Working for a world free of PSP and CBD Registered charity numbers: England and Wales 1037087 /...
Transcript of Working for a world free of PSP and CBD Registered charity numbers: England and Wales 1037087 /...
Working for a world free of PSP and CBDRegistered charity numbers: England and Wales 1037087 / Scotland SC0411991
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
[email protected] 01995 601533 01995 601533
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
Working for a world free of PSP and CBDRegistered charity numbers: England and Wales 1037087 / Scotland SC0411992
PSP and CBDPSP and CBD
Introduction to PSP/CBD:pathology, types,stages, symptoms disease management
Working for a world free of PSP and CBDRegistered charity numbers: England and Wales 1037087 / Scotland SC0411993
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
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Working for a world free of PSP and CBDRegistered charity numbers: England and Wales 1037087 / Scotland SC0411994
PSP - SymptomsPSP - Symptoms
Postural InstabilityEye ProblemsSwallow ProblemsSpeech ProblemsCognitive ChangesBladder and BowelPain
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CBD – What is it?CBD – What is it?
Numbness, jerking fingers, loss of use of one hand
Asymmetric; progressively affecting arm and legAlien limbLess common disturbance of eye movementIncreased frontal lobe deficit
Cortico Basal Degeneration – similar to PSP except:
Working for a world free of PSP and CBDRegistered charity numbers: England and Wales 1037087 / Scotland SC0411996
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
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Imaging in PSP/ MRI
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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
Working for a world free of PSP and CBDRegistered charity numbers: England and Wales 1037087 / Scotland SC0411999
PSP – frequent misdiagnosisPSP – frequent misdiagnosis
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PSP – Differential DiagnosisPSP – Differential Diagnosis
Poor levodopa response (compared to PD)No presenting tremorUsually affects people over 40Falls are often backwardsSteady deteriorationRestricted eye movementNeurofibrillary tangles not Lewy Bodies
Working for a world free of PSP and CBDRegistered charity numbers: England and Wales 1037087 / Scotland SC04119911
Service involvement (Source: PSPeur website)Service involvement (Source: PSPeur website)
Working for a world free of PSP and CBDRegistered charity numbers: England and Wales 1037087 / Scotland SC04119912
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
Working for a world free of PSP and CBDRegistered charity numbers: England and Wales 1037087 / Scotland SC04119913
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
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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
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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
Working for a world free of PSP and CBDRegistered charity numbers: England and Wales 1037087 / Scotland SC04119916
PSP – the starePSP – the stare
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PSP - Eye ProblemsPSP - Eye Problems
Problem with movement of the eye itselfStarts with slow saccadesLater limited voluntary vertical saccadesEspecially down gazeReflex movements remain normal (Doll’s eye
manoeuvre) Indicating supranuclear problem fault with eye muscle
innervation not eye musclesUpper motor neuron paresisLimited up gaze can be normal in elderly but never
accompanied by slow saccades
Working for a world free of PSP and CBDRegistered charity numbers: England and Wales 1037087 / Scotland SC04119918
Management of Eye Lid Management of Eye Lid ProblemsProblems
Position in sight linePrism glassesPtosis props or tapeEye drops, Artificial tears (clarymist spray)Tinted wrap around glassesBotox injectionsEducate / Awareness family
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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
Working for a world free of PSP and CBDRegistered charity numbers: England and Wales 1037087 / Scotland SC04119920
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
Working for a world free of PSP and CBDRegistered charity numbers: England and Wales 1037087 / Scotland SC04119921
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
Working for a world free of PSP and CBDRegistered charity numbers: England and Wales 1037087 / Scotland SC04119922
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
Working for a world free of PSP and CBDRegistered charity numbers: England and Wales 1037087 / Scotland SC04119923
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
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Palliative CarePalliative Care
Incurable deteriorating condition-QoLSupport from diagnosis-all aspects of livingGP Palliative care registerMDT management -identified care co-ordinatorProactive monitoring of changes and symptom
management.Continuing health care fundingAccess to respite / hospice careAnticipatory care planning/Advanced decisionsCare and support for carers
Working for a world free of PSP and CBDRegistered charity numbers: England and Wales 1037087 / Scotland SC04119925
DRIVERSDRIVERS
Long term neurological conditions NSFLiving matters dying matters 2010National Council for Palliative Care 2006World Health Organisation 2005Gold Standards FrameworkLiverpool Care PathwayMental Capacity Act 2005
Working for a world free of PSP and CBDRegistered charity numbers: England and Wales 1037087 / Scotland SC04119926
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
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CHALLENGES OF PSPCHALLENGES OF PSP
MEDICAL MANAGEMENT
CO-MORBIDITIES
INDIVIDUAL VARIABILITY OF CONDITION
DETERMINING END OF LIFE
Working for a world free of PSP and CBDRegistered charity numbers: England and Wales 1037087 / Scotland SC04119928
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