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Progressive Supranuclear Palsy and Cortico Basal Degeneration Presentation for St Johns Hospice Katrina Haines RGN, BSc, MSc Nurse Specialist North West and Midlands, North Wales, Northern Ireland and Republic of Ireland 1 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) 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 3 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 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 4 Working for a world free of PSP and CBD 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 5 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 6 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 7 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 8 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 9 Working for a world free of PSP and CBD Registered charity numbers: England and Wales 1037087 / Scotland SC041199 Management of PSP Multidisciplinary approach Multidisciplinary approach Contact details of Psychological support PSP Association Recognition and treatment of depression. support patient and family Early involvement of Palliative care services Symptom relief Advanced care planning, Management of complex needs NCPC guidelines . 10 Speech and language therapists for early monitoring of weight and discussion of PEG feeding. Prevention of aspiration pneumonia. Ophthalmologist Spectacles with “crutch” or prism spectacles. Botulinum toxin for eyelid apraxia. Lubricating eyedrops for sore eyes. Dark glasses. Facilities for visually impaired Working for a world free of PSP and CBD Registered charity numbers: England and Wales 1037087 / Scotland SC041199 Physiotherapy and Occupational therapy Walking aids and advice to prevent falls, equipment to maintain ADL and prevent contractures 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. 11 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 CBD Registered charity numbers: England and Wales 1037087 / Scotland SC041199 PSP 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 12 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 PSP - Symptoms Postural Instability Eye Problems Swallow Problems Speech Problems Cognitive Changes Bladder and Bowel Pain 13 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 14 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 15 Working for a world free of PSP and CBD Registered charity numbers: England and Wales 1037087 / Scotland SC041199 16 Working for a world free of PSP and CBD Registered charity numbers: England and Wales 1037087 / Scotland SC041199 17 Working for a world free of PSP and CBD Registered charity numbers: England and Wales 1037087 / Scotland SC041199 PSP - 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 18 Working for a world free of PSP and CBD Registered charity numbers: England and Wales 1037087 / Scotland SC041199 19 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 20 Working for a world free of PSP and CBD Registered charity numbers: England and Wales 1037087 / Scotland SC041199 PSP – Swallow Problems PROBLEMS Eat too quickly Overfill Mouth Weak cough / choke Excess salivation Aspiration pneumonia MANAGEMENT 21 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 22 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 23 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 24 Working for a world free of PSP and CBD Registered charity numbers: England and Wales 1037087 / Scotland SC041199 PSP – Bladder & Bowel Problems PROBLEMS Difficulty with initiating flow Oversensitivity Nocturnal Enuresis U.T.I Incontinence Constipation Loss of bowel control MANAGEMENT 25 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 PSP- 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 26 Discuss ACD and future wishes-will, POA Working for a world free of PSP and CBD Registered charity numbers: England and Wales 1037087 / Scotland SC041199 PSP- 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-geriatricianassessment 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 27 Daycare and Respite Working for a world free of PSP and CBD Registered charity numbers: England and Wales 1037087 / Scotland SC041199 PSP- End Stage CHCF NBM-poss PEG procedure (done earlier if poss – for optimum benefit) Palliative care –McMillan nurses, hospice, respite Pain and symptom control 28 Working for a world free of PSP and CBD Registered charity numbers: England and Wales 1037087 / Scotland SC041199 Palliative Care 29 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 30 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 31 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 32 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 33 •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