Progressive Supranuclear Palsy
and Cortico Basal Degeneration
Presentation for
St Johns Hospice
Katrina Haines RGN, BSc, MSc
kat.haines@pspeur.org- 01995 601533
Nurse Specialist
North West and Midlands,
North Wales,
Northern Ireland and Republic of Ireland
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Working for a world free of PSP and CBD
Registered charity numbers: England and Wales 1037087 / Scotland SC041199
PSP and CBD
Introduction to PSP/CBD:
 pathology,
 types,
 stages,
 symptoms
 disease management
2
Working for a world free of PSP and CBD
Registered charity numbers: England and Wales 1037087 / Scotland SC041199
Overview of Progressive
Supranuclear Palsy (PSP)
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What is PSP?
Also known as Steele Richardson Olszewski syndrome
Chronic, terminal neuro-degenerative disorder
Degeneration of brainstem structures
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What is the patient population?
Prevalence - 6.4 per 100,000 (may be higher)-MND
Adult onset (mid 50s - mid 70s)
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Patients usually die within 5/10 yrs -onset of symptoms
and 2/4 years from diagnosis
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What are the symptoms?
Disturbance of balance
Impaired mobility
Disordered vertical gaze
Progressive disorder of speech and swallowing
Working for a world free of PSP and CBD
Registered charity numbers: England and Wales 1037087 / Scotland SC041199
PSP - Symptoms
 Postural Instability
 Eye Problems
 Swallow Problems
 Speech Problems
 Cognitive Changes
 Bladder and Bowel
 Pain
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Working for a world free of PSP and CBD
Registered charity numbers: England and Wales 1037087 / Scotland SC041199
CBD – What is it?
Cortico Basal Degeneration – similar to PSP except:
 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
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Registered charity numbers: England and Wales 1037087 / Scotland SC041199
Pathology of PSP
Mid brain atrophy
Neuronal loss4
Neurofibrillary tangles in
the basal ganglia,
diencephalon and
brainstem4
Minimal cortical
pathology except for
motor areas4
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Some cases of PSP map
to a polymorphism in
the tau gene4
Working for a world free of PSP and CBD
Registered charity numbers: England and Wales 1037087 / Scotland SC041199
Imaging in PSP/ MRI
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Working for a world free of PSP and CBD
Registered charity numbers: England and Wales 1037087 / Scotland SC041199
Clinical Diagnosis of PSP
Axial rigidity2
Impaired mobility
(clumsy gait)2
Eye movements restricted
(up and down gaze)2
Presenting Features
Low frequency blinking3
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Early falls
(often backwards)1,3
Working for a world free of PSP and CBD
Registered charity numbers: England and Wales 1037087 / Scotland SC041199
PSP – frequent misdiagnosis
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Working for a world free of PSP and CBD
Registered charity numbers: England and Wales 1037087 / Scotland SC041199
PSP – 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
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Working for a world free of PSP and CBD
Registered charity numbers: England and Wales 1037087 / Scotland SC041199
Service involvement (Source: PSPeur website)
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Working for a world free of PSP and CBD
Registered charity numbers: England and Wales 1037087 / Scotland SC041199
Natural 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.
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Median interval (yrs) from
initial symptom to
development of major
deficits (Golbe 1988)
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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 CBD
Registered charity numbers: England and Wales 1037087 / Scotland SC041199
PSP types
Richardson Syndrome( most common)
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Postural instability and Falls
Cognitive dysfunction
Bulbar signs
Supranuclear gaze palsy/Abnormal saccades
PSP-Parkinsonism( less severe)
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Bradykinesia/tremor
Limb dystonia
Asymmetric onset
Levodopa responsive rigidity
Pure Akinesia with Gait Freezing
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Early falls and movement difficulties
Late bulbar problems
Rarely eye problems
No dementia
Working for a world free of PSP and CBD
Registered charity numbers: England and Wales 1037087 / Scotland SC041199
Posture
 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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Working for a world free of PSP and CBD
Registered charity numbers: England and Wales 1037087 / Scotland SC041199
PSP – 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
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Working for a world free of PSP and CBD
Registered charity numbers: England and Wales 1037087 / Scotland SC041199
PSP – the stare
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Working for a world free of PSP and CBD
Registered charity numbers: England and Wales 1037087 / Scotland SC041199
PSP - Eye Problems
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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
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Working for a world free of PSP and CBD
Registered charity numbers: England and Wales 1037087 / Scotland SC041199
Management of Eye Lid
Problems
 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
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Working for a world free of PSP and CBD
Registered charity numbers: England and Wales 1037087 / Scotland SC041199
PSP – Swallow Problems
PROBLEMS
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Eat too quickly
Overfill Mouth
Weak cough / choke
Excess salivation
Aspiration pneumonia
MANAGEMENT
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Early referral to speech therapist
Dietician – supplements
Swallowing techniques
Softened diet / thickened fluids
Videofluoroscopy
PEG insertion
Working for a world free of PSP and CBD
Registered charity numbers: England and Wales 1037087 / Scotland SC041199
PSP – 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
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Working for a world free of PSP and CBD
Registered charity numbers: England and Wales 1037087 / Scotland SC041199
PSP – 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
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Working for a world free of PSP and CBD
Registered charity numbers: England and Wales 1037087 / Scotland SC041199
PSP - 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
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Working for a world free of PSP and CBD
Registered charity numbers: England and Wales 1037087 / Scotland SC041199
PSP – Bladder & Bowel
Problems
PROBLEMS
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Difficulty with initiating flow
Oversensitivity
Nocturnal Enuresis
U.T.I
Incontinence
Constipation
Loss of bowel control
MANAGEMENT
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Increase fluids
Increase fruit and roughage in diet
Continence advice nurse
Bottle, commode
Pads, sheath
Catheter – either intermittent or permanent
Suppositories and enemas
Working for a world free of PSP and CBD
Registered charity numbers: England and Wales 1037087 / Scotland SC041199
Palliative Care
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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
Working for a world free of PSP and CBD
Registered charity numbers: England and Wales 1037087 / Scotland SC041199
DRIVERS
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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
Working for a world free of PSP and CBD
Registered charity numbers: England and Wales 1037087 / Scotland SC041199
Drug Treatment for PSP
No specific licensed treatment
Anti-parkinsonian drugs – modest benefit, may
cause side effects
Amantadine – may benefit some patients
Botulinum toxin – for neck muscle spasm and
blepharospasm
Artificial tears – for dry eyes
Antidepressants – (amitriptyline helpful for
sleep disturbance)
Excessive salivation -glycopyrronium/atropine
2,3
3
3
9
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Working for a world free of PSP and CBD
Registered charity numbers: England and Wales 1037087 / Scotland SC041199
CHALLENGES OF PSP
 MEDICAL MANAGEMENT
 CO-MORBIDITIES
 INDIVIDUAL VARIABILITY OF CONDITION
 DETERMINING END OF LIFE
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Working for a world free of PSP and CBD
Registered charity numbers: England and Wales 1037087 / Scotland SC041199
PSP – Nurse Specialists
Helpline
Support Groups
30 around the UK and RoI
Including
Lancaster, Manchester,
Holmes Chapel
Clinics
Talks/Study sessions
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•London
•Coventry
•Cambridge
•Newcastle
•Newport
•Manchester
•Brighton
•Edinburgh
Working for a world free of PSP and CBD
Registered charity numbers: England and Wales 1037087 / Scotland SC041199