung, Winnie Wong

POSTER PRESENTATION

11:00 – 11:20 CoffeeBreak11:20 – 12:30 DISSERTATION HIGHLIGHTS

Chairpersons: Thomas Leung, Winnie Wong

12:30 – 13:30 Lunch13:30 – 15:00 SYMPOSIUM ON PERIPHERAL NEUROPATHY

Chairpersons: Bun Sheng, Leonard Li

Focal NeuropathiesWindsor Mak

Guillain-Barré Syndrome: the Immunopathogenesis and Relationship between the Different Subtypes

Nobuhiro Yuki

Common Pitfalls in the Electrodiagnosis of Entrapment Neuropathy

Alex CP Chow15:00 – 15:20 CoffeeBreak15:20 – 16:45 SYMPOSIUM ON NEURO-OPHTHALMOLOGY

Chairpersons: SH Ng, Nelson YF Cheung

Idiopathic Intracranial HypertensionBL Man

Atypical Optic NeuritisCarmen KM Chan, Andy CO Cheng

Using Eye Movements for Topological Diagnosis of Central Disorders

David S Zee



09:30 – 09:40 OPENING CEREMONYWelcome Remarks: Dr Leonard SW Li

President of the Hong Kong Neurological SocietyGuest of Honour: Mr Richard MF Yuen, JP

Permanent Secretary for Food and Health (Health)

POSTER PRESENTATION

09:40 – 10:45 NOVARTIS SYMPOSIUM ON MOVEMENT DISORDERSJOINT SYMPOSIUM WITH THE HONG KONG

MOVEMENT DISORDER SOCIETYChairpersons: Mandy Au Yeung, KL Tsang

New Targets for Treatments of Parkinson’s DiseaseKen KL Yung

Levodopa/carbidopa/entacapone as First-line Levodopa Formulation: Pharmacokinetic Considerations

Thomas Müller10:45 – 11:00 CoffeeBreak11:00 – 12:35 NOVARTIS SYMPOSIUM ON MOVEMENT DISORDERS

JOINT SYMPOSIUM WITH THE HONG KONG MOVEMENT DISORDER SOCIETY

Chairpersons: Vincent Mok, XL Zhu

Routine Genetic Screening for Young Onset / Familial Parkinson’s Disease in Chinese—Are We Ready?

Ruey-Meei Robin Wu

Homocysteine and Entacapone TreatmentThomas Müller

Deep Brain Stimulation for the Treatment of Parkinson’s Disease

Eugene C Lai12:35 – 13:30 Lunch13:30 – 15:00 ORIENT EUROPHARMA SYMPOSIUM ON EPILEPSY

JOINT SYMPOSIUM WITH HONG KONG EPILEPSY SOCIETY

Chairpersons: Colin Lui, Ada Yung

Psychogenic Non-epileptic SeizuresDavid G Vossler

Upcoming Antiepileptic Drugs: the United States Experience

David G Vossler15:00 – 15:20 CoffeeBreak15:20 – 17:00 GLAXOSMITHKLINE SYMPOSIUM ON EPILEPSY

JOINT SYMPOSIUM WITH HONG KONG EPILEPSY SOCIETY

Chairpersons: Eric Chan, Howan Leung

Sleep and EpilepsyCarl Bazil

Psychiatric Issues in Epileptic Disorders Joyce Lam

29 octoBer 2011, Saturday



09:30 – 11:05 SYMPOSIUM ON NEUROGENETICSChairpersons: Jonas Yeung, YL Yu

Basic Neurogenetics: What Clinical Neurologists Need to Know

KY Mok

Molecular Neurogenetics: Tools and MethodsLiz YP Yuen

Advanced Neurogenetics: Frontiers in ResearchKY Mok

POSTER PRESENTATION

11:05 – 11:20 CoffeeBreak11:20 – 12:35 ALLERGAN SYMPOSIUM ON HEADACHE & PAIN

Chairpersons: YW Chan, Edmund Woo

Current Approach for Chronic Migraine ManagementSJ Wang

Updated Guidelines for Treatment of Neuropathic PainPP Chen

Trigeminal Autonomic Cephalalgias TH Tsoi

12:35 – 14:00 BAYER LUNCH SYMPOSIUMChairpersons: Patrick Li, PW Ng

Anticoagulation for Prevention of Stroke in Patients with Atrial Fibrillation

Jaseong Koo14:00 – 15:05 SYMPOSIUM ON MOYAMOYA DISEASE

JOINT SYMPOSIUM WITH THE HONG KONG STROKE SOCIETY

Chairpersons: Dawson Fong, WC Fong

Surgical Outcomes from Revascularisation Surgery for Moyamoya Disease: Experience in Tuen Mun Hospital, Hong Kong

KF Fok

Surgical Management of Moyamoya Diseases KC Wang

15:05 – 15:20 CoffeeBreak15:20 – 17:00 SYMPOSIUM ON ISCHAEMIC STROKE

JOINT SYMPOSIUM WITH THE HONG KONG STROKE SOCIETY

Chairpersons: Raymand Lee, Sonny Hon

Mechanical Thrombectomy in Ischemic Stroke: a Look at the Toolbox

Ferdinand Hui

Medical Management of Intracranial Stenosis Kazunori Toyoda

17:00 ClosingRemarksandPresentationofAwardstoBestFreePaper,BestPosterandBestDissertationPresentations

30 octoBer 2011, Sunday


FP 13D Rotational Angiography as a Screening Imaging Test for Intracranial Stenting

Florence SY FanDepartment of Medicine and Therapeutics, Prince of Wales Hospital, Hong Kong SAR

Background:3Drotationalangiography(3DRA)isanewtechniqueindelineatingfinedetailsofintracranialvasculature.Weinvestigatedtheroleof3DRAinselectingpatients for intracranialself-expandablestenting.Methods:Patientswhohadstrokesreferrabletoahigh-gradesymptomaticstenosiswererecruitedandunderwentscreeningforintracranialstentingbyaconventionaldigitalsubtractionangiography(DSA)anda3DRA.Bothimagingtestswereperformedbyabiplaneangiographicequipment(AlluraXperFD20/20,PhilipsMedicalSystems,Netherland).DSAconsistedofanteroposterior, lateralandbilateralobliqueviewsofthetargetarterywith10mLofiopamiro300ineachinjectionfroma4FH1catheterpositionedatinternalcarotidarteryorvertebralarteryostium.Bythesamecatheterandcontrast,3DRAwasobtainedbyarotatingarmcentredatthetargetlesion,comprising120imagesgatheredoverascantimeof4seconds.Aradiologistblindtothepatient’sclinicalinformationcomparedtheanatomyofthelesionanditsimmediatevicinityasrevealedbythetwoimagingmethods.ThedegreeofstenosiswasmeasuredbyWASIDtechnique.Lesioncharacteristicswhichmightalterthedecisionofendovasculartreatmentwererecorded.Results:PairedDSAand3DRAwereobtainedin169patients.Thetwomethodswerecomparable inthemeasurementofthestenosisseverity.However,amongthefourpatientswhohadfenestrationsmimickingoradjacenttothestenosis(middlecerebralartery=3;basilararterty=1), threewereonlyevidentin3DRAbutnotinconventionDSA.Inanotherthreepatients,angioplasty/stentingwasprecludedbasedon3DRAwhichshowedanadjacentperforatororbranchwithahighlystenosedostium.Conclusions:3DRAmaycompare favourablywithconventionalDSA indepictingminutedetailsofintracranialvascularanatomy,and ispotentiallyuseful inpreoperativeevaluationof intracranialangioplasty.

FP 2Intravenous Alteplase for Ischaemic Stroke Patients with Borderline Eligibility in Hong Kong—How Selective Should We Be?

Alexander Lau1, Edward Wong1, Yannie Soo1, Deyond Siu2, Venus Hui1, Edward Shum1, Cecilia Leung1, Colin Graham3, Thomas Leung1, Lawrence Wong1

1 Department of Medicine and Therapeutics, Prince of Wales Hospital, Hong Kong SAR2 Department of Imaging and Interventional Radiology, Prince of Wales Hospital, Hong Kong SAR3 Department of Accident and Emergency Medicine, Prince of Wales Hospital, Hong Kong SAR

Background:Varianceexistsinselectingpatientsforstrokethrombolysis.UnlikeUSlabelling,intravenousalteplase(IVTPA)isnotrecommendedforage>80yearsandNIHSS>25byEuropeanguideline.Besides,bothexcludepatients>80yearsorwithahistoryofdiabetesandstrokeforthe3-4.5hourswindow.WecomparedtheoutcomesofpatientswhoreceivedIVTPAoutsidetheseguidelinestothosewhocomplied,andtoacontrolgroup.Methods:ConsecutivepatientsreceivedIVTPA<4.5hoursbetweenJanuary2007andJune2011atourcentrewerereviewed.Patientscomplianttobothguidelineswerecomparedtothosewhodidnot.TheywerefurthercomparedtoacontrolgroupwiththesameborderlineeligibilitybutwerenotgivenIVTPAduetonon-officehoursadmissionbetweenOctober2008andMay2011.Outcomemeasureswere3-monthmodifiedRankinScale(mRS)≤2andmortality,andsymptomaticintracranialhaemorrhage(SICH)accordingtoECASS(EuropeanCooperativeAcuteStrokeStudy).Results:Fifty-fourpatientsreceivedIVTPA.Thirty-threewerecomplianttobothguidelinesand21werenon-compliant.Fornon-compliantgroup(meanage81.6±8.2,NIHSS18.6±8.2),16were>80years,5hadNIHSS>25,and2thrombolysedbetween3-4.5hourshadahistoryofbothdiabetesandstrokeor>80years.Forcomparison,22non-thrombolysedcontrols(meanage77.0±10.9,NIHSS19.1±10.1)wereidentified.Twenty(61%)ofthecompliantgroupversus4(19%)ofthenon-compliantandnoneofcontrolgroupsattained3-monthmRS≤2(non-compliantvscontrol,P=0.05).Three-monthmortalityrateswere3%,43%and27%forcompliant,non-compliant,andcontrolgroupsrespectively(non-compliantvscontrol,P=0.35).SICHoccurredinone,two,andonepatientsofcompliant,non-compliantandcontrolgroups,respectively.Conclusions:TheoutcomesofpatientsgivenIVTPAoutsidebothUSandEuropeanguidelineshadpooreroutcomescomparedtothosewhofulfilled.Still,thosewhowerenotgivenIVTPAfaredevenpoorer.FurtherprospectiverandomisedtrialsareneededtosubstantiatetheuseofIVTPAinborderlineeligiblepatients.


Subthalamic Deep Brain Stimulation for Advanced Parkinson’s Disease: Efficacy and Complications

Movement Disorder Group, Prince of Wales Hospital, The Chinese University of Hong Kong; Anne Chan1, Jonas HM Yeung1, Danny TM Chan2, XL Zhu2, Edith Wong2, KY Lau2, Rosanna Wong3, Christine Lau1, Vincent CT Mok1, Wayne WS Poon2

1 Division of Neurology, Department of Medicine and Therapeutics, Prince of Wales Hospital, The Chinese University of Hong Kong, Hong Kong SAR2 Division of Neurosurgery, Department of Surgery, Prince of Wales Hospital, The Chinese University of Hong Kong, Hong Kong SAR3 Department of Occupational Therapy, Prince of Wales Hospital, The Chinese University of Hong Kong, Hong Kong SAR

Background:Deepbrainstimulation(DBS)ofsubthalamicnucleus(STN)wasfirstperformedinHongKongsince1998atourcentreforpatientswithadvancedParkinson’sdisease(PD).WeherebyreporttheefficacyandcomplicationsofSTNDBSforadvancedPD.Methods:AllPDpatientswhoreceivedSTNDBSfromSeptember1998toJanuary2010wereincludedinthepresentanalyses.WecomparedtheUnifiedParkinson’sDiseaseRatingScale(UPDRS)beforeandatleast2monthsaftertheoperation.Peri-operativecomplicationswerenoted.Results:Atotalof41PDpatients(meanage,54years;21males)receivedbilateralSTNDBS.Themedianfollow-updurationwas12months(range,2-77months).Therewasasignificantoverallimprovement(31%;P<0.0001)inUPDRSinpartIII(motorperformance)postoperativelyat“onstimulationandoffmedicationcondition”comparedwithpreoperativelyat“offmedicationcondition”.Therewasalsoasignificantimprovement(32%,P<0.0001) inUPDRSpartII (functional level)score.Peri-operativecomplicationsincludedleadfracture(n=1),andleadmigration(n=1).Therewasnoperioperativemortality.Conclusions:Ourefficacyandsafetyresultswerecomparabletooverseascentres.Overall,STNDBSisaneffectiveandsafetreatmentforadvancedPD.

FP 3

FP 4Guillain-Barré Syndrome: a Retrospective Study of Clinical Profiles and Outcomes in a Tertiary Centre

Anna HY Wong, YF Cheung, WC Fong, KW Fong, HF Chan, YW Ng, LT Chan, YC Chan, WT Lo, M Ismail, HM Chan, P LiDepartment of Medicine, Queen Elizabeth Hospital, Hong Kong SAR

Background:Guillain-BarréSyndrome(GBS)isanacuteperipheralneuropathywhichispotentiallyfatalanddebilitating.Effectivetreatmentisavailable.Localdataarescarce.Inthisstudy,wereviewedtheclinicalprofilesandoutcomesofGBSmanagedinatertiarycentreinHongKong.Methods:AretrospectivestudywasperformedonGBSpatientswhomettheAsburyandCornblath(1990)diagnosticcriteriaandwereadmittedbetweenJuly2001andDecember2010totheDepartmentofMedicine,QueenElizabethHospital.DatawereanalysedbyPASWStatistics18software.Results:Wefound65GBSpatientswithinthestudyperiod;44(68%)weremale.Themeanageofonsetwas61(standarddeviation,15.7)years;94%wereChinese.Thenumberofpatientsdiagnosedperyearrangedfrom3to13.Symptomssuggestiveofantecedentupperrespiratorytractinfections(48%)andthegastrointestrinaltractinfections(11%)wererecordedrespectively.Twelvepercentofpatientshadahistoryofrecentvaccinationinthepast1to3weeks.Presentingfeaturesincludedlimbweakness(94%),facialweakness(10%),numbness(63%),visualsymptoms(14%),bulbarsymptoms(24%),hyporeflexia/areflexia(78%),ataxia(22%)anddysautonomia(27%).SimilartoCaucasianseries,acuteinflammatorydemyelinatingpolyradiculoneuropathy(AIDP)wasthecommonestsubtype(57%),followedbyaxonalsubtypes(AMANorAMSAN,21%),Fisher’ssyndrome(12%)andBickerstaffbrainstemencephalitis(2%).Aroundone-thirdreceivedintensivecareunit(ICU)careandone-fourthhadrespiratoryfailurerequiringmechanicalventilation.Seventeenpercentofpatientshadtemporarytracheostomyperformed.Fortheseselectedpopulations,themeanlengthofICUstaywas14days(95%confidenceinterval[CI],8.5-19.6)andthemeanlengthofmechanicalventilationwas25.5days(95%CI,14.4-36.7).At6months,84%ofpatientssurvivedand66%remainedindependentinactivitiesofdailyliving(ADL),asmeasuredbytheGlasgowOutcomeScale.Usingamultivariate logisticregressionmodelwithage,sex,antecedent infection/vaccination,bulbarpresentationanddiseasesubtypeascovariates,agewastheonlyprognosticfactorthatsignificantlydeterminedthe6-monthindependencyinADLbutnotsurvival.Conclusions:Inourcohort,GBScarriedsignificantmorbidityandmortality.Theonlysignificantpredictorwasage inourmodel.Larger,multicentrestudiesarewarranted forbetterdelineationofclinicalpredictorsofadverseoutcomes.


Stroke in Tuberculous Meningitis

YP Chu, MK Fong, B Sheng, YH Chan, WT Wong, KK LauDepartment of Medicine, Princess Margaret Hospital, Hong Kong SAR

Background:Theobjectiveof this studywas to identify thepredictorsof stroke inpatientswithtuberculousmeningitis(TBM)andtheirprognosticimplications.Methods:AllpatientswithTBMinPrincessMargaretHospitalfrom2000to2010wereincludedinthestudy.Ofthe34patientswithTBM,16patientsdevelopedstroke.Potentialpredictorsofstrokeincludingco-morbiditiesandbiochemicalfindingswerestudied.Radiologicalexaminationsincludingcomputedtomographyormagnetic resonance imagingbrainwerealsostudied.TheneurologicaloutcomemeasuredwasmortalityrateandModifiedRankinScoreat6months(0-3vs4-6).Results:Patientswithstrokewereolder(55.4±15.2vs45.9±16.6;P=0.09)andhadlowercerebrospinalfluid(CSF)–to–serumglucoseratio(0.24±0.14vs0.33±0.14;P=0.09).TherewerelessHIVpatientsinthosewithstroke(6.3%vs22.2%;P=0.19).MostoftheinfarctsinTBMweremultiple(87.5%),bilateral(62.5%),andlocatedinbasalganglion(31.3%)andsubcorticalwhitematter(50%).Patientswithstrokehadahighermortalityrate(31.2%vs11.15%;oddsratio[OR]=3.64;P=0.147)andmoreadverseneurologicaloutcomeat6months(56.2%vs29.4%;OR=3.09;P=0.119).HighCSFtotalprotein(2.59±1.3vs1.57±0.53;P=0.0096)andolderage(64±13.2vs44.4±14.5;P=0.014)wereassociatedwithadverseneurologicaloutcomeat6monthsinTBMpatientswithstroke.Conclusion:StrokeiscommoninpatientswithTBMleadingtoahighermortalityandworseneurologicaloutcome.

FP 5


DH 1Transient Axonal Glycoprotein-1 (TAG-1) Polymorphism and Its Correlation with Clinical Features and Prognosis in Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)

Shirley YY PangDepartment of Medicine, Queen Mary Hospital, Hong Kong SAR

Background:Chronicinflammatorydemyelinatingpolyradiculopathy(CIDP)isaheterogeneousgroupofacquireddisorderscharacterisedbyperipheralnervedemyelination.Previousstudieshaveshownagoodresponse to immunotherapybutasignificantproportionofpatientscontinue to requiremaintenance therapy.A recentstudy identifiedapotentialgeneticmarker for responsiveness tointravenousimmunoglobulin(IVIG).Inthisstudy,CIDPpatientswereexaminedtolookforassociationbetweenclinical,electrophysiologic,geneticfactorsandtreatmentresponseaswellasriskoftreatmentdependence.Methods:Case recordsof32CIDPChinesepatientsdiagnosedbetween January1995andMarch2010at the threeNeurologycentresonHongKongIsland(namely,QueenMaryHospital,PamelaYoudeNethersoleEasternHospital,andRuttonjeeHospital)werereviewedtoexaminetheirclinicalfeatures,electrophysiologicparametersonpresentation,diseasecourseandoutcome.Bloodsampleswereavailablefrom22patientsand147controls.DNAwasextractedfromperipheral leucocytesandthetransientaxonalglycoprotein1 (TAG-1)genotypefor thepreviouslyreportedsinglenucleotidepolymorphism(SNP)wasdeterminedusingtheSequenomMassARRAYsystem.Results:Theoverallresponseratetoimmunotherapywithprednisolone,IVIGorplasmaexchange(PE)was80%withnodifferencedetectedamongthethreetherapies.Clinicalfeaturessuchasageofonset,durationofsymptoms,presenceofdiabetesmellitus(DM),presenceofsensorysymptomsandmodifiedRankinscore (mRS)onpresentationdidnotpredict treatmentresponsiveness.Electrophysiologicparametersontheinitialnerveconductionstudywerealsonotassociatedwithtreatmentresponse.Fifty-eightpercentofourpatientsweredependentonmaintenancetherapyafterameanfollow-upperiodof5.8years.Clinicalfeaturessuchasageofonset,durationofsymptomsandpresenceofDMwerenotpredictiveoftreatmentdependence.Patientswithmoreprolongeddistalmotorlatency(DML)intheupperlimbshadahigherriskoftreatmentdependence(P=0.03,OR=1.03).Patientswithahighermeanmotornerveconductionvelocity(NCV)intheupperlimbsshowedatrendoflowerriskoftreatmentdependencebutthisdidnotreachstatisticalsignificance.TAG-1genotypesin147healthycontrolsandin22CIDPpatientswithregardtotheSNPdesignatedbyrs2275697weredeterminedandnosignificantdifferenceinallelefrequencywasdetected.Furthermore,amongCIDPpatients,therewasnoassociationbetweenTAG-1genotypeandageofonset,presenceofDM,modeofonsetanddiseasecourse.However,thosewhowerehomozygousforGhadsignificantlymoreprolongedDML.ThosewiththeGallelepresent(patientswhowereeitherhomozygousforGorheterozygouswithG/A)hadsignificantlylowercompoundmuscleactionpotential(CMAP)amplitudeintheupperlimbsandmoreprolongedDMLinthelowerlimbsthanthosehomozygousforA.Conclusions:TheoveralltreatmentresponserateinCIDPishighandevenpatientswithadvancedage,unfavourableelectrophysiologicfeaturesandseveredisabilitycanimprovewithtreatment.However,asignificantproportionwillrequirelong-termmaintenancetherapytopreventrelapses.PatientswhoseinitialnerveconductionstudyshowedmoreprolongedDMLintheupper limbswereat increasedriskofbeingtreatmentdependent.TheTAG-1GallelewasfoundtobeassociatedwiththeseverityofdemyelinationontheinitialnerveconductionstudyinCIDPpatients,whichsuggestsaroleofTAG-1inthepathogenesisofdemyelination.


Usefulness of ABCD2 Score in Prediction of 90-day Risk of Stroke in Patients with Transient Ischaemic Attack in a Chinese, Non-tertiary Care Community Hospital

KK WongDepartment of Medicine and Geriatrics, Yan Chai Hospital, Hong Kong SAR

Background:Strokewasthesecondcauseofmortalityworldwidein2004andrankedthefourthcauseofdeath inHongKongin2008. Itwasfrequentlyprecededbytransient ischaemicattack(TIA).TIAisrecognisedtobeamedicalemergencybecause itsrisksofearlystrokearehigh.VariousclinicalpredictiontoolsareproposedtoaidtheriskstratificationafterTIA.The“ABCD2”scoresareattemptedtoquantifythisrisk.Thosewiththehighestscoreshavesignificantlyincreasedriskofearlystroke.Thesescoreshadbeenvalidatedinseveralstudiesbutwerenotuniversallysuccessfulindifferentpopulations.Aim:ThepurposeofthestudywastoevaluatetheABCD2scoreinpatientswithTIAandtheriskofsubsequentstrokeinalocalChinese,non-tertiarycommunityhospital.Methods:From1January2004to31December2009,allpatients’recordswithprincipaldiagnosisas‘transient ischaemicattack’(TIA)codedbyICD9hadbeenretrieved.IncidenceofstrokeamongTIApatientshadbeenidentifiedat90days.Allthebaselineparameterswererecorded.IncidenceofstrokeamongTIApatientshadbeenidentifiedat90days.ABCD2scoreswerecalculatedretrospectively.Fromthedifferentialstrokeincidence,thepatientswithTIAhavebeendividedintotwogroupswithdifferentABCD2scoresforfurtherstatisticalcomputation.Primaryoutcomewasstrokewithin90daysafterTIA.SecondaryoutcomesarecardiovasculardiseasesincludingrecurrentTIAoracutestroke,cardiovasculardiseaseanddeath.Results:Therewereatotalof446patientswithdiagnosisofTIArecruited.Ofthem,23(5.4%)hadbeenrejected.Thetotal90-dayincidenceofstrokeinrecruitedpatientswas5.2%(22patients).Morethanhalfofthestrokerecurrenceoccurredwithin7days(4patients,18%)and30days(9patients,41%)whereastheremaining(9patients,41%)wereat90days.PatientswerecategorisedintotwogroupswithABCD2scoreat5.Therewassignificantdifferencein90-daystrokeincidencebetweenhigh(16patients,72.7%)andlow(6patients,27.3%)ABCD2score(P=0.047).Oddsratiowas2.817.TheKaplan-Meiersurvivalcurvefor90-daystrokeofdichotomisedABCD2scoreat5wasplotted.Pvalueoflog-ranktestwas0.0295.Conclusion:OurstudyhasconfirmedtheusefulnessofABCD2scoreinpredictingthe90-daystrokeriskinpatientswithTIAinaChinese,non-tertiarylocalcommunityhospital.ThosewithhighABCD2scorewouldbeassociatedwithhigher90-daystrokeincidence.MoreresourcesshouldbeallocatedtothosewithhigherABCD2scoreinTIApatients.

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DH 3Clinical Profile and Prognosis of Patients with Myasthenia Gravis in Hong Kong

Eric SW YeungDepartment of Medicine, Pamela Youde Nethersole Eastern Hospital, Hong Kong SAR

Background:Myastheniagravis(MG)isthecommonestneuromuscularjunctiondisorder.Previouslocalstudiesreportedanannualincidenceof4permillion,whilelatestwesternfiguresaremuchhigher.Withadvancesintreatment,theprognosisofMGpatientshasmuchimprovedinwesterncountriesoverthepastdecades.Howeverthereisascarcityoflocaldata.Objectives:Ourstudyhas fourobjectives: (1) tostudydemographicsandclinicalcharacteristics inpatientswithMG;(2)toexaminetheutilisationofvariousdiagnosticmodalitiesforMG;(3)toreviewourexperienceinmanagementofthedisease;and(4)todeterminetheclinicaloutcomesandprognosisofMGinChinesepatients.Method:This isaretrospectivereviewofpatientswithMGattendingourNeurologyunitofPamelaYoudeNethersoleEasternHospitalbetween1October1993and31December2010.Patientswereidentifiedbytheclinicaldatasystem(CDARS)withthediagnosisofMG,anddividedintothreegroups(ocular,generalised,oculartogeneralised)forcomparison.Demographics,clinicalprofile,historyofhospitalisationandintensivecareunitcare,myasthenic/cholinergiccrisis,investigationresults,treatmentmodalities,outcomeatthetimeofanalysisincludingfunctionalstatusandmortalitywereretrievedfromtheclinicalmanagementsystem(CMS)andhospitalchartsforreviewandanalysis.Factorsassociatedwithmortality,generalisationinocularonsetpatientswerestudied.Results:Atotalof152patientswereidentified.Twopatientswerelosttofollow-upandexcluded.Themeanageatdiagnosiswas51.18(range,13-91)years.Afemalepredominancewasnotedinallage-groups.Theannualincidencewas13.3permillion(range,8.3-23.3).OcularMGcomprisedthemajority(59.3%).Fifteenpatientshadocularonsetanddelayedgeneralisationofsymptoms,alloccurringwithin30(range,1-30)months.Significantassociatedfactorswithgeneralisationincludedpositiveanti-acetylcholinereceptor(anti-AChR)antibody,positiverepetitivestimulationtest(RST),andpresenceofpathologicallyconfirmedthymoma.Theanti-AChRantibodywasthecommonesttestperformed(92.7%)whileTensilontesthadthehighestpositiverate(75%).25.5%ofpatientshadthymomaoncomputedtomography(CT)scanofthorax.91.3%ofpatientswereonmestinon.40.7%wereonsteroids.39patientsrequiredin-patientmanagementoftheirMGexacerbation, leadingtoameanhospitalstayof7.95(range,2-125days).22patientshadmyastheniccrises,alloccurringbeforetreatmentwithstandardimmunotherapy.Thymectomywasdonein23patients,with19pathologicallyconfirmedthymomas.FourpatientsdiedofMG-relatedcomplications:threefromrespiratoryfailureandpneumonia,onefromseveresepsiswhileoncorticosteroid.Thedisease-relatedmortalitywas2.7%overameandiseasedurationof9.65(range,1-51years).Noriskfactorassociationwithmortalitywasidentified.Allsurvivingpatientsachievedclinicalremission,withmodifiedRankinScaleof0or1.Conclusion:MGwascommoninourlocality,withanannualincidencecomparabletowesternstudies.Noneoftheinvestigationmodalitieswassatisfactorytoserveasthe“goldenstandard”fordiagnosis.Utilisationofcurrentpharmacologicaloptionswasadequate,butthymectomywas lessconsidered.Prognosisisgoodinourgroup,comparabletowesterncountriesandpreviouslocalexperience.


Factors Associated with the Risk of Thymoma at the Presentation of Myasthenia Gravis

KY CheungDepartment of Medicine, Tuen Mun Hospital, Hong Kong SAR

Background:Thymoma is found in10 to15%ofpatientswithmyastheniagravis (MG).Thymoma-associatedMGisconsideredtobeamoreseverediseaseandallthymomapatientsareindicatedforthymectomy.ItisunclearwhethercertainclinicalorserologicalfindingsofMGpatientsatthetimeorearlyafterthediagnosiscanpredictthepresenceofthymoma.Objective:Todeterminethedemographic,clinicalaswellasautoimmuneantibodycharacteristicsthatmaydifferentiatebetweenthymoma-associatedMGandnon-thymoma–associatedMGintheearlycourseofthedisease.Comparisonfortheclinicalcoursesduringthewholefollow-upperiodofMGpatientswithorwithoutthymomawasalsomade.Methods:WeretrospectivelyevaluatedMGpatientsfollowedupatTuenMunHospital inHongKongfromyear2000to2010.MGwasdiagnosedbythetypicalhistoryandsignsoffluctuatingandfatigablemuscleweaknesswithitsassociationswiththefollowingvariables:thetitreofanti-acetylcholinereceptorantibody,anunequivocalclinical improvement inresponsetoanticholinesterase inhibitors,andadecrementalpatternonrepetitivenervestimulationtestandthestatusofthymomaonimagingstudy.Thebaselinecharacteristics,clinicalpresentation,coursesofdiseaseduringtheearlyandthewholefollow-upperiods,treatmentsoffered,anti-acetylcholineautoantibodytitersaswellastheotherassociatedautoimmunediseaseswererecordedandcomparedbetweenthegroupswithorwithoutthymoma.Results:Atotalof184MGpatientswhofulfilledthecriteriawereidentifiedandfollowedup.Thymomawasdiagnosedin19.6%ofthem.Themeanageofpatientswiththymomawasolder(55.08vs44.56;P=0.042);femalesexwasmoreprevalent(femalevsmaleratioof1.4vs1)butthesexratiowassimilarwhencomparedwiththosewithoutthymoma.AlthoughthepresentingsymptomsandbaselineMGFAscoresweresimilar,thymoma-associatedpatientshadasignificantlymorerapiddeteriorationofMGandmoreseverediseasewithhighermeanofmaximumMGFAscore(2.81vs1.26;P<0.001)withinthefirst6monthsoffollow-up,andwerealsomorelikelytodevelopgeneralisedMGeventually(83.3%vs38.5%;P<0.001)whencomparedwithnon-thymomagroup.Allthymomapatientshadpositiveanti-acetylcholinereceptorantibodyandahighertitrewasrecorded.Theirlong-termoutcomewascomparabletonon-thymoma–associatedMGpatientswithsimilarMGFAscoreon last follow-up.Autoimmunediseasewaspresentin37%ofpatientsoverallwiththyroiddiseasebeingthecommonestassociation.Usingmultivariatelogisticregressionmodel,ahighMGFAscoreat6months(MGFA3-5)andaveryhighanti-AChRtitre(>19nmol/L)werepredictiveofthymoma.Conclusion:MGpatientsatriskofthymomacouldbeidentifiedduringtheearlycourseofthedisease.AhighMGFAscoreat6monthsandaveryhighanti-AChRtitrepredictedtheoccurrenceofthymoma.

DH 4


DH 5Predictors of Early Neurological Deterioration During Acute Phase of Ischaemic Stroke: Experience in Acute Stroke Unit at a Regional Hospital in Hong Kong

Grace LY CheungDepartment of Medicine and Geriatrics, Tuen Mun Hospital, Hong Kong SAR

Background:Patientssufferingfromacuteischaemicstrokeareatriskofneurologicaldeteriorationwithinthefirstfewdaysafterstrokeonset.Thefrequency,clinicalcharacteristicsandcontributingfactorsofearlyneurologicaldeteriorationduringtheacutephaseofischaemicstrokeinalocalhospitalinHongKongwereinvestigated.Methods:ConsecutiveacuteischaemicstrokepatientsadmittedtotheAcuteStrokeUnitatTuenMunHospital,amajor regionalhospital located in theNewTerritoriesWest,HongKongwerestudiedprospectively.Demographicalcharacteristics,clinicalparameters,computedtomographic(CT)brainfindingsandlaboratoryresultswereevaluated.TheNationalInstituteofHealthStrokeScale(NIHSS)wasusedtoassessstrokeseverityandneurologicaldeficits.AssessmentsusingNIHSSwereperformedatbaselineandafteradmission.EarlyneurologicaldeteriorationwasdefinedasanincreaseinNIHSSscoreby4ormorepointsfrombaselinewithin7daysfromstrokeonset.Results:Atotalof110acuteischaemicstrokepatientswererecruited,ofwhom27(24.5%)sufferedfromearlyneurologicaldeterioration.ThepredictivefactorsofearlyneurologicaldeteriorationincludedahighbaselineNIHSSscore(P<0.001), lowGlasgowComaScalescoreonadmission(P<0.001),andbaselineCTbrainfindingsofinfarctsoncoronaradiata(P=0.024),territorialinfarcts(P<0.001),cerebraloedemaorpressureeffects(P<0.001),hyperdensemiddlecerebralarterysign(P<0.001)andhaemorrhagicinfarcts(P=0.002).Cerebralsmallvesseldisease(P<0.001)andlacunarinfarctsonCTbrain(P=0.001)wereprotectivefactors.Thosewhohadearlyneurologicaldeteriorationweremorelikelytohavediedat30days(33.3%vs1.2%;P<0.001)andhadalongerlengthofhospitalstay(24.3daysvs13.8days;P=0.025).Conclusion:Earlyneurologicaldeteriorationinacuteischaemicstrokeiscommon.Itisassociatedwithhigherriskofearlydeathandlongerlengthofhospitalstay.Thesepatientsmaybeidentifiedearlyattheirpresentations.Agreatsenseofawarenessandpromptinvestigationandmanagementareinneedtopreventitsoccurrence.

Focal Neuropathies

Windsor MakDepartment of Medicine, Queen Mary Hospital, Hong Kong SAR

Focalneuropathyisaproblemfrequentlyencounteredinclinicalpractice.Differentpatientgroupstendtohavedifferentunderlyingaetiologies.Patientswithdiabetesmellituscanhaveacutemononeuropathyaffectingthecranialaswellasperipheralnerves.Besides,theyaremorepronetochronicentrapmentneuropathies.Diabeticamyotrophyisalsoafocalproblembutpatientscanpresentheterogeneously.Vasculiticmononeuropathyandmononeuropathymultiplexcanbeseeninsomeconnectivetissuediseases.Thisconditionmayalsooccurinisolationintheabsenceofextra-neural involvement.Theelectro-andhistologicaldiagnosisofvasculiticmononeuropathymultiplexwillbediscussed.Apartfromdiabeticpatients,entrapmentneuropathyatmultiplesitescanoccur inhereditaryneuropathywithliabilitytopressurepalsiesandattheearlystageofGuillain-Barrésyndrome.Thesemodelsareillustrationsofthedouble-crushphenomenon.FocalinvolvementcanalsobeseeninmotorneuropathywithmultifocalmotorconductionblockandtheCANOMADsyndrome,whicharerarevariantsofchronicinflammatorydemyelinatingpolyneuropathy.

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Guillain-Barré Syndrome: the Immunopathogenesis and Relationship between the Different Subtypes

Nobuhiro YukiDepartments of Microbiology and Medicine, National University of Singapore, Singapore

The recentadvances inGuillain-Barré syndromeresearchhave improvedourunderstandingoftherelationshipbetweenthedifferentsubtypes.Thethreemajorsubtypesareacute inflammatorydemyelinatingpolyneuropathy(AIDP),acutemotoraxonalneuropathy(AMAN)andFishersyndrome,variationsintheclinicalpresentationsarelikelytorepresentdifferentendsofacontinuousdiseasespectrum.AIDP,whichfrequentlypresents facialpalsiesanddistalparesthesia, isassociatedwithcytomegalovirusandEpstein-Barrvirusinfections.‘Facialdiplegiaandparesthesia’showssubclinicalmotornervedemyelinationin limbsandisassociatedwithbothinfections,supportingthatthis isaregionalformofAIDP.AMANandFishersyndromeareassociatedwithCampylobacter jejunienteritis.C jejuni sialyltransferasegenepolymorphismdeterminestheganglioside-like lipo-oligosaccharidestructure.GM1-orGD1a-likelipo-oligosaccharideinducesanti-GM1or-GD1aautoantibodies,causingAMAN;whereas,GQ1b-likelipo-oligosaccharidestriggersanti-GQ1bantibodiescausingFishersyndrome.ThissuggeststhatAMANandFishersyndromearepartofthesamediseasespectrumalongwiththepresenceofoverlappingcasesofAMANandFishersyndrome.DependingontheseverityofnerveinsultinAMAN,thisautoimmuneattackcausesthelessextensive‘acutemotorconductionblockneuropathy’ormoreextensive‘acutemotor-sensoryaxonalneuropathy’subtypes.AlthoughtypicallyassociatedwithFishersyndrome,anti-GQ1bautoantibodiesarealsoassociatedwithacuteophthalmoplegiawithoutataxia, isolatedinternalophthalmoplegia,acuteoropharyngealpalsy,ataxicGuillain-Barrésyndrome,acutesensoryataxicneuropathy,Bickerstaffbrainstemencephalitisandpharyngeal-cervicalbrachialweakness,suggestingthatthesedifferentclinicalpresentationsarealsopartofacontinuousdiseasespectrum.

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S 3Common Pitfalls in the Electrodiagnosis of Entrapment Neuropathy

Alex CP ChowDivision of Rehabilitation, Hong Kong Sanatorium & Hospital, Hong Kong SAR

Carpal tunnel syndrome and ulnar nerve entrapment at the elbow are common entrapmentneuropathies.Althoughthesetwoconditionsarecommonlyseen,andtheperformanceofmotorandsensoryconductionstudiesarewelldescribedinstandardtextbooks,theelectrodiagnosticevaluationis frequentlycomplexandchallenging toeven themostexperiencedelectrodiagnosticmedicineconsultants.Anumberofthemorecommonpitfallsanditsconsequenceswouldbediscussed.


Idiopathic Intracranial Hypertension

BL ManDepartment of Medicine, Tuen Mun Hospital, Hong Kong SAR

Idiopathicintracranialhypertension(IIH)isdefinedasincreasedintracranialpressurewithoutaspace-occupyinglesionorhydrocephalusandnormalcerebrospinalfluidcomposition.IIHispredominantlyseeninwomenofchildbearingageandassociatedwithahistoryofrecentweightgain.Itisassociatedwithlossofvisualfunctioninupto25%ofcasesandprogressiontoblindnessifuntreated.TheincidenceofIIHisincreasingbecauseofanincreasedawarenessofthesyndromeandtheincreasingprevalenceofobesity.Thediagnosticevaluationofthesepatientsofteninvolvesseveralmedicalsubspecialties.Coordinatingthepatient’scarefrompresentationtofollow-upisanenormouschallenge.ThistalkoffersanupdatedreviewofIIH, includingepidemiologyandpathogenesis,clinicalpresentations,commonclinicalcourse,diagnosticcriteriaandchallenges,andtreatmentsandprognosis.

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S 5Atypical Optic Neuritis

Carmen KM Chan, Andy CO ChengHong Kong Eye Hospital, Hong Kong SAR

Typicallyweassociateopticneuritiswithretrobulbar,demyelinatingopticneuritis,whichoccurs inyoungfemales.Itmaybeassociatedwithmultiplesclerosis,anditusuallyimprovesspontaneouslywithnotreatment.WhilstthisscenariomaybetrueintheCaucasianpopulation,itisnotinfrequentthatweencounter‘atypical’opticneuritisintheChinesepopulation.Ifwemanage‘atypical’opticneuritisliketypicalones,wemaymisstheimportanttherapeuticwindow. Inthistalkwewillcoverthedifferentialdiagnoses,causesandmanagementofatypicalopticneuritis,andconditionswhichcanmimicopticneuritis.Therewillbeparticularemphasisonneuromyelitisopticaandopticperineuritis.


S 6Using Eye Movements for Topological Diagnosis of Central Disorders

David S ZeeProfessor of Neurology and Ophthalmology, Johns Hopkins School of Medicine, United States

Herewewillfocusonacarefulbedsideexaminationofeachoftheocularmotorsubsystems(saccades,pursuit,vestibularandvergence)tolocaliseandunderstandourpatients’eyemovementdisorders.Wewillapproachsaccadedisordersbasedonabnormalitiesininitiation,amplitudeandspeed,andlocalisethemtothecerebrum,brainstemorcerebellum.Wewillcomparemidbrain,pontine,medullaryandcerebellarlesionsandtheireffectsonsaccades.Midbrainlesionscauseverticalsaccadeslowing;pontinelesionscausehorizontalsaccadeslowingandmedullaryandcerebellarlesionscausesaccadeinaccuracy(dysmetria).Cerebellarvermal lesionscausesaccadehypometriaanddeepcerebellarfastigialnucleilesionscausesaccadehypermetria.Disordersofsaccadeinitiationreflecthigherlevel lesionsinthecerebralhemispheres.Pursuitdisordersarehardertolocalisebutcerebralhemispherelesionsproduceipsilateralsmoothpursuitdeficitsandcontralateralsaccadeinitiationandamplitudedeficits.Cerebellarlesions(primarilyflocculusandparaflocculus[tonsils])producemarkedpursuit(andeccentricgazeholding)deficits. Weapproachnystagmusandotherocularoscillationsusingaclassificationbasedfirstuponwhethertheslowphaseorthequickphaseistheprimaryabnormality.Wewillbuildadecisiontreebasedonwhichistheprimaryabnormality.

A Flow Chart For Classification Of NystagmusIsfixationimpairedbecauseofaslow drift,oranintrusive saccade,awayfromthetarget?

Ifaslow drift isculprit

Jerk

Unidirectional

Central Peripheral

Pendular

Acquired(MSorOPT)orCongenital

Changesdirectionwithdirectionofgaze(gaze-evoked)Vestibular(constant

velocity)

(Useeffectoffixationandvectorofnystagmus)

Changesdirectionwithviewingeye=LatentNystagmus

Acquired(velocitydecreasinge.g.cerebellar

Congenital(velocityincreasing)

A Flow Chart For Classification Of Nystagmus

Ifasaccadeawayfromthefixationtarget(saccadicintrusion)istheculprit?

Withanintersaccadicinterval(~200ms)beforeareturnsaccade

Square-wavejerks(smallsaccadesawayfromandbacktofixation),macrosquarewavejerks(muchlargersaccades)

Macrosaccadicoscillations,largesaccadesaroundfixation(extremedegreeofsaccadehypermetria)

Withoutanintersaccadicinterval(backtobacksaccades)

Opsoclonus(multiaxis)

Flutter(oneaxis)includes‘voluntarynystagmus’

OcularbobbingSingleverticalsaccadeawayfromfixationfollowedbyslowdriftback


New Targets for Treatments of Parkinson’s Disease

Ken KL Yung, John MT Chu, Cathy NP Lui, Olivia TW NgDepartment of Biology, Hong Kong Baptist University, Hong Kong SAR

ClinicalmanagementofParkinson’sdisease involvespharmaceutical interventionsanddeepbrainstimulation.Variable resultsare found indifferentpatientsand thereareneeds to look fornewtreatments.Dataobtainedfromlaboratorieshaveimplicatedthattherearepotentialtargetstoofferneuroprotectiontodopaminergicorprovidecellreplacementofdopaminergicneurons.Thepresenttalkwill focusonseveralaspects.Firstly,rolesandmechanismsofneurokininsandtheirreceptors,namelyNKreceptors,willbediscussed.RecentresultsinourlaboratoryhaveshownthatactivationofNK1receptorsisneuroprotectivewhereasactivationofNK3receptorsisdetrimentaltothesurvivalofdopaminergicneurons.Secondly,rolesofN-methyl-D-aspartatereceptor(NMDAR)2Bingenerationofcelldeathsignallinghavebeeninvestigated.GenesilencingapproachhasbeenemployedinreducingthefunctionalexpressionofNMDAR2Bproteinsinanimals,andtheseapproacheshavebeenfoundtoprovideneuroprotectiveeffectstothedopaminergicneurons.Moreover,wehavealsodiscoveredthatthereareexpressionsofreactiveastrocyteswithinanextremelyshorttimewindowaftertheonsetofParkinson’sdiseaseinanimals.Thereactiveastrocytesexpressmarkerofneuralstemcellsandtheyarefoundtoreleaseneurotrophicfactorswhichcanprotectdegenerationofdopaminergicneuronsandtheirterminals.Thereforereactiveastrocytesarepotentialcellsthatcanbeemployedincellreplacementtherapyinregenerativemedicine.Takingall together,therearepotentialnewtargetsforParkinson’sdiseaseandsomeofthosecouldbetakenintoaccountfordevelopmentofnewapproachesinclinicalmanagementofParkinson’sdisease.

AcknowledgementThe present work was supported by General Research Fund (HKBU 262107 and 262210), Hong Kong Research Grants Council and Hong Kong Baptist University RC/AoE/08-09/02.

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S 8Levodopa/carbidopa/entacapone as First-line Levodopa Formulation: Pharmacokinetic ConsiderationsThomas MüllerDepartment of Neurology, Center for Parkinson’s disease and Multiple sclerosis, St. Joseph Hospital Berlin-Weissensee, Gartenstr. 1, 13088 Berlin, Germany

Levodopa(LD)isthemostefficaciousandbesttoleratedcompoundforthetreatmentofpatientswithParkinson’sdisease(PD).Thiscompoundisrapidlymetabolisedinparticularbytheenzymesaromaticdopadecarboxylase(DD)andcatechol-O-methyltransferase(COMT).InhibitionofDDwithcarbidopa(CD)markedlyreducestheperipheralLDdegradation.Buttheshortplasmahalf-lifeofLD/CDpreparationslimits itsclinicalbenefit.Therefore,followingtheLDtransport intothebrainandtheconversiontodopamine,analternatingstimulationofnigrostriatalpostsynapticdopaminereceptorstakesplace.Inthelongtermthesefluctuationsofdopamineconcentrationssupportonsetofmotorcomplications(MC)inPDpatients.GeneralopinionisthatlossofcentralcompensatorymechanismsofdopaminemetabolismisresponsibleforthedevelopmentofMC.However,intheperiphery,LDtroughsarepreponderantlyassociatedinclinicalpracticeaccordingtopharmacokineticinvestigationswiththeMCwearingoff,whichisthereappearanceofmotorsymptomswithdecreasingdrugeffect.ApossiblestrategytoprolongplasmametabolismofLD/CDistheadditionofaCOMTinhibitor,ieentacapone(EN).Accordingly,combinationoftheCOMTinhibitorENtoLD/CDimprovedwearingoff,sinceENprolongsLDhalf-lifeandavoidstroughs.PharmacokinetictrialswithPDpatientsalsoshowedthatplasmaLDpeaksaremostlyrelatedtotheclinicalmanifestationoftheMCdyskinesia,whichappearasinvoluntarymovements.OnetimeadditionofENtoaLD/CDformulationshowednoincreaseofperipheralmaximumLDconcentration.ButrepeatcombinationofENtoeachLD/CDintakeelevatedplasmaLDbioavailabilityandpeaks.ThereforeswitchfromaLD/CD–toaLD/CD/ENregimenmayalsoaskforreductionofLD/CDdosingordelayofthenextLD/CDintake,toavoidonsetofthemostcommonpeakdosedyskinesia.MoreoveritmaybeusefultostartwithhigherdosingofLDformulationsinthemorningandthentotitratewithdifferentLDdosagesduringthedayaccordingtotheindividualpatient’smotorbehaviourinmoreadvancedstagesofPD,asLDplasmaavailabilitytendstoaccumulateduringthedaywithrepeatedLDdosing.Thisapproachmayalsopreventonsetofdyskinesia.Inconclusion,pharmacokineticstudiesonperipheralLDmetabolismandmodeofintakeunderlinetheirimportanceasperipheralcomponentsforMCmanifestationsinPDpatients.ThesepharmacokineticfindingsinPDpatientsandhealthyvolunteersontheimpactofCOMT-inhibitiononLDplasmabehaviourmayalsosuggest,thatLD/CD/ENasfirst-lineLDformulationmaybemoreappropriate.LD/CD/ENprovideslessfluctuatingLDplasmalevels,asshownbypharmacokinetictrials.Fromtheclinicalpointofview,LDtherapyis lesscomplex,whenLD/CD/ENis introducedasfirst-lineLDformulation,if tolerated, inPDpatients.ThisapproachcircumventstheabovementionedCOMT-inhibitorrelatedadaptionofLDintakefollowingtheonsetofwearing-offphenomenaduringrepeatedLDintake.SuchaswitchfromLD/CDtoLD/CD/ENmaybecomplexinparticularinmoreadvancedPDpatients,asitmayaskforarepeatedchangeofLDdoses,ofnumberofintakesandofdosingintervals.


Homocysteine and Entacapone Treatment

Thomas MüllerDepartment of Neurology, Center for Parkinson’s disease and Multiple sclerosis, St. Joseph Hospital Berlin-Weissensee, Gartenstr. 1, 13088 Berlin, Germany

Thesagaofharmfuladministrationoflevodopa(LD)inthetreatmentofParkinson’sdisease(PD)resultedfromoutcomesofanimal,andcellculturestudiesandtheclinicalobservationofmotorcomplicationsrelatedtotheshortplasmahalf-lifeofthedrug.Thisdiscussiondidonlypartiallyconsiderafurtheraspect,which isassociatedwith the long-termadministrationofLD.ChronicLD intake increaseshomocysteine levels,whichmaysupportprogressionof thediseaseduetoconcomitantonsetofneuropsychiatricsymptomsandcomorbidities, i.e.vasculardisease.Homocysteinedecreasemaythereforebeafuturetherapeuticchallenge,whichmaybeachievedbysupplementationwithcertainvitaminorLD/DDIadministrationwithacatechol-O-methyltransferase(COMT)inhibitor.Monitoringofhomocysteinemayalsoserveasbiomarkerforthedetoxificationpotentialofendogenous,exogenousandenvironmentaltoxins.Thesesubstratesmayalsoaccumulateinthenervoussystem,ashomocysteinesynthesisisassociatedwithO-methylationwhichhasabroaddetoxificationpotential.Intheperiphery,therapeuticapproachesforthisLDmediatedhomocysteineincreasearevitaminsupplementation,iefolicacid,orapplicationofLDwithaninhibitorofCOMT.Inthebrain,abloodbraintrespassingprecursoroffolicacidoracentrallyactingCOMTinhibitormayrepresenthypotheticaltherapeuticapproaches.ThisCOMTinhibitorshouldbeappliedtogetherwithanoxidativestressreducingMAO-B-inhibitor,inordertoforcecentraldopaminemetabolismfurtherdownviathemethylationpath.

Deep Brain Stimulation for the Treatment of Parkinson’s Disease

Eugene C LaiThe Methodist Neurological Institute, Houston, Texas, United States

Deepbrainstimulation,whichinvolvestheimplantationofadevicethatsendselectrical impulsestospecifictargetsinthebrain, isthesurgicaltreatmentofchoicewhenParkinson’sdisease(PD)motorcomplicationsareinadequatelymanagedwithmedications.Thispresentationwilldiscussthefindingsofadouble-blinded,randomisedcontrolledtrialcomparingbestmedicaltherapy(BMT)anddeepbrainstimulation(DBS)ofthesubthalamicnucleus(STN)andglobuspallidus(GPi)forthetreatmentofPD.Atotalof255patientswithidiopathicPDwereenrolledinPhaseIofthestudy.After6months,DBSwasmoreeffectivethanBMTinimprovingontimewithouttroublingdyskinesia,motorfunction,andqualityoflife,butwasassociatedwithanincreasedriskofseriousadverseevents.InPhaseII,299patientswererandomlyassignedtoundergoeitherSTNorGPisurgeryandstimulation.At24months,patientshadsimilarimprovementinmotorfunctionaftereithersubthalamicorpallidalstimulation.Seriousadverseeventsoccurredin56%ofpatientsundergoingsubthalamicstimulationandin51%ofthoseundergoingpallidalstimulationwithnosignificantbetween-groupdifferences.ThebeneficialeffectsofDBSweresustainedover36months.Slightdeclinesinsomequality-of-lifesubscalesfollowinginitialgainsandgradualdeclineinneurocognitivefunctionslikelyreflectedunderlyingdiseaseprogressionandtheimportanceofnonmotorsymptomsindeterminingqualityof life. InordertoplanasuccessfulDBStreatment,factorsincludingpatientselection,targetidentification,skilledteamwork,post-surgicalcareandlong-termsupportneedtobeconsidered.

References1. Weaver FM, Follett K, Stern M, et al. Bilateral deep brain stimulation vs best medical therapy for patients with advanced Parkinson

disease: a randomized controlled trial. JAMA 2009;301:63-73.2. Follett KA, Weaver FM, Stern M, et al. Pallidal versus subthalamic deep-brain stimulation for Parkinson’s disease. N Engl J Med

2010;362:2077-91.

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Psychogenic Non-epileptic Seizures

David G VosslerUniversity of Washington, Seattle, WA, United States

Psychogenicnon-epilepticseizures(PNES)arefoundinapproximately25to30%ofpatientsadmittedtoepilepsymonitoringunitsforseizurediagnosis.Formerlyknownaspseudoseizures,PNESareeventsofpsychologicalthatalters,orappearstoalter,neurologicfunctioninanepisodicmannercausingtransientmotorsigns,orsensory,autonomicorpsychicsymptoms,whichresemblethoseoccurringduringepilepticseizures.PNESareoftenincorrectlydiagnosedforyears.Thisleadstodelayinpropertreatment,over-utilisationofhealthcareresources,patientanxiety,andpotentialseriouscomplicationsfrominappropriatemedicaltreatments.OlderreportsclaimmanyPNESpatientsalsohaveepilepticseizures,butIandothershavefoundapproximately10%overlap.1Ifound41/52(79%)werefemalewithamedianageof38years.Kalogjera-SackellaresdescribestwomajorpsychologicalmechanismsforPNES:(I)Post-traumaticpseudoseizuresyndromeinwhichPNESdevelopduetoone/severaltraumaticexperiencesthatthepatientcannotadequatelyprocessorintegrategivenhis/herownpsychologicalresources,and(II)Developmentalpseudoseizuresyndrome.Inthefirstcategory,thetraumacanbesexual,minorphysical,parental,majorphysicalorso-calledchronictraumaticlifecourse.ExperiencedelectroencephalographersidentifyPNESusingclinicalsemiology.FeaturessuggestiveofPNESincludenon-physiologicornon-anatomicprogressionofsigns,out-of-phasebodymovements,closedeyes, thepresenceofstuffedanimalsinadultswithnormalintelligence,stuttering,2inappropriateemotions,andothers.

References1. Vossler DG. Nonepileptic seizures of physiologic origin. J Epilepsy 1995;8:1-10.2. Vossler DG, Haltiner AM, Schepp SK, et al. Ictal stuttering: a sign suggestive of psychogenic nonepileptic seizures. Neurology

2004;63:516-9.

Upcoming Antiepileptic Drugs: the United States Experience

David G VosslerUniversity of Washington, Seattle, WA, United States

Since1993, theUnitedStatesFood&DrugAdministration(FDA)hasapproved15newantiepilepticdrugs(AEDs).TheyearsofapprovalandtheAEDs,inorder,are1993felbamate,1994gabapentin,1995lamotrigine,1996fosphenytoin,1997topiramateandtiagabine,1999levetiracetam,2000oxcarbazepineandzonisamide,2005pregabalin,2008 lacosamideandrufinamide,2009vigabatrin,2010ACTHgel,2011ezogabine.Theuseoffelbamateislowduetoa1/4000riskofaplasticanaemiaorhepaticfailure.Vigabatrinhas lowusagedue to theriskofperipheralvisual field loss.Oxcarbazepine isused inmonotherapyforfocal-onsetseizures(FOS).ZonisamideisapprovedforadjunctivetherapyforadultswithFOS,but isalsousedaroundtheworldformyoclonicseizures.Tiagabine isaselectiveGABAreuptakeinhibitor(SGRI)usedinadjunctivetherapyforFOS.Fosphenytoinisthewater-solublepro-drugofphenytoin,fori.v.andi.m.use.Lacosamide,anaminoacid,hasanovelmechanismofaction:itenhancesslow inactivationofneuronal sodiumchannels.Thiscontrastswith theclassicAEDscarbamazepine,lamotrigine,oxcarbazepine,phenytoin,andrufinamidewhichenhancerapidinactivationofsodiumchannels.Lacosamideisavailableinivandoralforms.ItisapprovedforadjunctivetherapyforadultswithFOS,butresearchstudiesofgeneralised-onsetseizuresandchildrenareunderway.RufinamidehassomeefficacyinFOS,butisFDAapprovedonlyforLennox-Gastautsyndrome.ACTH(Acthargel) isapprovedforepilepticspasmsonly.AEDsawaitingFDAapprovalareeslicarbazepine,brivaracetam,perampanelandothers.

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Sleep and Epilepsy

Carl BazilCollege of Physicians and Surgeons at Columbia University, United States

Brainactivityduringsleepisverydifferentfromthatseenduringwakefulness.Duringnon-REMsleep,thebrainshowsmuchmoresynchronisedactivitythanduringwakefulnessorREMsleep.Sleepdisruptionisalsoknowntobeariskfactorforseizureexacerbation,andmanydrugsaffectsleep,sleepdisorders,andseizures.Itisthereforenotsurprisingthatepilepsyhasmanypotentialinteractionswithsleepandsleepdisorders.Theseincludedifferentialdiagnosis,timingofcertainseizuretypes,sleepdisruptionduetoseizures,andthefrequencyandtreatmentofcoexistingsleepdisorders. Fromaclinicalstandpoint,bothseizuresandsleepdisorderstypicallyoccuroutsideofamedicalsetting.Theyarenotwitnessedby the treatingphysician, thereforediagnosis reliesheavilyondescriptionsfrompatientandonlookers,whichcanbeincompleteatbestandmisleadingatworst.Asbothparasomniasandnocturnalseizuresinvolvealterationinbehaviourand/orconsciousness,thesecanbedifficulttodistinguish.Prominentexamplesincludenocturnalfrontallobeepilepsy,disordersofarousal,andcataplexy.Thusdifferentialdiagnosisofnocturnalseizuresthereforeoftenincludessleepdisorders,andviceversa. Certainepilepsysyndromesareprofoundlyaffectedbythesleep-wakestate.Prominentexamplesincludejuvenilemyoclonicepilepsy,nocturnalfrontallobeepilepsy,andbenignrolandicepilepsy.Somepartialseizuresalsohavearelationshipwithsleep;frontallobeseizuresforexampletendtoarisemorefrequentlyfromsleep.Theseobservationscanhaveimplicationsfordiagnosisandtreatment. Nocturnalseizuresdisruptsleep,evenwhenquitebrief.Thisdisruptioncanberesponsibleforthedifficulty indaytimefunctioningmanypatientshavefollowingseizures.Assleepdisruptioncanincreasetheriskofseizures,thiscansetupacycleofseizures,consequentsleepdisruption,andfurtherintractabilityofseizures. Sleepdisordersarecommoninthegeneralpopulation,andmanyoccurevenmorefrequentlyinpatientswithepilepsyincludinginsomnia,obstructivesleepapnoea,periodic limbmovements,andrestless legssyndrome.Theseareoftenunrecognised,butwhenuntreatedcanalsocontributetodecreasedfunctioningandtointractabilityofepilepsy. Mostpatientswithepilepsytakeanticonvulsantdrugs.Thesehavethepotentialtoaltersleepthroughimprovement innocturnalseizures; througheffects(positiveornegative)oncoexistingdepression,anxiety,orsleepdisorders;andthroughindependenteffectsonsleep. Qualitysleepisknowntobeimportantintheconsolidationofmanykindsofmemory,thereforesleepdisruption(duetosleepdisorders,seizures,ormedication)cancontributetothememoryproblemssooftendescribedbypeoplewithepilepsy.Vigilanceforpossibleconcurrentsleepdisordersisimportantparticularlywhenpatientshaveunexplainedsomnolence,memoryproblems,orcontinuedseizuresespecially ifseizuresarepredominantlynocturnal.Overall,knowledgeofthe interactionsbetweenepilepsyandsleepdisordersisessentialtotheoptimalcareofpatientswithepilepsy.

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Psychiatric Issues in Epileptic Disorders

Joyce LamDepartment of Psychiatry, The Chinese University of Hong Kong, Hong Kong SAR

Overthepastdecades,increasingliteraturessuggestedthatepilepticdisorderhascomplexrelationshipswithotherneurological,cognitive,psychiatricandbehaviouraldisorders.Epilepsyisnowconceptualisedasaspectrumcondition,as theco-morbidconditionshavebeendemonstratedtosharecommonphenotypesandaetiologicalfactorswithepilepsy.Psychiatricillnesses,suchaspsychosis,anddepression,havebeendemonstratedtobehighlyprevalentamongpatientswithepilepsy.Forexample,cross-sectionalstudiesfoundthattheprevalenceofdepressioninpatientswithepilepsyrangedfrom10%to30%incommunity-basedsamplesandevenmountedupto50%inclinicpopulations.Insteadofsimplyapsychologicalreactiontochronicillness,studiesfocusingonthetemporalassociationsestablishedthatdepressionisariskfactorforincidentunprovokedseizures,suggestinganunderlyingbi-directionalrelationship.Psychiatricdisordersarealsofoundtosharegeneticorneurochemicalbasiswithepilepsy.The“iatrogenic”effect, includingtheuseofpsychotropics,anticonvulsantsandsurgicaltreatmentofepilepsy,furthercomplicatestherelationships. Thispresentationwill focusonthepsychiatricandcognitiveco-morbiditiesofepilepticdisorder,illustratingtheclinicalepidemiology,aetiologicalaspects,treatmentimpactandtheco-morbiditiesinspecialclinicalpopulations.

Basic Neurogenetics: What Clinical Neurologists Need to Know

KY MokDepartment of Molecular Neuroscience, UCL Institute of Neurology, United Kingdom

Atbedside,understandingthegenetictransmissionandtheroleinpathomechanismis important.Itiscrucialnotonlyinmanagementofthepatient.Theimportanceextendstothecareoftheirfamilymembersandmayhavesignificantsocial implication.Atmacrolevel,understandingthegeneticsofdiseaseprovideusaviewonthepathophysiology. Syndromescausedbychromosomal insertionsanddeletionsarewelldocumented.Majorityofthesepatientsmaynotbeunderthecareofneurologists.Mendelianormonogenicdisorderisprobablythemostwell-knowntypeofgeneticmechanismknowntoclinicianbutit isnottheonlyone.Manyneurodegenerativediseases,egParkinson’sdisease,motorneurondiseasehaveasmallportioncausedbymonogenicdisorder.Majorityaremulti-factorial,iecomplexdiseasesorcommondisease.Thispatientgroupcontributesthebulkofclinicalload.Theapparentlysameclinicalentitycanbecausedbyasinglemutationwithverylargeeffectsizeoramultipleoffactorswithintermediate/smalleffectsize. Genotypephenotypecorrelationcanbedifficult todissect.Monogenicdisorderscanhavethephenotypealteredbyotherfactors.Sexandthespecialgenetictransmissionofmitochondriaareamongthesefactors.Furthermore,differentmutationswithinthesamegenemayhavedifferentphenotypeandmodeofinheritance. Itisabsolutelytruethatpeoplefromdifferentethnicoriginhavemorefeaturesincommonthanthedifference.Thereistheothersideofthecoin,iedifferenceindiseasepatternisnolessgenuinethanthecommonality.Thisisparticularimportantintheapproachtogeneticdisorders,bydefinition,causedbythegeneticmakeupoftheindividual.Thedifferentialmakeupcontributestothedifferenceinethnicgroupandatthesametime,thediseasespectrum.

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Molecular Neurogenetics: Tools and Methods

Liz YP YuenDepartment of Chemical Pathology, The Chinese University of Hong Kong, Prince of Wales Hospital, Hong Kong SAR

Inthepost-genomicera,thenumberofcausativegenesidentifiedforneurologicaldisordersisincreasinginaveryrapidpace.Thesediscoveriesareaccompaniedbyaproliferationofmolecularmethodologiesthatcanbeappliedinaclinicallaboratoryforaccuratedetectionofmutationsinthesegenes. Thefollowingfactorsthatdeterminethechoiceofmolecularmethodsforaparticulardiseasewillbediscussed.1. Mutationtypes:smallmutations(egsinglebasesubstitutions,smalldeletions/insertions,splicesite

mutations,etc),trinucleotiderepeatsandlargemutations(egexonicorwholegenedeletion,largeinsertion,complexrearrangements)

2. Geneticandallelicheterogeneity3. ProblemsspecifictomitochondrialDNA Anoverviewofcommonlyusedmolecularmethodsthatareusedinclinical laboratorieswillbepresented.1. Numberingoftrinucleotiderepeats2. Mutationscanningversusdirectsequencing3. Methodsfordetectionoflargemutations4. Methodsonthehorizon:nextgenerationsequencing Interpretationofmolecular findingsremainsoneof themostdifficult tasks ingenetic testing.Determinationofpathogenicityofanovelvariantmaynotbestraightforward.Commoninterpretationproblemsandpotentialsolutionswillbediscussed.

Advanced Neurogenetics: Frontiers in Research

KY MokDepartment of Molecular Neuroscience, UCL Institute of Neurology, United Kingdom

RecentadvancesinParkinson’sdiseaseandmotorneurondiseasewillbeusedasillustration.Discussionswill includeunderstandingdiseasemechanismsbasedon thestudyresultsandpracticalclinicalimplicationsinthenewtechnologies.


S 19Current Approach for Chronic Migraine Management

SJ WangThe Neurological Institute, Taipei Veterans General Hospital; National Yang-Ming University School of Medicine, Taipei, Taiwan

Chronicdailyheadache(CDH)isdefinedisdefinedas≥15headachedayspermonthfor≥3months.Chronicmigraine (CM) isclassifiedasadisablingcomplicationofmigrainewithoutauraby theInternationalClassificationofHeadacheDisorders,2ndedition(ICHD-2),2004.ItisonesubtypeofCDHandisdefinedas≥15headachedayspermonth,≥8migrainedaysfor≥3monthsintherevisedICHD-2criteria,2006.Thepopulationprevalenceisestimatedtobeabout1.5to2%.ItisthecommondiagnosticentityinheadacheclinicsandmanypatientswithCMoverusedabortivetreatmentincludingergotamine,triptans,analgesicsornarcotics.EmergingevidencesuggeststhatepisodicmigraineandCMdiffer,notjustindegree,butalsoinkind.Theexactunderlyingmechanismisnotclearbutsomestudiessuggestcorticalhyper-excitabilityordysinhibition.PatientswithCMhaveworsesocioeconomicstatus,reducedhealth-relatedqualityoflife,increasedheadache-relatedburdenincludingimpairmentinoccupational,social,andfamilyfunctioning,andgreaterpsychiatricandmedicalcomorbiditiesincomparisonwithepisodicmigraine. Adetailedhistorytakingandneurologicalexaminationareimportantforthediagnosisofchronicmigraine.Secondaryheadachedisordersshouldbeexcludedbeforemakingthediagnosis.Routineneuroimagingstudiesarenotevidence-based.Aheadachediaryisbothhelpfulfordiagnosisandfollow-up.Psychiatriccomorbidityespeciallydepressiveandanxietydisordersareverycommoninpatientswithchronicmigraine.Psychiatristsshouldplayamajorroleinthemulti-disciplinarytreatmentteamforbothpsychiatricconsultationandtreatment.Withdrawalofoverusedabortivetreatmenthasbeensuggestedforthosewithmedicationoveruseheadache.However,whetherprophylacticagentsaregivenatthesametimeorafterwithdrawaldonothaveconclusions. PatientswithCMusuallyareexcludedfrommigraineprophylaxistrialsbecausetheyareconsideredtobetoohighlydisabledandtreatmentresistant.However,thereareasubstantialamountofthesepatients,andindeed,theyarethepatientswhoimperativelyrequireeffective,safe,andwell-toleratedheadacheprophylactictherapy.Uptonow,onlyonabotulinumtoxinAandtopiramatehadthebestevidenceforchronicmigraine. The largetrialPhase3ResearchEvaluatingMigraineProphylaxisTherapy(PREEMPT),consistingof twostudies(PREEMPT1andPREEMPT2),aimstoevaluatetheefficacy,safety,andtolerabilityofonabotulinumtoxinAusinga fixed-site, fixed-dose injectionprotocol (in total155Uover31sites)andanoptionaladditionof40U‘follow-the-pain’paradigm.PREEMPT1and2demonstrated thatonabotulinumtoxinAisaneffectiveprophylactictreatmentforCMinreducingheadachedayspermonthastheprimaryoutcomeandmostsecondaryheadachesymptommeasures.Mostadverseevents(AEs)weremildtomoderateinseverity,andfewpatientsdiscontinuedthetrial(onabotulinumtoxinA,3.8%;placebo,1.2%)duetoAE.TworandomisedcontrolledstudiesconductedintheUnitedStatesandEurope,respectively,demonstratedthattopiramateiseffectiveasheadacheprophylaxis inCM.However,thetherapeuticgainofthesetwotreatmentagentsalsowasmodestdespitedifferentstudyend-points.ThemoderateeffectsizesofthesestudiespointedouttherefractorinessofCMandapotentialneedofcombinationtherapyinfuturetrials.Ofnote,bothtopiramateandonabotulinumtoxinAshowedsimilarefficacyintreatmentofCMbetweenthosewithandwithoutmedicationoveruse.


S 20Updated Guidelines for Treatment of Neuropathic Pain

PP ChenDepartment of Anaesthesia, Alice Ho Miu Ling Nethersole Hospital / North District Hospital, Hong Kong SAR

Severalrecommendationsregardingthetreatmentofneuropathicpainhavebeenpublishedintheliterature,mostlyunder theauspicesofmajorprofessionalandacademicorganisations inNorthAmerica,LatinAmericaandEurope.1-6Overall, therearefewdifferencesamongthepharmacologicalrecommendationsfortreatingneuropathicpain.Inmost,tricyclicantidepressantsandcalciumchannelα2-δ ligandsareoftenregardedasfirst-linetherapy,whileselectiveserotoninnoradrenalinereuptakeinhibitorsandtopicallignocaineareconsideredfirst-orsecond-linetreatments.Tramadolandopioidanalgesicsareregardedassecond-orthird-linetreatment.Afewguidelinessuggestedcombinationtherapywhenmonotherapyisunsatisfactory.5-7 Despite these guidelines providing evidence-based recommendations for the treatment ofneuropathicpain,studieshaveshownthatsuccessfultreatmentsofneuropathicpainatbestprovideonlypartialpainreliefforabouthalfofthosetreated.8Inpart,thisoutcomeisduetothelimitationsintheexistingclinicalevidenceandresearchinneuropathicpain.Recentrecommendationssuggestamorerationalstrategyforthetreatmentofneuropathicpain,includingthefollowing:(1)carefulassessmenttoestablishadiagnosis,(2)considerthesymptomswhichmaysuggesttheunderlyingmechanisms,(3) identifyco-morbiditiesthataffectthetreatment,(4)establisharealisticexpectationwithpatient,(5)selecttheappropriatetreatment—first-,second-,third-linedrugs,avoiddrugwithsignificantadverse

effects,considerdrugthatwillalsoimproveothercomorbidities,egsleepandmood,(6)measurefunctionalandHRQOLoutcomesotherthanpain,(7)reassessthepatientaftertrialperiodoftherapy,(8)considercombinationtreatmentifmonotherapyfailed,(9)refertopainspecialistsiftherapyfailedandifmultidisciplinarymanagementisdeemednecessary. Othernewertreatmentoptionsarealsodiscussedinthelecture.

References1. Dworkin RH, O’Connor AB, Backonja M, et al. Pharmacologic management of neuropathic pain: evidence-based

recommendations. Pain 2007;132:237-51.2. Moulin DE, Clark AJ, Gilron I, et al. Pharmacological management of chronic neuropathic pain—consensus statement and

guidelines from the Canadian Pain Society. Pain Res Manag 2007;12:13-21.3. Finnerup NB, Otto M, McQuay HJ, Jensen TS, Sindrup SH. 4. Algorithm for neuropathic pain treatment: an evidence based

proposal. Pain 2005;118:289-305.4. Attal N, Cruccu G, Haanpää M, et al. EFNS guidelines on pharmacological treatment of neuropathic pain. Eur J Neurol

2006;13:1153-69.5. Attal N, Cruccu G, Baron R, et a. EFNS guidelines on the pharmacological treatment of neuropathic pain: 2010 revision. Eur J

Neurol 2010;17:1113-e88.6. Tan T, Barry P, Reken S, Baker M; Guideline Development Group. Pharmacological management of neuropathic pain in non-

specialist settings: summary of NICE guidance. BMJ 2010;340:c1079.7. Acevedo JC, Amaya A, Casasola Ode L, et al. Guidelines for the diagnosis and management of neuropathic pain: consensus of a

group of Latin American experts. J Pain Palliat Care Pharmacother 2009;23:161-81.8. Dworkin RH, O’Connor AB, Audette J, et al. Recommendations for the pharmacological management of neuropathic pain: an

overview and literature update. Mayo Clin Proc 2010;85(3 Suppl): S3-14.


S 21Trigeminal Autonomic Cephalalgias

TH TsoiDepartment of Medicine, Pamela Youde Nethersole Eastern Hospital, Hong Kong SAR

Trigeminalautonomiccephalalgias(TACs)areagroupofprimaryheadachedisorderscharacterisedbyunilateraltrigeminaldistributionpaininassociationwithipsilateralcranialautonomicfeatures.TACsincludeclusterheadache,paroxysmalhemicraniaandshort-lastingunilateralneuralgiformheadacheattackswithconjunctivalinjectionandtearing(SUNCTsyndrome),whichareallshort-lastingheadaches.Theseshort-lastingheadacheswithautonomic featuresarecharacterisedbyanexusofactivationbetweentrigeminalafferents(givingrisetopain)andcranialparasympatheticefferents(givingrisetoautonomicfeatures). In1997,GoadsbyandLiptonfirstproposedthattheseheadachesshouldbeclassifiedtogetherasa linkedpathophysiology is thehallmarkof thesesyndromesandsuggestedthetermtrigeminal-autonomiccephalgias(TACs)thoughitdoesnot implyall theseheadacheshaveacommonpathogenesis.Theirproposalwasadoptedby the InternationalHeadacheSociety.TheheadachesaregroupedintoChapter3ofthe2ndeditionofInternationalClassificationofHeadacheDisorders(ICHD-II).ThenosologyofhemicraniacontinuaisunclearandisplacedintheChapter4oftheICHD-II.Ithasacontinuouspainwithexacerbationthatcanincludecranialautonomicsymptomsaspartofthephenotype. Cranialparasympatheticactivation(lacrimation,rhinorrhoea,nasalcongestionandeye-lidoedema)andsympathetichypofunction(ptosisandmiosis)arethesignaturefeaturesofallTACsandhemicraniacontinua.Despitetheirsimilarities,thesedisordersdifferintheirclinicalmanifestationsandresponsetotherapy,thusitisimportanttorecognisethem.Theimportanceofdiagnosingthesesyndromesresidesintheirexcellentbuthighlyselectiveresponsetotreatment.Trigeminal-autonomicreflexisbelievedtobeimplicatedinthepathophysiologyofTACsandithasbeenshowninanimalexperimentsthatstimulationoftrigeminalefferentscanresult incranialautonomicoutflow.PETandfunctionalMRIstudieshavedemonstratedipsilateralhypothalamicactivationinclusterheadacheandSUNCTsyndrome,suggestinganimportantroleofhypothalamicabnormalityinthesetwoTACs.TheimplicationofhypothalamusinpathogenesisofTACsisfurthersupportedbythesuccessoftreatmentwithhypothalamicstimulationinpatientswithdrug-resistantchronicclusterheadache. Inthepastfewyears,manycasesofsecondaryTACorTAC-likesyndromehavebeenaddedintheliterature.Thereareno‘typical’warningsignsorsymptoms.NeuroimagingshouldbeconsideredinallpatientswithTACorTAC-likesyndromesbeforemakingthediagnosisofaprimaryheadache.


S 22Anticoagulation for Prevention of Stroke in Patients with Atrial Fibrillation

Jaseong KooDepartment of Neurology, Stroke Center, Seoul St. Mary's Hospital, Catholic University of Korea, Korea

Atrial fibrillation (AF) is themostcommoncardiac rhythmdisorder.Comparedwith thegeneralpopulation,peoplewithAFhavea5-foldincreasedriskofstroke.AspirinandvitaminKantagonists(VKAs)areusedforpreventionofAF-relatedstroke.Comparedtoplacebo,aspirinreducesthestrokeriskby22%.VKAsreducethestrokeriskby68%comparedtoplaceboand52%comparedtoaspirin.VKAsarethereforecurrentlyrecommendedasfirst-linetherapyinpatientswithAFandamoderateorhighriskforstroke.However,treatmentwithVKAsisoftenpoorlymanagedandunderusedbecauseofseveraldrawbacks.Themajordrawbacksareunpredictableinteractionswithfoodandotherdrugs,theinconvenienceofINRmonitoring,theneedfordoseadjustment,andtheperceivedriskofbleeding. Recently,threenewnoveloralanticoagulants(dabigatran,rivaroxaban,apixaban)weredeveloped.TheneworalanticoagulantsallsharenotableadvantagesovertheVKAs,includingpredictableanticoagulanteffectsatfixeddoses,limiteddrugandfoodinteractions,noneedforroutinebloodlevelmonitoring,andabroadertherapeuticwindow.Currently,datafromfourlargeclinicaltrials(3warfarintrials[RE-LY,ROCKET-AF,ARISTOTLE]and1aspirin-controlledtrials[AVERROS])ontheefficacyandsafetyoftheseagentsareavailable. DabigatranisadirectthrombininhibitorandwastestedintheRE-LYtrial.RE-LYisarandomisednon-inferioritystudywithanopentreatmentandblindedendpointassessment.InRE-LY,18113patientswhohadAFandariskforstrokewererandomisedtoreceiveeitherafixeddoseof110or150mgdabigatrantwicedailyordose-adjustedwarfarin(INR2-3).Atadoseof110mgtwicedaily,dabigatranwasassociatedwithasimilarrateofstrokeandsystemicembolismtowarfarin,andasignificantlylowerrateofmajorbleedingthanwarfarin.Atthehigherdoseof150mgtwicedaily,therateofstrokeandsystemicembolismwassignificantlylowerthanwithwarfarin,buttherateofmajorbleedingwassimilartothatassociatedwithwarfarin. RivaroxabanisadirectfactorXainhibitorandwastestedintheROCKET-AFstudy.ROCKET-AFisadouble-blindnon-inferioritystudywhichrandomised14264patientswithAFwhowereat increasedriskforstroketoreceiveeitherrivaroxaban20mgoncedailyordose-adjustedwarfarin(INR2-3). Intheprimaryanalysis,rivaroxabanwasassociatedwithasignificantlylowerrateofstrokeandsystemicembolismthanwarfarin.Intheintention-to-treatanalysis,rivaroxabanwasnon-inferiortowarfarinforpreventionofstrokeorsystemicembolism.Theratesofmajorandnon-majorclinicallyrelevantbleedingweresimilarbetweenthegroupswithasignificantlylowerrateofintracranialhaemorrhageandfatalbleedingintherivaroxabangroup. ApixabanisananotherdirectXainhibitorandwastestedintheAVERROSandARISTOTLEstudies.AVERROSisarandomiseddouble-blindstudytoinvestigatethesuperiorityofapixaban5mgtwicedailycomparedtoaspirin(81-324mgperday)in5599patientswithAFandariskforstrokebutarenotsuitablefororareunwillingtoreceiveVKAs.Apixabanwasassociatedwithasignificantlylowerrateofstrokeandsystemicembolismandasimilarrateofmajorbleedingcomparedwithaspirin.ARISTOTLEisadouble-blindnon-inferioritywhichrandomised18201patientswithAFandatleastoneadditionalriskfactorforstroketoreceiveeitherapixaban5mgtwicedailyordose-adjustedwarfarin(INR2-3).Theratesofstrokeorsystemicembolismandmajorbleedingweresignificantlylowerwithapixabanthanwithwarfarin. It isnotappropriate tocomparedirectly theefficacyandsafetyof theneworalanticoagulantsbecausethestudydesignandthestudypopulationweredifferentamongtheclinicalstudies.However,theROCKET-AFstudyismoreexcitingtostrokecliniciansbecause50%ofthestudypopulationhadpriorstrokewithTIAwhichmeansthattheyareahigher-riskpopulationwhiletheRE-LYstudyrecruitedabout20%oftheirpopulationwhohadpriorstrokewithTIA,asdidARISTOTLEandAVERROES.Aftermanyyearsofwrestlingwiththedifficultiesofwarfarin,itseemsquitelikelythatcliniciansnowhaveanembarrassmentofrichesinnew-generationoralanticoagulantsforAF.


Surgical Outcomes from Revascularisation Surgery for Moyamoya Disease: Experience in Tuen Mun Hospital, Hong Kong

KF Fok1, WL Poon2, YC Wong2, Florence Kwok3, Dawson Fong1

1 Department of Neurosurgery, Tuen Mun Hospital, Hong Kong SAR2 Department of Radiology, Tuen Mun Hospital, Hong Kong SAR3 Psycho-behavioural Unit, Tuen Mun Hospital, Hong Kong SAR

Moyamoyadisease(MMD)isararecerebrovasculardiseasecharacterisedbystenosisandocclusionofthebilateralinternalcarotidarteriesresultinginthecharacteristicdevelopmentofanabnormalnetworkintheareasofthearterialstenosis.Variousrevascularisationprocedureshavebeenusedinthetreatmentofthisgroupofpatients.Tostudytheclinicalcharacteristicsandlong-termoutcomesinthetreatmentofMMDinHongKong,weanalysedourresultatDepartmentofNeurosurgery,TuenMunHospital,HongKong. Dataobtainedin23patientswithMMD(meanage31years,range5-64years)managedinTuenMunHospitalbetween1995and2011wereanalysed.Nineofthemareinpaediatricagerange(age<18years).Nineteenpatientspresentedwithacutesymptom, threewithrecurrent transient ischaemicattack(TIA)andonewasdetectedincidentally.Amongthosewithacutepresentation,almosthalfofthem(52%)demonstratedacutecerebralhaemorrhageinneuroimaging,therestshowedcerebralinfarction.Only17%oftheseMMDpatientsshowednocerebralinsultintheneuroimagingonpresentation.ThediagnosiswasestablishedclinicallyandalsoconfirmedbyMRIandcerebralangiograminallpatients.Preoperativecerebralangiographyshowedthatthepatientsareinvariousstagesofdisease,butthemajorityofthem(20outof38hemispheres)wereinstage3ofSuzukiclassification.RecentlywealsoincludedCTperfusionstudy(CTP)withacetazolaminechallengeandneuropsychologicalassessmentinourpre-surgicalassessmentprotocol. Of the 23 patients, 18 received microsurgical revascularisation procedures. Seven patientsunderwentunilateraland11patientsbilateral revascularisation(30 treatedhemispheres included1repeatedrevascularisation).Thesurgicalprocedures includedencephaloduroarteriosynangiosis(EDAS)in15,encephalodurogaleoarteriosynangiosis(EDGAS)in3,combinedEDASandEDGASin9,encephaloduroarteriomyosynangiosis(EDAMS)in1,Burrholein1andSTA-MCAbypasswithEDGASin1patient.Therewasnomortalityinourseriesbutweexperiencedtwo(6.7%)postoperativecerebralinfarctiondevelopedoutof30hemispheres treated.Bothof themshowedworsenneurologybutimprovedwithconservativemanagement. Themeanfollow-upperiodforthese18patientsunderwentrevascularisationprocedurewas75.2months(range,1-154months).Postoperativecerebralangiogramperformedshowed90%offrontalgraftdevelopedcollateralformationachievinggradeAorBinMatsushimagradingscale(synangiosismorethanone-thirdfillingofanteriorcerebralarteryterritory),while55%ofEDASgraftshowedsimilargrading inmiddlecerebralartery territories.Duringthis follow-upperiod,onepatientdevelopedrecurrenthaemorrhage12yearsafterEDASprocedure.Nopatientinischaemicgroupdevelopednewinfarctonrevascularisedhemisphereinourpatientseries.Functionally,66%(12patients)demonstratedimprovementinmodifiedRankinScale(mRS)by1pointormore.TherestofthegroupshowedthesamemRSasinpreoperativestatus. Inconclusion,revascularisationsurgery inpatientswithMMDcarriesa lowrisk; it iseffectiveatpreventingfutureischaemiceventandimprovesqualityoflife.

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Surgical Management of Moyamoya Disease

KC WangSeoul National University Hospital, Korea

Moyamoyadisease(MMD)isthemostcommonpaediatriccerebrovasculardiseaseofsurgicalconcern. MMD with symptoms such as TIA, infarction, seizure, headache/orbital pain, haemorrhageis candidate of surgical treatment. For paediatric patients, indirect revascularisation methods,includingencephaloduroarteriosynangiosis (EDAS)usingsuperficial temporaloroccipitalarteries,encephalomyosynangiosis(EMS),encephalogaleo(periosteo)synangiosis(EGS,EGPS),multipleburrholetrephinationwithinsertionofgaleoperiostealflap,andtheircombination,arerecommended.Inadultcases,directrevascularisationmethods,suchassuperficialtemporalartery-middlecerebralartery(STA-MCA)anastomosis,with/withoutindirectrevascularisationmethods,arefrequentlyused.Directsurgeryismoreuseful inadultpatientswithMMDbecausetheindirectsurgery iseffectiveforrecruitmentofcollateralsonlyin40to50%ofadults, incontrasttothepaediatricpatientswhoshownearly100%improvementofCBFifextensiveinfarctionisabsent.Directanastomosisisfrequentlynotfeasibleinchildrenbecauseofsmallsizeandfragilityofthevessels. Accordingtotheresultsofhaemodynamicstudies,suchascerebralangiography,SPECT,PETandperfusionMRI,thebrainareasforrevascularisationareselected.Almostalwaysbothmotorcortexareasareincluded.WerecommendadditionofbifrontalEGS/EGPSforrevascularisationofanteriorcerebralartery(ACA)territories.Insomepatientswithischaemiaintheposteriorcerebralartery(PCA)territory,occipitalartery isusedforEDAS.SomecasesofunilateralMMD(probableMMD)aretreatedwithunilateralsurgery.Because25to35%ofthemshowprogressiontothebilateraldisease,theyshouldbemonitored.Stagedoperationsaredesignedaccordingtothepatient’ssymptom,haemodynamicdataandtherateofdiseaseprogression. Regardlessofthesurgicalmethod,postoperativegoodcollateralformationisfoundin80to90%ofthepatients.Patientswithpreoperativeextensivecerebralinfarctionshowpoorcollateralformationaftersurgery. Perioperative ischaemiccomplicationsarenotrare.Becauseof lackofvascularreserve inthesepatients,theyarevulnerabletoischaemiacausedbytheintraoperativeandpostoperativehypotension,anaemia,hypovolemiaandhypocarbia.CarefulpreservationofthecollateralchannelsandmeticulousmonitoringofthehaemodynamicfactorsareessentialfortheperioperativemanagementofMMD.Mostofthepostoperativeischaemiccomplicationsareevidentduringthefirstpostoperativeweek. Inpatientswithdirectrevascularisation,suddenincreaseofperfusionpressureintheischaemicbrainmaycausebraindamagebytemporaryhyperperfusion,especiallyinpatientswithpreoperativeprolongedprofoundischaemia. MMDpatientsyoungerthan3yearsfrequentlyshowrapidprogressionofdiseasetowidespreadcerebral infarction.Theoutcomemaybeadverselyaffectedbythepreoperativebilateral ischaemicdamage inthisage-group.Eventhoughtheyaremorevulnerabletoperioperative ischaemicbraindamagethanolderchildren,theoverallmanagementoutcomeisbetterincaseswithearlysurgery. ThereisnosolidevidencethatsurgeryisbeneficialincasesofMMDwithhaemorrhage,althoughithasareasonabletheoreticalbasis.Surgeryisfrequentlyrecommendedforthesepatients.

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S 25Mechanical Thrombectomy in Ischaemic Stroke: a Look at the Toolbox

Ferdinand HuiCleveland Clinic Foundation, United States

SincetheintroductionoftheMercifamilyofclotretrieversanddisruptiondevice,therehasbeenablossominginthenumberofdevicesdesignedtore-establishflowinthesettingofischaemicstrokerangingfromthrombectomydevices,aspirationdevices,stents,andstent-retrieverswithmanymoretocome.Efficacyandeaseofuseareprobablytheprimarydeterminantsofphysicianutilisation. ThispresentationwillreviewthemajordevicesavailableinNorthAmerica,EuropeandHongKong,compareandcontrastthemechanismsofaction,easeofusecharacteristics,andreviewsomeofthetipsandtricksassociatedwiththedevicesinquestion. Reviewwillalsoincludemajorintra-arterialtrialsandforthcomingtrialsthatwillbeofinterestinthefurtherdevelopmentofintra-arterialstrategies.


Medical Management of Intracranial Stenosis

Kazunori ToyodaDepartment of Cerebrovascular Medicine, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan

Atheroscleroticstenosisofintracranial largearteriesisanimportantstrokemechanismespeciallyforAsianpopulations.Intracranialatherosclerosiscausesstrokeduetothebranchocclusion,arterytoarteryembolism,andboth.Theintracranialstenosisisnotstatic,andmayprogressorregressinarelativelyshortperiodof time.Progressivestenosisof intracranialarteries is relatedto thedevelopmentofischaemicevents. InJapan,weoftenuseintravenousargatroban,adirectthrombininhibitor,aloneorwithantiplateletagents as acute antithrombotic therapy for atherothrombotic stroke patients with intracranialatherosclerosis.Arationaleofargatrobanuse inacuteatherothromboticstroke isderivedfromadomestictrialresults,but itsefficacyfor intracranialatherosclerosis isnotscientificallyestablished.Asanotherintravenousanticoagulant,theFISS-trisstudy1failedtoshowasignificantbenefitof low-molecular-weightheparinoveraspirininpatientswithacutestroke(<48h)withlargearteryocclusivedisease(mostlyintracranial).Forsubacutestroke/TIApatients(<90days)withintracranialarterystenosis,theWASIDtrial2showedhigherratesofadverseeventsandnobenefitofwarfarin(INR2.0-3.0)overaspirin.Thus,anticoagulationisnotaworld-standardstrategyforintracranialatherosclerosis. Dualantiplatelet therapy isapromisingchoice for intracranialatherosclerosis, although thecombinationofaspirinandthienopiridineisnotroutinelyrecommendedforgeneralstrokepatientsduetoincreaseinbleedingrisk.IntheCLAIRtrial,3combinationofclopidogrelwithaspirinwasmoreeffectivethanaspirinaloneinreducingmicroembolicsignalsinacutestroke/TIApatients(<7days)withpredominantlyintracranialarterystenosis.ProgressionofM1orbasilarstenosisduring6monthsafterstroke(<2weeks)wassignificantlylowerinpatientstreatedwithcilostazolplusaspirinthanthosetreatedwithaspirinaloneintheTOSS.4Recently,TOSS-25showedsimilarefficacyofpreventingprogressionofintracranialarterystenosisandnewischaemiclesionsbetweendualtherapyusingcilostazolandaspirinandthetherapyusingclopidogrelandaspirininacutestrokepatients(<2weeks). Statin,pioglitazone,andsomeantihypertensiveagentsareotherpromisingmedicaloptionforintracranialatherosclerosis; their therapeuticpowershouldbeproperlyevaluated.Angioplastyandstentingforsymptomatic intracranialarterystenosisremainscontroversial.Therecruitmentof theSAMMPRIStrialwasprematurelyterminatedbecauseofthehigher30-daystrokeanddeathratesaftertreatmentwithintracranialangioplastycombinedwithstentingusingtheGateway-Wingspansystemthanthoseafteraggressivemedicaltherapyalone.6Furtherstudies,andespeciallystudiesfromAsia,arerequiredtoobtainthebesttherapeuticstrategyinpatientswithintracranialatherosclerosis.

References1. Wong KS, Chen C, Ng PW, et al. Low-molecular-weight heparin compared with aspirin for the treatment of acute ischaemic

stroke in Asian patients with large artery occlusive disease: a randomised study. Lancet Neurol 2007;6:407-13.2. Chimowitz MI, Lynn MJ, HOwlett-Smith H, et al. Comparison of warfarin and aspirin for symptomatic intracranial arterial

stenosis. N Engl J Med 2005;352:1305-16.3. Wong KS, Chen C, Fu J, et al. Clopidogrel plus aspirin versus aspirin alone for reducing embolisation in patients with acute

symptomatic cerebral or carotid artery stenosis (CLAIR study): a randomised, open-label, blinded-endpoint trial. Lancet Neurol 2010;9:489-97.

4. Kwon SU, Cho YJ, Koo JS, et al. Cilostazol prevents the progression of the symptomatic intracranial arterial stenosis: the multicenter double-blind placebo-controlled trial of cilostazol in symptomatic intracranial arterial stenosis. Stroke 2005;36:782-6.

5. Kwon SU, Hong KS, Kang DW, et al. Efficacy and Safety of Combination Antiplatelet Therapies in Patients With Symptomatic Intracranial Atherosclerotic Stenosis. Stroke 2011 Jul 28. Epub ahead of print.

6. Leung TW, Yu SC, Wong KS. Have medical therapy and stenting been fairly compared? A repercussion upon termination of recruitment in the SAMMPRIS trial. Int J Stroke 201;6:312-4.

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Off-label Use of Rituximab in Refractory Neurological Autoimmune Diseases—Experiences of a Regional Teaching Hospital in Hong Kong

Vincent HL Ip, Alexander YL Lau, Anne YY Chan, Lisa WC Au, Florence SY Fan, Nick NC Ng, Edward HC Wong, Yannie OY Soo, Vincent CT Mok, Thomas WH Leung, Lawrence KS WongDivision of Neurology, Department of Medicine and Therapeutics, Prince of Wales Hospital, The Chinese University of Hong Kong, Hong Kong SAR

Background:Rituximabisachimericmouse-humanmonoclonalantibodyagainsttheproteinCD20whichispresentonthesurfaceofallB-celllineagesexceptforthestemcells,pro-Bcellsandnormalplasmacells.BecauseoftheincreasingawarenessoftheroleofB-cellinthepathogenesisofvariousautoimmuneneurologicaldiseases,thereareincreasingoff-labelprescriptionsofrituximabfortheseseriousdiseasessuchasrelapsingmultiplesclerosis(MS),neuromyelitisoptica(NMO)andmyastheniagravis(MG).However,therearenorandomisedcontrolledtrialsinstudyingtheseoff-labelusesofrituximab.Wereporteightcaseswhereoff-labeluseofrituximabinseveralrefractoryneurologicalconditionsatthePrinceofWalesHospitalinHongKong,atertiaryteachinghospitalofTheChineseUniversityofHongKong.Methods:Patientswhohavereceivedrituximabasanoff-labelindicationwereidentifiedfromtheMSregistries,aswellasfromrecallbyindividualphysicianwhohasuseditinotherconditionssuchasNMOandMG.Datawereretrievedretrospectivelyfromtheelectronicpatientrecord(ePR)betweenAugust2008andJuly2011.Informationincludingthediagnosis,clinicalpresentations,concurrentmedicationsandprevioustreatments,dosagesandregimensofrituximabused,side-effectsexperiencedandclinicalprogresswererecorded.Results:Eightpatientsreceivedrituximabasoff-labeluseduringtheperiodaudited.IndicationsincludedprimaryprogressiveMS,NMOandMG.Twoofthecasesaremalepatients.Rituximabwasusedmainlyasmaintenancetherapyinallcasesexceptinonecaseinwhichitwasusedasanacuteinductiontherapyinoneepisode.Themostcommonprescribeddosageis2000mgpercoursewithregimenof1000mggivenbi-weekly.Coursesoftreatmentgivenrangedfrom1to5,andthetimeintervalbetweencoursesrangedfrom5to11months.Therewerenoreportedseriousinfusionreactionandnonotableadverseeventsassociatedwithrituximabuseduringtheaboveauditedperiod.Conclusions:Overall,theoff-labeluseofrituximabappearstobesafeandpromisingresultscanbeseenincasesofNMOandMG.

Cisplatin Neuropathy: a Prospective Study on Chinese Patients

KF Ko, WY Lau, WK Cheng, MC Kwan, LK Yip, KP YiuDepartment of Medicine and Geriatrics, Kwong Wah Hospital, Hong Kong SAR

Background: Cisplatin is a frequently used anti-cancer drug, and with inclusion of this drug incombinationchemotherapy,highremissionratesfordifferenttumourshavebeenachieved.However,itstherapeuticuseislimitedbyadose-dependentandpredominantlysensory,peripheralneuropathy.Methods: Inthisstudy,theincidenceofthiscomplicationwasevaluatedin19Chinesepatientstreatedwithcisplatin120mg/m2every4weeks.Patientswereexcludediftheywereover70yearsold,withahistoryofpreviouschemotherapy,diabetesmellitus,alcoholabuse,brainandleptomeningealinvolvement,AIDSandimpairedliverorrenalfunction.Thepatientswereclinicallyandelectrophysiologicallytestedbeforeandat3,6,9and12monthsaftertheinitiationofchemotherapy.Results: None of the 19 patients examined before treatment showed symptoms or signs orelectrophysiologicalevidenceofneuropathy.Atthecumulativedoseof360mg/m2,12(63%)outofallthepatientsdevelopedsensoryneuropathy.However,onepatientatthecumulativedoseof1080mg/m2hadnoclinicalorelectrophysiologicalevidenceofneuropathywhatsoever,whereasatthecumulativedoseof1440mg/m2,allthesevensurvivorsdevelopedsensoryneuropathyofvaryingseverity.Despitesubstantialinvolvementofsensorynerves,therewasbarelyanychangeinmotornerveconduction.Ototoxicityduetodamageoftheeighthnervewasdetectedineight(42%)patients.Implications/Conclusions: Cisplatin-inducedneuropathyischaracterisedbyasensoryneuropathy.Ithasbeenproposedthatlargemyelinated1afibresarederanged,probablyatthelevelofdorsalrootganglioncells.Surfacerecordingispreferredtothenearneedleelectroderecording,whichhasbeenshowntobemoresensitiveindetectingSAPsofsmallamplitude.

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A Lady with Autoimmune Lymphocytic Hypophysitis Presenting with Right 3rd, 4th and 6th Palsies

SH LiDepartment of Medicine, North District Hospital, Hong Kong SAR

A52-year-oldladywithgoodpasthealthwasadmittedtohospital inJuly2011becauseofacuteonsetofdiplopiaworstonlookingtotheleftanddroopyrighteyelid.Shedidnothaveheadacheorotherfocalneurologicalsymptom.Examinationrevealedright3rd,4th,and6thpalsies.Pupilsandvisualfield(confrontationtest)wereunremarkable.OtherexaminationsincludingENTwerenormal. HerRFT,LFT,glucose,CBP,CRPandCXRwerenormal.ESRwas60mm/h.CTbrainscanrevealedasellarmass(Fig1).ContrastCTBshowedthickenedpituitarystalk(0.6cm), intensehomogenouslycontrastenhancingmassinvolvingthehypothalamus,infundibulumstalkandpituitarygland.Neithererosionofthesellarfloornorabnormalleptomeningealenhancementwasnoted.CircleofWilliswasnormal(Figs2and3). Immunemarkers,syphilisserology,tumourmarkerswerenegative.Pituitaryhormoneconfirmedpanhypopituitarism(includingcranialdiabeticinsipidusandhyperprolactinemia).CSFshowedslightlyraisedprotein(0.6g/L),normalglucose,mildlyelevatedwbc(8x106/l, lymphocyte90%)butnegativeGram’sstain,AFBsmear,indianink,culture,cytology,andMTBPCRDNA. Theclinicalandradiologicalfindingsandlaboratoryresultswerehighlysuggestiveofautoimmunelymphocytichypophysitis(AIH).Intravenoushydrocortisone100mgq8hwasinitiatedforsecondaryhypoadrenalism,followedby2weeksoforalprednisolone60mg/dayandsubsequenttaperingforAIH.Rapidimprovementincranialnervepalsieswasnotedwithinfewdays.Hydrocortisone,DDAVPandlaterthyroxineweregiven.ContrastMRIbrain/pituitary6daysaftersteroid(Fig4)showedthickenedpituitarystalk(0.4cm),enlargedpituitaryglandwithlossofT1hyperintensesignalofposteriorpituitaryandhomogenouscontrastenhancement.Featurescouldrepresentlymphocytichypophysitis. Thepatientwasdischargedwithalmostcompleteneurologicalrecovery.Follow-upassessmentandMRIwillbeperformed.Herendocrinestatewillbemonitored.

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Fig 1. CT brain, plain Fig 2. Contrast CT brain (coronal)

Fig 3. Contrast CT brain (sagittal)

Fig 4. MRI contrast (coronal)


Long-term Outcome of Stroke Thrombolysis in Hong Kong Chinese Patients

Edward WongDepartment of Medicine and Therapeutics, Prince of Wales Hospital, Hong Kong SAR

Background:Emergingdatahavedemonstratedthesafetyandefficacyofstrokethrombolysisinorientalpopulations.Long-termbenefitwasalsoobservedinCaucasianpopulations.However,dataonlong-termoutcomeofChinesestrokepatientswhoreceivedthrombolysisremainlimited.Methods:Clinicalrecordandstrokeregistrydataofconsecutive ischaemicstrokepatientsreceivedintravenousthrombolysiswithin4.5hoursorintra-arterialthrombolysiswithin6hoursbetweenJanuary2007andAugust2010atourcentrewerereviewed.ModifiedRankinScale (mRS)scoresrecordedprospectivelyat3monthswerecomparedtothatat12monthsandatmostrecentclinicfollow-up.Results:Atotalof60patientswereidentified;42receivedintravenousthrombolysisand18underwentintra-arterialthrombolysis.Themeanagewas71.7±11.8yearsandNationalInstituteofHealthStrokeScalescorewas16.5±6.7.Twenty-five(42%)patientsattainedfavourableoutcomeofmRSscore≤2(iefunctional independence)at3months.Therewere11(18%)mortality.Theproportionofpatients(45%)withmRSscore≤2wassustainedat12months.Siximprovedandfiveworsenedbetween3and12months.Atthemostrecentfollow-upatameanof22.5±9.1months,19(32%)patientshaddeceasedbuttheproportion(42%)withmRSscore≤2remainedstatic.Conclusion:The3-monthoutcomeofstrokethrombolysis inourgroupof localpatientsappearedsustainablelongterm.After3months,asmallproportioncontinuestoimproveinfunctionalstatus,whichwasoffsetbypatientswhodeterioratedorsuccumbedduetoadvancedageandcomorbidities.

Do Cerebral Microbleeds Increase Intracranial Haemorrhage Risk in Hong Kong Stroke Patients Receiving Thrombolytic Therapy?

Edward Wong1, Alexander Lau1, Yannie Soo1, Deyond Siu2, Jill Abrigo2, Tom Cheung2, Venus Hui1, Lawrence Wong1

1 Department of Medicine and Therapeutics, Prince of Wales Hospital, Hong Kong SAR2 Department of Imaging and Interventional Radiology, Prince of Wales Hospital, Hong Kong SAR

Background:Cerebralmicrobleeds (MBs)onT2*-weightedmagneticresonance imaging (MRI)areassociatedwithincreasedintracranialhaemorrhage(ICH)risk.Still,strokethrombolysisisregardedassafeforpatientswithMBsasrecentCaucasianstudiesdemonstratedsmallbutinsignificantincreaseinICHrisk.WeaimedtoinvestigateifthrombolysisisequallysafeforlocalChinesepatientswithMBs.Methods:Consecutive ischaemicstrokepatientsreceivedintravenousor intra-arterial thrombolysisbetweenJanuary2007andJune2011withMRIperformedwithin72hourswererecruited.Thepresence,location,andnumberofMBswererecorded.PrimaryoutcomeswereanyICHandsymptomaticICHbyECASS(EuropeanCooperativeAcuteStrokeStudy)definition.Results:Atotalof54patientsintravenousand24intra-arterialthrombolysispatientswereidentified:46ofthemhadMRIperformedwithin72hours;45wereincludedasoneMRIwasaffectedbymotionartefact.MBsweredetectedinnine(20%)patients.Sevenhad1MB,onehad2MBs,andonehad6MBs.NineMBsweresubcorticalandsixwerecortical.One(11%)of9patientswithMBshadasymptomaticICHatdifferentsitesfromMB,versus6(17%)of36patientswithout(P=1.00).NonehadsymptomaticICH.Forreference,among33patientswithnooruninterpretableMRI,10hadICHofwhichfiveweresymptomatic.Conclusion:MBsdidnotappeartoincreaseICHriskfollowingstrokethrombolysisinthisgroupoflocalpatients.However,ourresultsshouldbeinterpretedwithcautionasMRIwasnotuniformlyperformed.UnderestimationofbothprevalenceofMBsandICHriskcouldnotberuledout.

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Intravenous Thrombolysis Versus Intra-arterial Recanalisation Therapy for Acute Ischaemic Stroke Due to Large Artery Occlusion—Comparison of Outcomes from a Hong Kong Hospital

Edward Wong1, Simon Yu2, Alexander Lau1, Joyce Hui2, Deyond Siu2, Colin Graham3, Cecilia Leung1, Venus Hui1, Lawrence Wong1, Thomas Leung1

1 Department of Medicine and Therapeutics, Prince of Wales Hospital, Hong Kong SAR2 Department of Imaging and Interventional Radiology, Prince of Wales Hospital, Hong Kong SAR3 Department of Accident and Emergency Medicine, Prince of Wales Hospital, Hong Kong SAR

Background:Forischaemicstrokepatientswithlargearteryocclusionandhighclotburden,recanalisationrateislowdespiteintravenoustissueplasminogenactivator(IVTPA).Intra-arterialtherapy(IAT)hasbeenproposedasanalternativebutrandomisedtrialdatacomparingtoIVTPAislacking.HerewecomparedtheoutcomesofourpatientstreatedwitheitherIATorIVTPA.Methods:BetweenJanuary2007andMay2011,consecutivepatientswithlargearteryocclusion(ICA,MCAM1/M2,VA,BA)treatedwithIAT,withonset-to-IAT-at-clottime≤4.5hourswereidentified.FavourableoutcomewasdefinedasmodifiedRankinScale(mRS)score≤2,andmortalityratesat3monthswerecomparedtopatientswithlargearteryocclusiontreatedwithIVTPAatsimilartime-frame.Results:ThirteenpatientstreatedwithIAT≤4.5hourswereidentified.Meanonset-to-IATtimewas209±38minutes(range,150-260minutes).Forcomparison,13receivedIVTPAbetween2.5and4.5hours(mean179±19,range157-230minutes).Therewerenostatisticallysignificantdifferencesinbaselinecharacteristicsofage,NIHSSscoreandCT-ASPECTSbutIVTPAgroupweretreatedearlier(P=0.02).At3months,7(54%)ofIATand5(38%)ofIVTPAgroupattainedfavourableoutcome(P=0.69).Mortalityratewas23%inIATversus8%inIVTPAgroup(P=0.59).Conclusion:NostatisticallysignificantdifferenceswerefoundinfavourableoutcomeandmortalityrateforoursmallgroupofpatientstreatedwithIATorIVTPA.LargerprospectiverandomisedtrialisneededtoseeifIATissuperiortoIVTPAforthispatientsubgroup.Inthemeantime,IATshouldbereservedforpatientswithcontra-indicationtoIVTPA.

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The Efficacy of Deep Brain Stimulation for Advanced Parkinson’s Disease

YF Cheung1, HF Chan1, TL Poon2, PMP Choi2, WS Chan1, FC Cheung2, HM Chan1, P Li11 Department of Medicine, Queen Elizabeth Hospital, Hong Kong SAR2 Department of Neurosurgery, Queen Elizabeth Hospital, Hong Kong SAR

Background:Deepbrainstimulation (DBS) isawell-established treatmentmodality foradvancedParkinson’sdisease(PD)patientswhosesymptomsarerefractorytomedical therapyalone.QueenElizabethHospital(QEH)isoneofthelocalpubliccentreswhichprovidesthisservicesinceJuly2000.Methods:WestudiedtheefficacyofDBSinPDpatientsover11yearsbyrecordreview.Results:UptoJune2011,28patientsreceivedDBSinQEH.Subthalamicnucleuswastargetedinallbutonepatients(whoreceivedthalamicventralisintermediusnucleus[Vim]stimulationononesideandSTNstimulationontheother).Eleven(39%)weremale.Themean(±standarddeviation)ageofonsetandsurgerywere43.2±8.9and55.2±7.9,respectively.Threepatientshadalreadydied,tworelatedtodiseaseprogression(pneumonia)andonecommittedsuicidebyinsecticidepoisoning(5yearsaftersurgery).Webelieveoneofthesepatientswhodiedofpneumoniaactuallysufferedfrommultiplesystematrophy,butwhenhereceivedDBStheclassicalsignshadnotyetbeenmanifested(excludedfromtheremaininganalysis). Baselineand1-yearpostoperativeUnifiedParkinson’sDiseaseRatingScale(UPDRS)scoreswereavailablein25and18patientsrespectively,asshowninthefollowingtable.TheimprovementsofUPDRS-IIand-IIIwerestatisticallysignificant.

Significantreductionofanti-Parkinsonianmedicationswasalsoachieved(levodopaequivalentdailydose1090±622mgto587±382mg,P<0.001,pairedsamplesT-test).Tenpatientshadtheirimplantablepulsegenerator (IPG)replacedduetobatteryrun-out.Themedianbattery lifewas7.1years (95%confidenceinterval,5.1-9.0years,Kaplan-Meierestimates).Conclusion:DBS isproventobeefficacious inourcohort. Italleviatessymptomswith feweranti-Parkinsonianmedications.BatterylongevityisanessentialelementwithasignificantimpactontheneedofIPGreplacementandhealtheconomy.

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UPDRS-I UPDRS-II UPDRS-III

Baseline (OFF-med) 4.0 ± 2.7 18.3 ± 6.3 32.7 ± 14.5One-year post-op (OFF-med, ON-stim) 3.8 ± 1.8 12.3 ± 6.3 16.8 ± 9.1P value (paired samples T-test) 0.7 0.001 <0.001


A Case with Atypical Neuroleptic Malignant Syndrome

OC Lau, BH Fung, PW Ng, CH Lo, CK Chan, KY CheungDepartment of Medicine and Geriatrics, United Christian Hospital, Hong Kong SAR

Case reportA22-year-oldmanwithmildmentalretardation,schizophrenia,anxietyneurosisandepilepsy,receivingquetiapine350mgdailyandvalproate400mgBD.Thepatientwasfoundagitatedinworkplace,quarrelledwithworkmatesandquittedthejobfinally.Hethenexperiencedunstablemoodathomeassociatedwithpalpitation,diarrhoeaandreducedoralintakeforrecentfewdays. HeattendedAccidentEmergencyDepartment(AED)for5timeswithin76hoursandjointconsultationwithpsychiatristsatthattimerevealedanxious-lookingpatientwithgeneralisedfinetremors.Duringhis5thAEDattendance,hewasnotedtohaveabodytemperatureof37.5°C.Hewasgivenintramuscularinjectionof5mghaloperidol inviewofunderlyingunstablemoodandsuspectedhallucination.Hewasadmittedtomedicalwardanddevelopedlead-piperigidity,mutism,diaphoresisandsialorrhoea.Furtherdrugcountdiscoveredpatientmissing400mgofquetiapineandpossibilityofdrugoverdosecouldnotbeexcluded.Vitalsignsshowednormalbloodpressurewithsinustachycardiaupto200+bpm.Temperatureraisedupto39°ClaterthesamedayofadmissionwithdecreaseinGCStoE4V1M5.Laboratoryinvestigationshowedmildleukocytosis(WBC16.3x109 /L)andcreatinekinase(CK)levelraisedupto2535IU/L.CTbrainandCSFanalysisdidnotrevealanyabnormality. Allpsychotropicmedicationswerestopped,supportivetreatmentwithbromocriptine, lorazepamandmadopargiven.Cyproheptadinewasempiricallystartedtocoversuspectedserotoninsyndrome.Hisconditiongraduallyimprovedandmusclerigiditysubsided.CKlevelpeakedat8080IU/Londay7andlaternormalised.However,thepatientremainedmuteandtransferredtothepsychiatricwardforfurthermanagement.

DiscussionWith thecommonuseofatypicalneuroleptics, theprevalenceofatypicalneurolepticmalignantsyndrome(whichisamildervariantofNMS)isincreasing.OurcasemaybeacaseofatypicalNMStobegin,withsubsequentdeteriorationtotypicalNMSbytheconcurrentinjectionofhaloperidol.

Headache Associated with Chest Pain and ECG Abnormalities: What Can It Be?

Raymond CK Chan, CH Lo, PW NgDepartment of Medicine and Geriatrics, United Christian Hospital, Hong Kong SAR

Case reportA60-year-oldman,withahistoryofischaemicheartdisease,wasadmittedforconstrictingchestpainandbilateraldullheadache.Thereweremildexertionaldyspnoea,dizzinessandnausea.Physicalexaminationwasunremarkable.ECGshowedQwaveinleadIII,STsegmentdepressionandTwaveinversioninV4-6.Itwastreatedasischaemicheartdiseasewithantiplatelet.Cardiacenzymescamebacknormal.Nextdaymorning,hesuddenlydevelopedgeneralisedtonic-clonicseizure.UrgentCTbrainshowedsubarachnoidhaemorrhage.Cerebralangiogramrevealedanelongatedaneurysmmeasuring2.5mmx5mminthejunctionofleftA1-A2andanteriorcommunicatingartery.Extraventriculardrainageandembolisationofaneurysmweresuccessfulbutheremainedcomatosewithpoorneurologicalrecovery.Severecerebraloedemasecondarytovasospasmdevelopedandhedied5dayslater.

DiscussionThediagnosisofcerebralaneurysmwasmaskedbycardiacsymptomandECGchanges.ItisknownthatthecardiogenicpresentationofSAHspansfromECGabnormalitiestoelevationofcardiacenzymesandregionalwallmotionabnormalities.ThesemanifestationsareduetothesurgeofcatecholaminereleaseaccompanyingSAH.STsegmentabnormalitieswerereportedin15to51%ofSAHandSTdepressionwasmorecommoninpatientswithpooroutcome. Headachewasapredominantsymptominmyocardialischaemiainanumberofcasereports.Inthe2004InternationalClassificationofHeadache,theentity“Cardiaccephalalgia”wasclassifiedinthegroupofsecondaryheadachesattributedtodisorderofhomoeostasis.Itcouldmimicmigraine,tension-typeheadacheorothertypesofheadachesthatcanhardlyclassified. Thiscaseillustratedtwotalesintwosystems.ECGabnormalitiescouldrepresentprimarychangesinmyocardialischaemiaoroccursecondarytoSAH.Headachemaybesignifyingunderlyingintracranialpathologyorsolelythepredominantmanifestationincardiaccephalgia.AttentionshouldbepaidtodifferentiatethecardiacandneurologicalcausesofheadachewithECGchangesandneuroimagingshouldbeconsideredindividually.

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AUTHOR INDEXPageNo.

AJAbrigo 37LWCAu 35

BCBazil 24

CAYYChan 11,35CKChan 39CKMChan 19DTMChan 11HFChan 11,38HMChan 11,38LTChan 11RCKChan 39WSChan 38YCChan 11YHChan 12PPChen 28ACOCheng 19WKCheng 35FCCheung 38GLYCheung 17KYCheung 16,39TCheung 37YFCheung 11,38PMPChoi 38ACPChow 18JMTChu 21YPChu 12

FFSYFan 10,35KFFok 31DFong 31KWFong 11MKFong 12WCFong 11BHFung 39

GCGraham 10,38

HFHui 33JHui 38VHui 10,37,38

IVHLIp 35MIsmail 11

KKFKo 35JKoo 30MCKwan 35FKwok 31

LECLai 22JLam 25

AYLLau 10,35,37,38CLau 11KKLau 12KYLau 11OCLau 39WYLau 35CLeung 10,38TWHLeung 10,35,38PLi 11,38SHLi 36CHLo 39WTLo 11CNPLui 21

MWMak 17BLMan 19KYMok 25,26VCTMok 11,35TMüller 21,22

NNNCNg 35OTWNg 21PWNg 39YWNg 11

PSYYPang 13TLPoon 38WLPoon 31WWSPoon 11

SBSheng 12EShum 10DSiu 10,37,38KPSiu 35YOYSoo 10,35,37

TKToyoda 34THTsoi 29

VDGVossler 23

WKCWang 32SJWang 27AHYWong 11EHCWong 10,35,37,38EWong 11KKWong 14LKSWong 10,35,37,38RWong 11WTWong 12YCWong 31

YESWYeung 15JHMYeung 11

PageNo. PageNo.

LKYip 35KPYiu 35SYu 38LYPYuen 26NYuki 18KKLYung 21

ZDSZee 20XLZhu 11

Acknowledgements

TheOrganisingCommitteewouldliketoextendtheirgratitudetothefollowing

sponsors(inalphabeticalorder)fortheircontinuingsupport:

AllerganHKLtd

BayerHealthcareLtd

BiogenIdec

BoehringerIngelheimHKLtd

Eisai(HK)CoLtd

GlaxoSmithKline

IPSEN(HK)

JanssenPharmaceuticaHongKong

MedtronicInternationalLtd

MerckPharmaceuticalHKLtd

MerckSharp&Dohme(Asia)Ltd

NovartisPharmaceuticals(HK)Ltd

OrientEuropharmaCoLtd

OtsukaPharmaceutical(HK)Ltd

PfizerCorporationHKLtd

SanofiBMSHongKong

ServierHKLtd

Lastbutnotleast,wewouldliketothankallspeakers,chairmen,presentersand

participantsfortheirparticipationandcontribution.

2nd hong kong neurological congress 24th annual … committee of the 2nd hong kong neurological...

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