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Page 1: Neurologic Disease Approach Handbook封面
Page 2: Neurologic Disease Approach Handbook封面

《內容》

1. Steps in the diagnosis of neurologic disease

2. Neurologic history taking i) Pain ii) Headache iii) Seizure disorder iv) Sleep disorder

3. Physical examination of neurological patients

4. Screening neurological examinations 5. Anatomic Localization 6. Etiology

1 1 3 4 8 16 20 21 22 30

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1. Steps in the diagnosis of neurologic disease 2. Neurologic history taking ◆ Positive symptoms:如抽筋、肌鍵反射增強,可能是神經元的活動性增加、自發性神經軸放電或抑制功能喪失。

◆ Negative symptoms:如肌無力、感覺喪失等,可能是神經元的活動性喪失、神經軸或神經肌肉突觸的傳導障礙。

◆ Suggested order of inquiry about each symptom 1. Character(Quality) 2. Severity(Intensity) 3. Location and extension:unilateral,bilateral,dermatome distribution,depth from the

surface,radiation,extent of diffusion,and paths of reference 4. Time relationships(Temporal factor)

甲、Date and time of onset:morning,afternoon,evening,midnight,sleep 乙、Mode of onset:

i. Abrupt/sudden:seconds to minutesàVascular,Traumatic,Seizure ii. Acute:minutes to days(polyneuropathy:days to weeks,

<4wks)àVascular,Infectious,Metabolic

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iii. Subacute:days to weeks(polyneuropathy,weeks to months,4wks to 2months)àInfectious,Inflammatory or demyelinating,Neoplastic

iv. Chronic:months to years(polyneuropathy,months to years,>2 months)àNeoplastic,Degenerative

v. Gradual 丙、Age of onset 丁、Frequency or periodicity 戊、Duration:seconds,minutes,days,weeks,years,persistent 己、Course:stationary,improving,rapidly or slowly progressive,fluctuated,

paroxysmal,episodic,remittent,intermittent,regressive 5. Associated complaints/symptoms:aura or prodrome,vomiting,deafness,tinnitus,

perspiration,prostration,body weight loss,fatigue,malaise,palpitation,chest discomfort,abdominal pain,dyspnea,visual disturbance(scotomas,diplopia,blurring vision,hemianopia),hyperacusis,hyperosmia,photophobia,phonophobia,tenderness,vertigo,syncope,aphasia,drowsiness,hallucination,illusions,tongue biting,incontinence,limb twitching,fever,chills,cough,rhinorrhea,sputum,dysuria,impotence,preictal,ictal,and postictal symptoms

6. Aggravating(provoking, precipitating) and alleviating(relieving) factors:physical activity or exercise,weather,caffeine,insomnia,stress,mense,alcohol,medication,posture(stand, sit, lie down, stooping), walking,neck movement,head position,rest,

sleep,food,coughing,mental effort,use of the eyes 7. Previous treatment and effects

◆ 病史詢回記憶法:AIOPQRST A Accompanying(Associated) symptoms

I Ictus(preitcal, ictal, and postictal phases) Iatrogenic Intensity

O Onset P Precipitating factor Q Quality(nature)

R

Relieving factor Risk factor Radiation Review of system

S Site Severity(intensity)

T Temporal factors(onset, duration, frequency, progression)

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◆ Specific inquiry about some common neurologic problems 1. Pain: 甲、Character:sharp,dull,aching,burning,tingling,lancinating,prickling

i. Neuropathic pain(Disease of the nervous system):sharp electric-like flashes(lancinating), burning or “pins and needles”(paresthesia),squeezing,sewelling,itching or prickling,allodynia,hyperalgesia 1. Dysesthetic pain: 甲、Burning,prickling,itching,and occasionally,lancinating 乙、typically located distally in the dermatomal distribution of the

nerve and may be elicited by percussion over the injuried nerve(Tinel’s sign)

2. Nerve trunk pain: 甲、Dull,aching,or gnawing pain 乙、More proximaly,stimulates the nociceptive afferents that supply

the epineurium(nervi nervorum) ii. Nociceptive pain(Disease of visceral or somatic structures):

iii. Idiopathic pain(sensation without a definite organic cause) 乙、Location:dermatomal distribution of a specific peripheral nerve,plexus,root,

or central sensory pathway,glove-and-stocking distribution,radiating pain 丙、Intensity:pain scale[0(no pain)~10(worst pain)],interference of sleep,work and

daily activity 丁、Time relationship:

i. Time of onset:nightàdiabetic polyneuropathy,nerve root lesions 戊、Associated symptoms:Weakness,Reflex changes,hyperesthesia,

hyperalgesia,allodynia,hyperpathia,sensory deficit,autonomic dysfunction 己、Aggravating factors:Valsalva maneuver(cough,strain,sneezing),position

change,spinal motion,chin-chest maneuver,horizontal position,taking,eating,chewing,swallowing,taste of food,trigger point

庚、Relieving factors:upright position(nerve root lesion)

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2. Headache: 甲、Character:Dull,Pulsatile(throbbing)(vascular headache),stabbing,jabbing,

or ice-pick head pains,tightness,pressure i. Subarachnoid hemorrhageàexplosive

ii. Cluster headacheàsevere,boring,and steady,like a hot poker iii. Migraineàthrobbing(pulsatile) iv. Tension-type headacheàtightness,pressure,viselike,bandlike 乙、Location:Unilateral,localized or generalized

i. Frontal regions:sinusitis,supratentorial lesions,glaucoma,thrombosis of the vertebral or basilar artery,pressure on the tentorium,or increased intracranial pressure,meningitis

ii. Occipitonuchal region:posterior fossa lesions,meningitis,cervical spine disease,dissection of vertebral artery

iii. Ear pain:disease of ear,referred pain from throat,cervical muscles,spine,posterior fossa lesion

iv. Periorbital and supraorbital pain:local disease,dissection of the cervical portion of the internal carotid artery

v. Vertex of biparietal regions:sphenoid or ethmoid sinus disease or thrombosis of the superior sagittal sinus

vi. Migraineàoften unilateral(frontotemporal) but may be bilateral vii. Cluster headacheàunilateral,most commonly localized behind or in the eye

or in the temporal region(orbitotemporal) viii. Tension-type headachesàgeneralized or localized to the frontal or to the

occipitonuchal regions ix. Meningeal irritation:generalized,or bioccipital or bifrontal x. Brain tumor:unilateral or generalized

xi. Temporal arteritis:Unilateral or bilateral,usually temporal xii. Lesions of paranasal sinuses,teeth,eyes, and upper cervical

vertebrae(referred to forehead or maxilla or around the eyes) 丙、Intensity:

i. Pain scale[0(no pain)~10(worst pain)],interference of sleep,work and daily activity

ii. Most intense cranial pain:meningitis,subarachnoid hemorrhage,migraine,cluster headache,tic douloureux

丁、Time relationship: i. Mode of onset:

1. Sudden(thunderclap-like):subarachnoid hemorrhage ii. Time-intensity curve

1. Thunderclap-like(expolosive):head pain that peaks in an instant,thunderclap-like manner:subarachnoid hemorrhage,primary

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thunderclap headache 2. Peak over 3 to 5 minutes,remain at maximal intensity for about 45

minutes:Cluster headache 3. Rapidly or build up over hours, maintained for several hours to days:

migraine iii. Duration:

1. Short-lasting Unilateral Neuralgiform headache attacks with Conjunctival injection and Tearing(SUNCT):5~240 seconds

2. Primary cough headache:1 second to 30 minutes 3. Paroxysmal hemicrania:2~30 minutes 4. Primary exertional headache:5 minutes to 48 hours 5. Hypnic headache:lasts ≥15 minutes after waking(不一定要符合) 6. Cluster headache:15-180 minutes if untreated,一般 30~45 min 7. Tension-type headache:Headache lasting from 30 minutes to 7 days 8. Primary thunderclap headache:lasting from 1 hour to 10 days 9. Migraine:4-72 hours(一般 4~24hours) (untreated or unsuccessfully

treated) 10. Status migrainosus:>72 hours

iv. Time of onset: 1. Upon awakening or later in day:migraine 2. Usually nocturnal,1~2h after falling sleep,occasional diurnal:cluster

headache 3. At any time of the day or night,interrupt sleep,worse in the morning

on awakening:brain tumor 4. Upon awakening:migraine,brain tumor,sinusitis,cervical spine

disease(after a period of inactivity,first movements of the neck are stiff and painful),chronic pulmonary failure with hypercapnia(early morning headache)

5. Nocturnal awakening:cluster headache,brain tumor(少數),hypnic headache,migraine(較少)

v. Frequency or periodicity: 1. Migraine:Irregular intervals,weeks to months 2. Cluster headache:Nightly or daily(at predictable times) for several

weeks to months 3. Tension headache:one or more periods of months to years

vi. Age and sex of onset: 1. Adolescents,young to middle-aged adults,sometimes children,more

common in womenàmigraine 2. Adolescent and adult males(90%)àcluster headache 3. Mainly adults,both sexes,more in womenàtension headache

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4. Over 50 years,either sex:temporal arteritis 戊、Associated symptoms:

i. Nausea,vomiting,photophobia,phonophobia,diarrhea,prodromal symptoms(hunger,scintillating lights,visual loss,scotoma,psychologic change,weakness,somatosensory disturbance,aphasia)àmigraine

ii. Neck stiffness on forward bending,Kernig and Brudzinski signsàMeningeal irritation(meningitis,subarachnoid hemorrhage)

iii. Tearing,conjunctival injection, nasal stuffiness,miosis and ptosis(oculosympathetic paresis or partial Horner’s syndrome)àcluster headache

iv. Jaw claudication,tenderness of the scalp,scalp nodule,polymyalgia rheumaticaàTemporal arteritis

v. Amenorrhea,galactorrheaàpolycystic ovary syndrome or a prolactin-secreting pituitary adenoma

vi. Red eyeàGlaucoma,ophthalmic disease vii. Dark green purulent nasal exudatesàSinusitis

viii. Taking,eating,chewing,swallowing,taste of food,trigger pointàNeuralgia ix. Unexpected forceful(projectile) vomitingàbrain tumor,IICP 己、Aggravating factors:

i. Physical activity,fatigue,tension ii. Eye movement

iii. Sexual intercourse iv. Bending,lifting,cough,strain,Valsalva maneuver àbenign cause or brain

tumor v. Bright light,noise,menses,excessive sleep,sleep deprivation,ingested

substances(hot,cold,or sweet food,alcohol,red wine,caffeine,味精monosodium glutamate (MSG)等),pungent odors,hunger,weather change

vi. Lying down(supine posture):chronic subdural hematoma and some tumors(especially those in the posterior fossa),pseudotumor cerebri

vii. Orthostatic headache:post-lumbar puncture headache,spontaneous low-pressure headache

庚、Relieving factors: i. Massageàtension-type headache

ii. Gentle compression of the carotid or superficial temporal artery on the painful sideàmigraine

iii. Darkness,sleep,pregnancyàmigraine

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ICHD(The International Classification of Headache Disorders), 2nd ed

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3. Seizure disorders: 甲、Character:What kind of seizure(from patient and witness)?

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The "Proposed Diagnostic Scheme for People with Epileptic Seizures and Epilepsy(ILAE Commission Report 2001)" contains five axes Axis 1: Ictal phenomenology - from the Glossary of Descriptive Ictal Terminology can be used to describe ictal events with any degree of detail needed. (P9 Table 1 & P10 Table2) Axis 2: Seizure type - from the List of Epileptic Seizures. Localization within the brain and precipitating stimuli for reflex seizures should be specified when appropriate.(P9 Table2 & P12 Table3) Axis 3: Syndrome - from the List of Epilepsy Syndromes, with the understanding that a syndromic diagnosis may not always be possible.(P13 Table 4) Axis 4: Etiology - from a Classification of Diseases Frequently Associated with Epileptic Seizures or epilepsy syndromes when possible, genetic defects, or specific pathological substrates for symptomatic focal epilepsies. Axis 5: Impairment - this optional, but often useful, additional diagnostic parameter can be derived from an impairment classification adapted from the WHO ICIDH-2.

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ILAE 1998 Semiological seizure classification(表徵發作分類)(http://www.neuro.org.tw/magpdf/13-3P136.PDF)

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乙、Time relationship:

i. Time of onset and Age of onset ii. Frequency and Duration

iii. Course 丙、Associated symptoms:

i. automatism, prodrome(aura), alternation of consciousness, tongue biting, incontinence, sustained postures, presence of myoclonus, post-ictal behavior, postictal phenomena(post-ictal confusion, Todd's post-epileptic paralysis, postictal stupor, sensory loss, aphasia, hemianopia, headache, postical amnesia等), trauma

ii. fever, neck stiffness, headache, migrainous aura, body weight loss, cold sweating, nausea, light-headedness, faintness等

丁、Provoking factors:insomnia, sleep deprivation, alcohol or drug(BZD,heroin, Marijuana, antiepileptic drug等) administration, visual stimulation(flickering light, patterns and others), thinking, music,eating, praxis, somatosensory stimulation, proprioceptive stimulation, reading, hot water, startle,hyperventilation, relationship to menstruation or pregnancy, and fever等

戊、Previous treatment and effects 己、Past history:abnormal gestation, trauma, old CVA, febrile seizure, mental

retardation, meningitis, encephalitis, brain abscess, and autoimmune disease等 庚、Neurological examination:

i. Focal signs ii. Asymmetry of the hand or face

iii. Phakomatoses(neurocutaneous disorders):café-au-lait spots, facial angioma, conjuctival telangiectasia, hypopigmented macules, fibroangiomatous nevi, or lumbosacral shagreen patches等

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4. Sleep disorders: 甲、Overview of a typical night’s sleep:regular vs irregular sleep-wake cycle,

premorbid sleep pattern, 可以自己安排時正常的上床和睡醒的時間 i. Sleep initiation:什麼時候上床?上床前在作什麼?什麼時候關燈?花

多久才能入睡?睡覺的姿勢是怎麼樣? ii. Sleep maintenance:睡著後醒來幾次?有沒有被什麼原因吵醒 [如:

external noise, restless sleep partner, snoring, dyspnea, cramps, other pain, indigestion, dreaming, leg kicking, sweating, need to urinate(和醒過來之後再去上廁所不同)]?醒過來多久才再睡著?醒過來後作些什麼(worry, clock-watch, remain in bed or get up)?估計總共睡了多久?

iii. Sleep termination:什麼時候醒來?自然睡醒或是鬧鐘叫醒?醒來覺得神清氣爽(refreshed)還是疲倦(tired)?有沒有 early morning headache, dry mouth, sore throat, blocked nose, or confusion?

乙、Specific nocturnal symptoms i. Respiration:睡覺時睡幾個枕頭?半夜會喘嗎?會打鼾嗎(snoring)?打

鼾多久了?有多嚴重?有多大聲?什麼姿勢最厲害?病人知道自己打

鼾嗎?睡著時是否會停止呼吸?停止多久?會有 nocturnal stridor嗎? ii. Movements:睡著時是否會有什麼動作?會不會打傷枕邊人或自己?和

作夢有關嗎?什麼時間會出現動作?會磨牙 (bruxism)嗎?是否會sleepwalking or sleep terrors?睡覺時會 talk, shout or scream嗎?

iii. Miscellaneous:是否會有 sleep paralysis或 hallucinations before sleep or on awakening?

丙、Daytime symptoms i. General:病人有工作和沒有工作時白天的活動是什麼?有沒有什麼時

間覺得比較清醒(late at night or early in the morning?) ii. Excessive daily sleepiness:會覺得白天很想打瞌睡嗎?在什麼情況下和

多久後較容易想打瞌睡 (如 working at a desk,waiting for an appointment,sitting in a conference,religious service,or theater,relaxing with friends,talking to a single person,eating,watching television,reading,driving)?是否因打瞌睡而發生事故?是否有打盹(naps)的習慣?打盹完會比較有精神嗎?

iii. Cataplexy:會有猝倒的症狀嗎?(spells of weakness with emotions,

包括 laughter, fear, excitement, pleasure,和 sadness),會有小發

作嗎?像是臉或下巴下垂(sagging of the face, drooping of the jaw)

或是軟腳(buckling of the knees) 丁、Background history

i. Psychosocial:有精神病史嗎?特別是 depression和 anxiety disorders,是否有什麼壓力?是否被虐待(abuse)?家庭狀況?是否抽煙,喝酒,咖啡?是否有其他藥物濫用?是否運動?是否有足夠的時間曬太陽?

ii. Medical:是否有高血壓,心肺疾病或神經系統疾病?(特別是 epilepsy or

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neurodegenerative diseases)?是否過胖?是否有 sexual dysfunction? iii. Family History:是否有 snoring,obstructive sleep apnea syndrome,

insomnia,excessive sleepiness,narcolepsy,restless legs syndrome,sleepwalking or sleep terrors,epilepsy,psychiatric disease,或 substance abuse的家族史?

戊、Examination of the patient with a sleep complaint: i. Obstructive sleep apnea syndrome:oropharynx(tonsils, uvula, soft palate和

anteriorposterior and lateral diameters),the jaw(retrognathia),the size of tongue,external nares,neck

ii. Height,weight,blood pressure,pulmonary,cardiac,neurologic,or psychiatric examination

The International Classification of Sleep Disorders

1. Dyssomnias (disorders of initiating and maintaining sleep and disorders of excessive sleepiness) A. Intrinsic sleep disorders 1. Psychophysiological insomnia 2. Sleep state misperception 3. Idiopathic insomnia 4. Narcolepsy 5. Recurrent hypersomnia 6. Idiopathic hypersomnia 7. Posttraumatic hypersomnia 8. Obstructive sleep apnea syndrome 9. Central sleep apnea syndrome 10. Central alveolar hypoventilation syndrome 11. Periodic limb movement disorder 12. Restless legs syndrome 13. Intrinsic sleep disorder NOS B. Extrinsic sleep disorders 1. Inadequate sleep hygiene 2. Environmental sleep disorder 3. Altitude insomnia 4. Adjustment sleep disorder 5. Insufficient sleep syndrome 6. Limit-setting sleep disorder 7. Sleep-onset association disorder 8. Food allergy insomnia 9. Nocturnal eating (drinking) syndrome 10. Hypnotic-dependent sleep disorder 11. Stimulant-dependent sleep disorder 12. Alcohol-dependent sleep disorder 13. Toxin-induced sleep disorder 14. Extrinsic sleep disorder NOS C. Circadian rhythm sleep disorders 1. Time zone change (jet lag) syndrome 2. Shift work sleep disorder 3. Irregular sleep-wake pattern

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4. Delayed sleep phase syndrome 5. Advanced sleep phase syndrome 6. Non-24 hour sleep-wake disorder 7. Circadian rhythm sleep disorder NOS 2. Parasonmias (disorders that primarily do not cause a complaint of insomnia or excessive sleepiness) A. Arousal disorders 1. Confusional arousals 2. Sleepwalking 3. Sleep terrors B. Sleep-wake transition disorders 1. Rhythmic movement disorder 2. Sleep starts 3. Sleep talking 4. Nocturnal leg cramps C. Parasomnias usually associated with REM sleep 1. Nightmares 2. Sleep paralysis 3. Impaired sleep-related penile erections 4. Sleep-related painful erections 5. REM sleep-related sinus arrest 6. REM sleep behavior disorder D. Other Parasomnias 1. Sleep bruxism 2. Sleep enuresis 3. Sleep-related abnormal swallowing syndrome 4. Nocturnal paroxysmal dystonial 5. Sudden unexplained nocturnal death syndrome 6. Primary snoring 7. Infant sleep apnea 8. Congenital central hypoventilation syndrome 9. Sudden infant death syndrome 10. Benign neonatal sleep myoclonus 11. Other Parasomnia NOS 3. Sleep Disorders Associated with Medical/Psychiatric Disorders A. Associated with mental disorders 1. Psychoses 2. Mood disorders 3. Anxiety disorders 4. Panic disorder 5. Alcoholism B. Associated with neurological disorders 1. Cerebral degenerative disorders 2. Dementia 3. Parkinsonism 4. Fatal familial insomnia 5. Sleep-related epilepsy 6. Electrical status epilepticus of sleep 7. Sleep-related headaches C. Associated with other medical disorders

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1. Sleeping sickness 2. Nocturnal cardiac Ischemia 3. Chronic obstructive pulmonary disease 4. Sleep-related asthma 5. Sleep-related gastroesophageo reflux 6. Peptic ulcer disease 7. Fibrositis syndrome 4. Proposed sleep disorders These are the disorders for which insufficient information is available to confirm their acceptance as definitive sleep disorders. 1. Short sleeper 2. Long sleeper 3. Subwakefulness syndrome 4. Fragmentary Myoclonus 5. Sleep hyperhidrosis 6. Menstruation-associated sleep disorderly 7. Pregnancy-associated sleep disorder 8. Terrifying hypnagogic hallucinations 9. Sleep-related neurogenic tachypnea 10. Sleep-related laryngospasm 11. Sleep choking syndrome Source: American Sleep Disorders Association: International Classification of Sleep Disorders: Diagnostic and Coding Manual. Rochester, Minn.: American Sleep Disorders Association, 1990.

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3. Physical examination of neurological patients

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4. Screening neurological examinations ◆ Mental status:Partially assessed while asking the history ◆ Cranial nerves: △ I:Not tested △ II: ★ Gross visual acuity each eye ★ Visual field by confrontation ★ Fundoscopy

△ III,IV,VI: ★ Lateral and vertical eye movement ★ Pupillary response to light

△ V: ★ Pinprick sensation on face

△ VII: ★ Close eyes,show teeth

△ VIII: ★ Whisper voice each ear

△ IX,X: ★ Palate lifts in midline,gag reflex

△ XI: ★ Shrug shoulders

△ XII: ★ Protrude tongue

◆ Each limb separately △ Tone △ Power of main muscle groups △ Coordination,finger-nose and heel-shin tests △ Tendon reflexes △ Plantar responses △ Pinprick and light touch on hands and feet △ Double simultaneous stimuli on hands and feet △ Joint position sense in hallux and index finger △ Vibration sense at ankle and wrist ◆ Gait ◆ Romberg’s test ◆ Soft sign:很難決定是否正常或異常的 sign,如左右兩邊臉頰之鼻唇溝稍微不對稱,兩側手腳對針刺感覺稍微不一樣,舌頭伸出來時稍微歪向一邊等

◆ Hard sign:確定異常的 sign ◆ MCMCRSG(Mental status, Cranial nerves, Motor, Coordination, Reflexes, Sensory, Gait

and station)

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5. Anatomic Localization The presenting complaint

Mental Complaint

(1) Consciousness:awareness(confusion and delirium vs dementia),alertness(drowsiness,stuporous,and coma)àhemisphere,thalamus,brainstem,diffuse cerebral disease

(2) Behavioral abnormality:psychomotor activity(agitation versus abulia) and emotional response(elation,sadness,anger or flattening)àFrontal lobe,limbic system or psychogenic

(3) Language problem:aphasia(Broca’s,Wernicke’s,conduction,transcortical,global),alexia,agraphiaàDominant hemisphere

(4) Memory problem:dementia,amnesiaàPapez circuit,temporal lobe,thalamus

(5) Higher cognitive function:abstract reasoning,insight,judgment,calculations,Visuospatial ability,praxis,thought form(flight of ideas等),thought content(paranoia,delusions,compulsions等),perceptions(hallucination or illusions),behavior,affect and mood ◆ Personality(disinhibition),initiative(apathy,abulia,akinetic,

indifferrence,sparse verbal output),cognition(inattention,poor abstract thought,poor judgment,impersistnece和perseveration),emotional lability,incontinent,motor aphasia,apraxiaàFrontal lobe

◆ Alexia,Agraphia,Acalculia,Left-right disorientation,constructional or dressing apraxia,topographic agnosiaàParietal lobe

◆ Amnesia,Sensory aphasiaàTemporal lobe ◆ Prosopagnosia,Color agnosiaàOccipital lobe

Motor Complaint (1) Weakness(-plegia vs –paresis, hemiplegia, quadriplegia, paraplegia, monoplegia, neck weakness, weakness of cranial muscles) ◆ Upper motor neuron system (Pyramidal):Increase tone,

spasticity and hyperreflexia ☆ Cortex:cortical function, differential weakness of limbs and

face ☆ Corona radiate:differential weakness of limbs and face ☆ Internal capsule:一般較 homogenous ☆ Brain stem(midbrain,pons,medulla):crossed sign,brainstem

sign, combined cranial nerve palsy and long tract sign(motor or sensory tract),unilateral or bilateral weakness

☆ Spinal cord:sensory level,sphincter function ◆ Lower motor neuron system:Decreased tone,flaccidity and

hyporeflexia

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☆ Anterior horn(Lower motor neuron)(fasciculation) or brain stem motor nucleus

☆ Root/plexus ☆ Peripheral nerve(fasciculation:rare) or cranial motor nerve ☆ Neuromuscular junction(fluctuated, fatigability and diurnal

change, muscle tone and tendon reflex一般是 normal) ☆ Muscle

◆ Psychogenic (2) Involuntary movements or coordination disturbance

◆ Extrapyramidal system(tremor, chorea, athetosis, akinesia, akathisia, dystonia, dyskinesia, ballismus, asterixis, tic, torticollis, hemifacial spasm, parkinsonism, muscle stiffness, myoclonus等)

◆ Cerebellum(HANDS[Hypotonia, Hand tremor(intention tremor), Ataxia(limb, trunk, ocular), Nystagmus, Dysdiadochokinesia, Dysmetria, Scanning flexible speech)

(3) Speech disorder:hypophonia,dysarthria ◆ Upper motor neuron:spastic dysarthria ◆ Cerebellar:ataxic dysarthria ◆ Extrapyramidal system:hypokinetic or hyperkinetic dysarthria ◆ Lower motor neuron(V,VII,X,XII):Flaccid dysathria

(4) Gait disorder ◆ Sensory and lower motor gait disorders(Lower sensorimotor

dysfunction):如 steppage gait(proprioception),vestibular ataxia(labyrinthine sense),visual ataxia(vision),waddling gait(musculoskeletal system)

◆ Simple gait disorders of central origin(Middle sensorimotor dysfunction):Upper motor neuron system, extrapyramidal system, cerebellum(spastic, cerebellar ataxic, hemiplegic, paraparetic, parkinsonian, dystonic, hemiballistic, choreic gaits)

◆ Complex gait disorders of central origin(Higher sensorimotor dysfunction):cautious gait,magnetic gait(frontal gait disorder, apraxia of gait),disequilibirum and disorganized gait(frontal disequilibrium),isolated gait ignition failure等

(5) Gaze disorder(Pursuit,Saccade,conjugate gaze palsies,disconjugate gaze palsies,Skew deviation,Nystagmus) ◆ Supra-nuclear control:frontal eye field & occipital lobe ◆ Brain stem connections: ◇ Gaze center:vertical gaze center, convergence center(midbrain),

lateral gaze center(paramedian pontine reticular

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formation[PPRF]) ◇ Vestibular system,cerebellum ◇ MLF(medial longitudinal fasciculus) ◇ Ocular Motor Nucleus(CN III,IV,VI nucleus) ◆ Ocular Motor Nerve(CN III,IV,VI) ◆ Ocular Muscle ◆ Associated sensory input:vision,auditory processing,vestibular

system (6) Swallowing disorder

◆ Cortical swallowing control:inferior precentral and the posterior inferior frontal gyri

◆ Swallowing center:medulla ◆ Cranial nerve V,VII,IX,X,XII ◆ Swallowing muscle(tongue, oropharynx, esophagus) ◆ Swallowing Phase:Oral phase(Oral preparatory phase,Oral

transit phase),Pharyngeal phase,Esophageal phase (7) Disorders of respiration ◆ Voluntary act of breathing:descending pathways from the motor

and premotor cortex ◆ Respiratory center:pons and medulla ◆ Phrenic nerve(C3,4,5) and intercostals nerve(thoracic spinal

cord) ◆ Respiratory muscle:diaphragm, intercostal and abdominal

muscles, accessory muscles of respiration in the neck(cervical spinal cord)

◆ Vocal cord(recurrent laryngeal nerve) ◆ Respiratory drive(CN IX):chemoreceptors(carotid body[pH] and

ascending aorta[hypoxia]) ◆ Stretch receptors in the lung(CN X)

Sensory Complaint

(1) Anatomic distribution(superficial[light touch, pain, temperature],deep[joint sense, deep pain, vibration],cortical sensation[two-point discrimination, traced-figure identification, stereognosis, double simultaneous stimulation]) ◆ Peripheral nerve or branch ◆ Root/Plexus ◆ Spinal Cord(Anterior column[spinothalamic tract], Posterior

column[posterior tract]) ◆ Brain stem(medulla,pons,midbrain):crossed sign,brainstem

sign ◆ Thalamus

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◆ Internal capsule ◆ Corona radiate ◆ Cortex

(2) Nonanatomic(body concept) distribution ◆ Hysteria ◆ Malingering ◆ Psychosis

Special Complaint

(1) Visual disturbance ◆ Visual filed defects ◇ Monocular visual loss ★ Refraction problem:corneal, lens ★ Retinal lesions ★ Optic nerve lesions(Optic disk and retrobulbar optic nerve

lesions) ★ Glaucoma

◇ Binocular visual loss ★ Optic chiasm ★ Optic tracts ★ Thalamus(Lateral geniculate body) ★ Optic radiations ★ Calcarine cortex ★ Migraine

◆ Diplopia ◇ Refraction problems:retinal detachment, dislocated lens,

keratoconus ◇ Oculoparesis ★ Ocular muscle cause ★ Neuromuscular junction cause ★ Cranial nerve[CN III,IV,VI]) cause ★ CNS cause:brainstem lesions, Wernicke’s syndrome

◇ Factitious ◆ Anisocoria ◇ Iris disease(sphincter muscle and dilator fibers) ◇ Parasympathetic fibers:ciliary ganglion, CN III,

Edinger-Westphal nucleus ◇ Sympathetic fibers:hypothalamus, brainstem, cervical spinal

cord, anterior roots of C8, T1~3, the cervical sympathetic trunk, postganglionic pathway

◆ Unusual visual syndromes ◇ Anton’s syndrome,Balint’s syndrome,Bonnets’ syndrome,

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Dyschromoatopsia,Pallinopsia,Prosopagnosia (2) Auditory disturbance ◆ Hearing loss ◇ Conductive deafness:external auditory meatus,tympanic

membrane,auditory ossicle ◇ Sensorineural deafness:Cochlea,cochlear nerve and nuclei,

central auditory pathways(pons and medulla的 ventral and dorsal cochlear nucleuiàSuperior olivary complexàlateral lemniscusàinferior colliculusàmedial geniculate bodyàauditory cortex)

(3) Olfactory disturbance ◆ Anosmia,dysosmia:Olfactory receptorsàOlfactory

nerveàOlfactory bulbàOlfactory tractàMedian and lateral olfactory striaeàprimary olfactory cortex(piriform lobe of the temporal cortex)àamygdaloid nucleus, septal nuclei和hypothalamus

(4) Gustatory disturbance ◆ 舌前 2/3味覺(主要 CN VII,可能會經由 CN V的 mandibular

division):lingual nerveàchorda tympaniàgeniculate ganglionàsolitary nucleiàmedial lemniscusàthalamusà tongue-face area of the postrolandic sensory cortex

◆ 舌後 1/3味覺(主要 CN IX,少數 CN X):glossopharyngeal nerve and ganglion nodosumàthe nucleus of the tractus solitariusà medial lemniscusàthalamusàtongue-face area of the postrolandic sensory cortex

(5) Autonomic dysfunction ◆ Supranuclear regulatory apparatus of the autonomic nervous

system:1.frontal lobe cortex 2. insular cortex 3. Amygdaloid and adjacent nuclei(limbic system, visceral brain)

◆ Hypothalamus(前副交感,後交感) ◆ Thoracolumbar(Sympathetic) Outflow ◆ Craniosacral(Parasympathetic) Outflow

Others (1) Headache (2) Vertigo:Peripheral(vestibular labyrinthine disease),

Central(dysfunction of the vestibular connections),Systemic causes(如endocrine,metabolic disease等)

(3) Sleep disorder (4) Syncope (5) Epilepsy (6) Pain

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Localization in the Upper Motor Neuron(Pyramidal) System Principle:Tone is increased,causing spasticity and hyperreflexia Site Symptoms Signs Cortex ● Differential weakness of limbs

and face ● Sensory symptoms ● Language, visual, or attentional

alterations

● Fractionated weakness(e.g., arm greater than face and leg)

● Aphasia, hemianopia, or hemineglect

● Cortical and primary sensory loss ● Cognitive dysfunction

Corona radiate ● Differential weakness of limbs and face

● Fractionated weakness ● Primary sensory loss

Internal capsule ● Weakness only ● Face, arm and leg affected equally and densely

Brain stem ● Unilateral or bilateral weakness ● Diplopia, vertigo, dysarthria, or

dysphagia

● Dense hemiparesis ● Ocular or oropharyngeal weakness

Spinal cord ● Diffuclty with gait ● Difficulty walking ● Urinary incontinence

● No face involvement ● Spastic quadriparesis (cervical) or

paraparesis(thoracic) ● Sensory level

Localization in the Lower Motor Neuron System

Principle:Tone is decreased,causing flaccidity and hyporeflexia Site Symptoms Signs Anterior horn ● Progressive flaccid weakness

● Wasting, weakness, fasciculations ● No sensory loss

Root/plexus ● Single limb weakness and sensory loss

● Pain in the neck, back, or limb

● Weakness in radicular/plexus distribution

● Electromyogram(EMG) shows denervation in affected muscles

Nerve ● Focal weakness (mononeuritis) ● Distal weakness

(polyneuropathy)

● Focal or distal weakness ● Atrophy in affected distribution ● Fasciculations ● Hyporeflexia ● Slowing or low amplitude on

conduction studies; denervation on EMG

Neuromuscular junction

● Fluctuating weakness ● Diplopia

● Positive edrophonium test ● Decremental response with

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repetitive stimulation on EMG Muscle ● Proximal weakness

● Difficulty climbing stairs and brushing hair

● Muscle aches

● Proximal weakness ● Normal nerve conduction ● Polyphasic, low-amplitude motor

units on EMG

Localization in the Brain Stem Principle:Specific cranial nerve involvement guides localization Site Signs and Symptoms Midbrain ● Impaired vertical gaze

● CN 3 palsy( plus contralateral abduction nystagmus suggest ipsilateral internuclear ophthalmoplegia[INO]

● CN4 palsy ● Contralateral motor signs(hemiapresis suggests Weber’s syndrome;

ataxia suggests Claude’s syndrome; tremor or chorea suggests Benedikt’s syndrome

● Alerations in consciousness, perception, or behavior(peduncular hallucinosis)

Pons ● Dysarthria and dysphagia ● Contralateral hemiparesis or hemisensory loss ● Ipsilateral facial sensory loss(CN 5) ● Ipsilateral gaze palsy(paramedian pontine reticular formation[PPRF] or

one-and-a-half syndrome(PPRF and median longitudinal fasciculus[MLF])

● Locked-in syndrome(bilateral basis pontis; associated with ocular bobbing)

● Horizontal nystagmus(often brachium pontis) ● Ataxis

Pontomedullary junction

● Vertigo(CN 8) ● Dysarthria ● Horizontal or vertical nystagmus ● Contralateral hemisensory loss and hemiparesis

Lateral medulla(Wallenberg) syndrome

● Ipsilateral Horner’s syndrome ● Ipsilateral limb ataxia ● Ipsilateral face and contralateral body numbness ● Gait ataxia ● Vertigo, dizziness, nauses(CN 8) ● Dysphagia(CN 9, CN 10, and CN 12 palsies)

Medial ● Contralateral hemiplegia

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medulla(rare) ● Contralateral posterior column sensory loss ● Ipsilateral tongue weakness(CN 12 palsy)

Localization in the Spinal Cord

Principle:Localization is assited by the combination of tracts involved Site Signs and Symptoms Common Causes Hemicord (Brown-Séquard’s syndrome)

● Ipsilateral hemiparesis ● Contralateral spinothalamic sensory loss ● Ipsilateral dorsal column sensory loss ● Sphincter dysfunction

● Penetrating trauma ● Extrinsic cord

compression

Anterior cord ● Upper and lower motor paralysis ● Spinothalamic sensory loss ● Sphincter dysfunction ● Sparing of posterior columns

● Anterior spinal artery infarction(often involves T4 to T8)

Central cord ● Paraparesis ● Lower motor paralysis; wasting and

fasciculations in arms ● Sensory loss in “shawl” distribution( if in

cervical region)

● Syringomyelia ● Neck flexion-extension

injury ● Intrinsic tumor

Posterior cord ● Proprioceptive and vibratory sensory loss ● Segmental tingling and numbness ● Sensation of constricting “ bands”

● Vitamin B12 deficiency ● Demyelination(multiple

sclerosis) ● Extrinsic compression

Foramen magnum ● Spastic quadriparesis ● Neck pain and stiffness ● C2 to C4 and upper facial numbness ● Ipsilateral Horner’s syndrome ● Ipsilateral tongue and trapezius muscle

weakness

● Tumor(meningioma, chordoma)

● Atlantoaxial subluxation

Conus medullaris ● Lower sacral saddle sensory loss(S2 to S5) ● Sphincter dysfunction; impotence ● Aching back or rectal pain ● L5 and S1 motor deficits(ankle and foot

weakness)

● Intrinsic tumor ● Extrinsic cord

compression

Cauda equine ● Sphincter dysfunction ● Paraparesis with weakness in the

distribution of multiple roots ● Sensory loss in multiple bilateral

dermatomes

● Extrinsic tumor ● Carcinomatous

meningitis ● Arachnoiditis ● Spinal stenosis

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6. Etiology VITAMINS VITAMINSDDE V(Vascular) I(Infection) T(Traumatic) A(Autoimmune/inflammatory) M(Metabolic/toxic) I(idiopathic/degenerative/inherited/intracranial pressure/iatrogenic) N(Neoplastic/nutrition) S(Seizure/Structural/pSychiatric)

V(Vascular) I(Inflammation) T(Trauma) A(Apoplexy) M(Metabolic) I(Infection) N(Neoplasm) S(pSychogenic) D(Drug/Toxin) D(Degeneration) E(Epilepsy)

V(vascular): (1) Ischemia:

A. Cerebral infarction(emobolic, atherothrombotic,lacunar, others),Transient ischemic attack,VBI(Vertebrobasilar insufficiency)

B. Retinal ischemia,Central retinal artery occlusion(CRAO),Ischemic optic neuropathy(AION&PION)

C. Spinal cord ischemia:Brown-Sequard’s(hemicord) syndrome,Central cord syndrome,Anterior spinal artery syndrome

(2) Hemorrhage: A. Intracerebral hemorrhage,Subarachnoid hemorrhage B. Epidural or subdural hematoma

(3) Others(structural anomaly, trauma, and hereditary disease, etc):Arteriovenous malformation,Venous sinus thrombosis,Amyloid angiopathy,Hypertensive encephalopathy,Carotid or vertebral artery dissection,Moyamoya disease(progressive intracranial large artery occlusion),Migraine,Cholesterol emboli syndrome,vasodepressor syncope,CADASIL(Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy)

I(infectious): (1) Brain & meninges:Meningitis(bacterial,viral,fungal,TB,syphilis or rickettsial),

Encephalitis(herpes simplex encephalitis , HIV encephalitis or other viral encephalitis,etc),neurosyphilis,Lyme disease,or Abscess(bacteria,fungal,or parasitic[eg. Toxoplasmosis, cysticercosis),prion disease,progressive multifocal leukoencephalopathy,subacute sclerosing panencephalitis

(2) Spine and cord:Spinal epidural abscess,spinal tuberculosis(Pott’s disease),Vertebral osteomyelitis,HTLV-1 myelopathy,Poliomyelitis

(3) Infectious neuropathy

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(4) Infectious myopathies:trichinosis,toxoplasmosis,cysticercosis,influenza virus,rubella virus,coxsackievirus,echoviruses,and mycoplasma,HIV myopathy

(5) Others:Systemic infection(eg. urosepsis or pneumonia),sinusitis,Whipple’s disease,Tetanus

T(traumatic): (1) Head injury:Epidural,Subdural,intracerebral,or subarachnoid hemorrhage,diffuse

axonal injury,concussion,carotid cavernous fistulae: (2) Spinal injury:HIVD, compression fracture, cervical spondylotic myelopathy (3) Cranial nerve or peripheral nerve injury (4) Post-traumatic sequela:posttraumatic(postconcussion) headache,dementia pugilistica,

post-traumatic epilepsy,posttraumatic pain syndrome (5) Others:Radiation injury,Electrical and lightning injury,Decompression Sickness

A(autoimmune/inflammatory disorders): (1) Autoimmune disorder/inflammatory disorders:Systemic lupus erythematosus,Central

nervous system(CNS) vasculitis,autoimmune vasculitis or cerebritis,rheumatoid arthritis,Sjögren’s disease,Behçet’s disease,temporal arteritis,Sarcoidosis,Inflammatory myopathies(polymyositis,dermatomyositis,inclusion body myositis,sarcoidosis),Sydenham chorea,Myasthenia gravis

(2) Demyelinating disorders:如 multiple sclerosis,Guillaine-Barre’s sydnrome,Acute demyelinating encephalomyelitis[ADEM],Central pontine myelinolysis or extrapontine myelinolysis,Marchiafava-Bignami Disease

(3) Immunologic or paraprotein-mediated diseases A. AIDP,CIDP,Paraneoplastic disease,Multiple myeloma,Anti-myelin-associated

glycoprotein antibody-mediated disease,Amyloidosis,Lymphoma with paraprotein,Monoclonal gammopathy,Cryoglobulinemia,Collagen vascular disease,Sarcoidosis,Waldentrom’s macroglobulinemia

M(metabolic/toxic): (1) Global hypoxia-ischemic(shock) (2) Electrolyte or acid-base disorders(pH disturbances,hypo- or hypernatremia,hypo- or

hypercalcemia,hypomagnesemia,hypo- or hyperglycemia) (3) Temperature disorder(hyper- or hypothermia) (4) Organ system dysfunction

A. Liver[hepatic encephalopathy,hyperammonemia] B. Kidney[uremia,uremic encephalopathy] C. Thyoid[hyperthyroidism[thyrotoxicosis] or hypothyroidism[myxedema] D. Parathyroid disease E. Adrenal[hyper- or hypoadrenalism]) F. Lung[CO2 narcosis,hypoxia]

(5) Fluid disturbance:Dehydration (6) Nutrition:Nicotinic acid(pellagra),Vitamine E,Thiamine(Wernicke-Korsakoff

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syndrome,beriberi),Folic acid,Vitamin B6(optic neuritis) or Vitamin B12(Subacute combined degeneration) deficiency

(7) Toxic: A. Drug intoxication or withdrawal(ethanol(EtOH) toxication or EtOH withdrawal,

other drugs including cocaines,phencyclidine,amphetamines,methanol,lithium等)

B. Heavy metal poisoning(lead,arsenic,mercury,thallium) C. Organic and industrial solvents:carbon disulfide,n-hexane,acrylamide,methyl-N-butyl

ketone D. Carbon monoxide exposure E. Botulism F. Toxic myopathies:medications,Critical illness myopathy,Neuroleptic malignant

syndrome,malignant hyperthermia (8) Endocrine myopathies:thyroid disease,Cushing’s disease(hyperadrenalism) or

parathyroid disease I(idiopathic/degenerative/inherited/intracranial pressure/iatrogenic): (1) Idiopathic:BPPV(Benign paroxysmal positional vertigo),Meniere’s disease,Idiopathic

epilepsy,Transient global amnesia(TGA),Primary headache disorders(Tension headache,Migraine headache,Cluster headache,Paroxysmal hemicrania,Trigeminal neuralgia,Occipital neuralgia(cervical osteoarthritis)

(2) Degenerative:Alzheimer’s disease,Huntington’s disease,Parkinson’s disease,Lewy body disease,Pick’s disease,Progressive supranuclear palsy(PSP),amyotrophic lateral sclerosis-parkinsonism-dementia complex of Guam

(3) Inherited/hereditary/genetic A. Chromosomal diseases:Trisomy 21(Down syndrome),Prader-Willi syndrome,

Angelman Syndrome B. Mitochondrial DNA disorders:

i. Mitochondrial encephalomyopathies:MELAS(Mitochondrial encephalomyopathy with lactic acidosis and stroke),MERRF(myoclonic epilepsy with ragged-red fibers),PEO(Progressive external oophthalmoplegia),KSS(Kearns-Sayre syndrome),NARP(Neuropathy,Ataxia,and Retinitis Pigmentosa),LHON(Leber hereditary optic neuropathy),Leigh syndrome,Alpers syndrome

C. Inherited metabolic disease i. Disorders of amino acid metabolism:

1. Disorders of amino-acid metabolism:Phenylketonuria,Maple syrup urine disease,Defects in the metabolism of sulfur amino acids(homocystinuria)

2. Disorders of amino-acid transport:Lowe Syndrome,Hartnup disease ii. Disorders of purine metabolism:Lesch-Nyhan syndrome

iii. Lysosomal and other storage diseases:

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1. Lipidoses:GM2-Gangliosidoses,GM1-Gangliosidoses,Fabry disease,Gaucher disease,Nieman-Pick disease,Farber disease,Wolman disease,Refsum disease,Cerebrotendinous xanthomatosis,Neuronal ceroid lipofuscinoses(Batten disease)

2. Leukodystrophies:Metachromatic leukodystrophy,Krabbe leukodystrophy(Globoid cell)

3. Mucopolysaccharidosis:Hurler syndrome(MPS I),Hunter syndrome(MPS II),Sanfilippo syndrome(MPS III),Morquio syndrome(MPS IV),Maroteaux-Lamy syndrome(MPS VI)

4. Mucolipidoses:Sialidoses,Galactosialidosis,Salla disease(Free sialic acid storage diseases),Fucosidosis,Mannosidoses,Aspartylglycosaminuria,Mucolipidoses II~IV

iv. Disorders of carbohydrate metabolism: 1. Glycogen storage diseases 2. Lafora disease and other polyglucosan storage diseases

v. Glucose Transporter protein syndrome vi. Hyperammonemia

vii. Peroxisomal diseases:Adrenoleukodystrophy(ALD),Zellweger syndorme,and Refsum disease

viii. Organic acidurias:Amino acid metabolism(Maple syrup urine disease),Krebs cycle(Fumaric aciduria),Fatty acid metabolism

ix. Disorders of metal metabolism 1. Hepatolenticular degeneration(Wilson disease) 2. Menkes disease(Kinky hair disease)

x. Acute intermittent porphyria D. Neurological syndromes with acanthocytes

i. Abetalipoproteinemia ii. Neuroacanthocytosis

iii. McLeod syndrome E. Cerebral degenerations of childhood

i. Spongy degeneration of the nervous system(Canavan disease),Infantile neuroaxonal dystrophy,Hallervorden-Spatz disease,Pelizaeus-Merzbacher disease,Alexander disease,Cockayne syndrome

F. Diffuse sclerosis G. Neurocutaneous disorders

i. Neurofibromatosis ii. Encephalotrigeminal angiomatosis(Sturge-Weber-Dimitri syndrome)

iii. Incontinentia pigmenti iv. Tuberous sclerosis

H. Hereditary myopathies:

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i. Muscular dystrophy(Duchenn’s muscular dystrophy,Becker’s muscular dystrophy,Myotonic dystrophy,Fascioscapulohumeral muscular dystrophy,limb girdle muscular dystrophy,oculopharyngeal muscular dystrophy,Emery-Dreifuss myopathies)

ii. Metabolic myopathies:McArdle’s disease iii. Periodic paralysis iv. Congenital myopathies v. Mitochondrial myopathy

I. Hereditary neuropathies:Charcot-Marie-Tooth diseases,Familial amyloidotic polyneuropathy

J. Hereditary ataxias:Friedreich ataxia,Ramsay Hunt syndrome,ataxia telangiectasia,SCA(Spinocerebellar ataxia),DRPLA(Dentato-rubral-pallidoluysian atrophy),Episodic ataxia

K. Heditary spastic paraplegia(HSP) (4) Intracranial pressure

A. Increased i. Intracranial mass lesions,Malignant hypertension,Idiopathic intracranial

hypertension(pseudotumor cerebri),SAH,Meningitis B. Decreased

i. Spontaneous intracranial hypotension,Post-lumbar puncture headache (5) Iatrogenic:medications[psychotropic drugs,steroids,digoxin,cimetidine,

anticonvulsants,anticholinergics,dopaminergics],procedure N(neoplastic/nutrition) (1) Neoplastic:brain metastasis or primary CNS tumor,paraneoplastic syndrome,meningeal

carcinomatosis,postradiation effects (2) Nutrition:Nicotinic acid(pellagra),Vitamine E,Thiamine(Wernicke-Korsakoff

syndrome,beriberi),Folic acid,Vitamin B6(optic neuritis) or Vitamin B12(Subacute combined degeneration) deficiency

S(seizure/structural/psychiatric): (1) Seizure:postictal state,nonconvulsive status epilepticus,complex partial seizure (2) Structural:Trauma,Cerebrovascular Events,Infection,Inflammatory disorders,

neoplasm,hydrocephalus,Congenital structural defects[Arnold-Chiari malformation,etc],herniation and compression,degenerative diease[herniated intervertebral disc,osteophyte],syringomyelia

(3) Psychiatric:bipolar disorder/mania,schizophrenia,neurosis,psychosis,depression,conversion disorder,malingering,personality disorder,hyperventilation