progressive supranuclear palsy and cortico basal degeneration

1 Working for a world free of PSP and CBDRegistered charity numbers: England and Wales 1037087 / Scotland SC041199

Progressive Supranuclear Palsy Progressive Supranuclear Palsy and and Cortico Basal DegenerationCortico Basal Degeneration

Presentation for Presentation for St Johns Hospice St Johns Hospice

Katrina HainesKatrina Haines RGN, BSc, MScRGN, BSc, MSc

Nurse Specialist Nurse Specialist North West and Midlands, North West and Midlands,

North Wales, North Wales, Northern Ireland and Republic of IrelandNorthern Ireland and Republic of Ireland


PSP and CBDPSP and CBD

Introduction to PSP/CBD: pathology, types, stages, symptoms disease management


Overview of Progressive Overview of Progressive Supranuclear Palsy (PSP)Supranuclear Palsy (PSP)

What is PSP? Also known as Steele Richardson Olszewski syndrome

Chronic, terminal neuro-degenerative disorder

Degeneration of brainstem structures

What is the patient population? Prevalence - 6.4 per 100,000 (may be higher)-MND

Adult onset (mid 50s - mid 70s)

Patients usually die within 5/10 yrs -onset of symptoms

and 2/4 years from diagnosis

What are the symptoms? Disturbance of balance

Impaired mobility

Disordered vertical gaze

Progressive disorder of speech and swallowing

1


CBD – What is it?CBD – What is it?

Numbness, jerking fingers, loss of use of one hand

Asymmetric; progressively affecting arm and leg Alien limb Less common disturbance of eye movement Increased frontal lobe deficit

Cortico Basal Degeneration – similar to PSP except:


Pathology of PSPPathology of PSP

Mid brain atrophy

Neuronal loss4

Neurofibrillary tangles in the basal ganglia, diencephalon and brainstem4

Minimal cortical pathology except for

motor areas4

Some cases of PSP map to a polymorphism in

the tau gene4


Imaging in PSP/ MRI


Clinical Diagnosis of PSPClinical Diagnosis of PSP

Eye movements restricted (up and down gaze)2

Low frequency blinking3

Early falls (often backwards)1,3

Impaired mobility (clumsy gait)2

Axial rigidity2

Presenting Features


PSP – frequent misdiagnosisPSP – frequent misdiagnosis


PSP – Differential DiagnosisPSP – Differential Diagnosis

Poor levodopa response (compared to PD) No presenting tremor Usually affects people over 40 Falls are often backwards Steady deterioration Restricted eye movement Neurofibrillary tangles not Lewy Bodies


Management of PSPManagement of PSP

Multidisciplinary approach

Speech and language therapists for

early monitoring of

weight and discussion of PEG feeding. Prevention

of aspiration pneumonia.

OphthalmologistSpectacles with “crutch”

or prism spectacles. Botulinum toxin

for eyelid apraxia. Lubricating eyedrops

for sore eyes. Dark glasses. Facilities for

visually impaired

Physiotherapy and

Occupational therapy

Walking aids and adviceto prevent falls,

equipment to maintain ADL

and prevent contractures

Multidisciplinary approach

Contact details of

PSP Association

support patient and family

Psychological support

Recognition and

treatment of

depression.

Early involvement of Palliative care

services

Symptom relief Advanced care planning,

Management of complex needs

NCPC guidelines.


Natural history of PSPNatural history of PSP

PSP/CBD is relentlessly progressive

It is now recognised that some forms of PSP follow a more benign course.

The proximate cause of death in many cases is aspiration pneumonia.

Median interval (yrs) from initial symptom to development of major deficits (Golbe 1988)

Initial gait difficulty 0.3 Aid needed to walk 3.1 Dysarthria 3.4 Visual symptoms 3.9 Dysphagia 4.4 Confined to bed or

wheelchair 8.2 Death 9.7


PSP typesPSP types

Richardson Syndrome( most common) Postural instability and Falls Cognitive dysfunction Bulbar signs Supranuclear gaze palsy/Abnormal saccades

PSP-Parkinsonism( less severe) Bradykinesia/tremor Limb dystonia Asymmetric onset Levodopa responsive rigidity

Pure Akinesia with Gait Freezing Early falls and movement difficulties Late bulbar problems Rarely eye problems No dementia


PSP - SymptomsPSP - Symptoms

Postural Instability Eye Problems Swallow Problems Speech Problems Cognitive Changes Bladder and Bowel Pain


PosturePosture

Increased stiffness Nunchal rigidity-coat hanger

pain refers into arms or head Head pokes forward at the chin

or chin in the air- retrocollis Thoracic spine -axial rigidity or

kyphosed if slumped Pelvic area becomes stiff


PSP – Postural InstabilityPSP – Postural Instability

PROBLEMS Falls - often backwards Difficulty with down gaze, shuffling, broad based gait Stiff arched neck, axial rigidity Tiredness, light-headedness Sudden change of direction, loss of balance Sitting “en bloc” “rocket sign” make stairlifts difficult

MANAGEMENT O.T. – grab rails, ramps, through floor lift, adaptations to home,

equipment Helmet and hip protectors Limited benefit from PD drugs


PSP - Eye ProblemsPSP - Eye Problems

Problem with movement of the eye itself Starts with slow saccades Later limited voluntary vertical saccades Especially down gaze Reflex movements remain normal (Doll’s eye

manoeuvre) Indicating supranuclear problem fault with eye muscle

innervation not eye muscles Upper motor neuron paresis Limited up gaze can be normal in elderly but never

accompanied by slow saccades


Management of Eye Lid Management of Eye Lid ProblemsProblems

Position in sight line Prism glasses Ptosis props or tape Eye drops, Artificial tears (clarymist spray) Tinted wrap around glasses Botox injections Educate / Awareness family


PSP – Swallow ProblemsPSP – Swallow Problems

PROBLEMS Eat too quickly Overfill Mouth Weak cough / choke Excess salivation Aspiration pneumonia

MANAGEMENT Early referral to speech therapist Dietician – supplements Swallowing techniques Softened diet / thickened fluids Videofluoroscopy PEG insertion


PSP – Speech ProblemsPSP – Speech Problems

PROBLEMS Delay in response, word search Reduced facial expression Palalalia, stuttering Three types of speech pattern:

– Hypokinetic Dysarthria – like PD, quiet, repetition of sounds– Spastic Dysarthria – strained, slow and slurred– Ataxic Dysarthria – slurred and imprecise, sounding drunk

MANAGEMENT Early involvement of Speech and Language Therapist Exercises for speech-singing Communication aids: Picture and alphabet boards, amplifiers,

electronic aids


PSP – Cognitive ChangePSP – Cognitive Change

PROBLEMS Intelligence largely intact Loss of higher executive function Sleep disturbance Short term memory problems Impaired judgement or reasoning Irritability, aggression, apathy Emotional lability Depression, general lack of interest Vague changes in personality

MANAGEMENT Need for families to understand Support for families, recognising emotions Antidepressant drugs


PSP - PainPSP - Pain

COMMON PAINS Check with GP for any underlying cause Neck (and referred) U.T.I Cramp as stiffness increases Possible contractures General aches from postural and muscle weakness

MANAGEMENT Passive Exercises Postural management Warmth, analgesia Muscle relaxants Massage Complementary therapies, acupuncture, reflexology, aromatherapy, exercise for

as long as possible


PSP – Bladder & Bowel PSP – Bladder & Bowel ProblemsProblems

PROBLEMS Difficulty with initiating flow Oversensitivity Nocturnal Enuresis U.T.I Incontinence Constipation Loss of bowel control

MANAGEMENT Increase fluids Increase fruit and roughage in diet Continence advice nurse Bottle, commode Pads, sheath Catheter – either intermittent or permanent Suppositories and enemas


PSP- Initial StagePSP- Initial Stage

Early symptoms – balance problems and postural instability Initial visit to GP and neurologist-possibly diagnosis (maybe

incorrect) Meds - poss L’dopa, Domperidone, poss antidepressant

On diagnosis-GSF-palliative care register- out of hrs care, better MDT communication – does the relevant question stand?

Support from PSP Assoc, Nurse Specialists, support groups, forum

Baseline assessments-Physio, Salt and OT

Care assessment from SW, poss Carers Assessment too – benefits Day care. Falls Clinic-poss protective equipment

Discuss ACD and future wishes-will, POA


PSP- Intermediate StagePSP- Intermediate Stage

Increasing difficulty with speech and swallowing-further input from Salt and dietician/ nutritionalist nurse, assistive technology-communication aids. Meds for excessive salivation

Visual problems-neuro-optometrist-fresnel lenses,eyedrops,dark glasses

Behavioural and cognitive changes -psychologist, psycho-geriatrician-assessment of capacity and memory (A-CER,FAB not MMSE)

Increased movement difficulties – medications

Sleeping/mood problems-sleep hygiene, medication

Bladder and bowel issues-Continence Nurse-medication

Re-assessment of care needs and benefits

Daycare and Respite


PSP- End StagePSP- End Stage

CHCF

NBM-poss PEG procedure (done earlier if poss –

for optimum benefit)

Palliative care –McMillan nurses, hospice, respite

Pain and symptom control


Palliative CarePalliative Care

Incurable deteriorating condition-QoL Support from diagnosis-all aspects of living GP Palliative care register MDT management -identified care co-ordinator Proactive monitoring of changes and symptom

management. Continuing health care funding Access to respite / hospice care Anticipatory care planning/Advanced decisions Care and support for carers


DRIVERSDRIVERS

Long term neurological conditions NSF Living matters dying matters 2010 National Council for Palliative Care 2006 World Health Organisation 2005 Gold Standards Framework Liverpool Care Pathway Mental Capacity Act 2005


Drug Treatment for PSPDrug Treatment for PSP

No specific licensed treatmentAnti-parkinsonian drugs – modest benefit, may

cause side effects2,3

Amantadine – may benefit some patients3

Botulinum toxin – for neck muscle spasm and blepharospasm3

Artificial tears – for dry eyes9

Antidepressants – (amitriptyline helpful for sleep disturbance)

Excessive salivation -glycopyrronium/atropine


CHALLENGES OF PSPCHALLENGES OF PSP

MEDICAL MANAGEMENT

CO-MORBIDITIES

INDIVIDUAL VARIABILITY OF CONDITION

DETERMINING END OF LIFE


PSP – Nurse SpecialistsPSP – Nurse Specialists

Helpline

Talks/Study sessions

Support Groups30 around the UK and RoI

Including

Lancaster, Manchester,

Holmes Chapel

Clinics •London•Coventry•Cambridge•Newcastle•Newport•Manchester•Brighton•Edinburgh

progressive supranuclear palsy and cortico basal degeneration

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