The PSP Association. Presentation on the symptoms, care and support of patients with PSP.

Transcription

1 The PSP Association Presentation on the symptoms, care and support of patients with PSP. 1 Presented by: Kathy Miller-Hunt Development Officer Southwest

2 Other names Steele Richardson Olszewski Syndrome Parkinson s Plus Atypical Parkinsonism PSP is not a variant nor end stage of PD PSP is a distinctly different disease from PD but the early symptoms are similar to those experienced in patients with PD. 2

3 Prevalence At least 6 per 100,000 in the UK, as common as Motor Neurone Disease Actual figure believed higher Up to 10,000 living in the UK Up to 15 people per 100,000 Tucked in the shadow of PD 3

4 What is PSP? Comparatively rare neuro-degenerative brain disease Cause unknown No disease modifying treatment and no cure Terminal illness life expectancy 5 7 years from onset Progressively affects balance, mobility, vision, speech and swallowing Possible personality change and behavioural problems 4

5 What is CBD? 5 Cortico Basal Degeneration is similar to PSP but for the following features: Numbness, jerking fingers, loss of use of one hand It is asymmetric in that it affects a leg and an arm on one side of the body and then progressively moves to the other side Alien limb (where the limb does not seem to belong to its owner) Disturbance of eye movement is less common than in PSP Increased frontal lobe deficit

6 PSP Differential Diagnosis Non levodopa response No presenting tremor Rarely affects people under 40 Falls are often backwards Steady deterioration Restricted eye movement No loss of sense of smell Post mortem-neurofibrillary tangles not Lewy Bodies Tauopathy -not loss of Dopamine 6

7 Neurofibrillary Tangles 7

8 Care Pathway 8

9 PSP Postural Instability Falls often backwards without warning Difficulty with down gaze, shuffling, broad based gait Stiff arched neck, axial rigidity Tiredness, light-headedness Sudden change of direction, loss of balance O.T. grab rails, ramps, stair lift, through floor lift, adaptations to home, equipment Sitting en bloc rocket sign Limited benefit from PD drugs 9

10 PSP Visual Problems 10 Complex slow saccades- reading problems Slowness of up and down gaze Reflex movements remain normal (Doll s eye manoeuvre) Blurred, double, tunnel vision Sore, gritty, inflamed Eyelid apraxia/blepharospasm Light sensitivity Changing prescription for lenses of little benefit Register partially sighted or blind

11 Management Prism glasses Ptosis props or tape Eye drops Artificial tears (clarymist spray) Tinted wrap around glasses Botox injections Educate / Awareness family 11

12 PSP Speech & Swallowing Early involvement of Speech and Language Therapist Techniques taught for safe swallowing Exercises for speech Dietician PEG feeding Aspirational pneumonia Communication aids Oral Hygiene 12

13 Feeding Difficulties Difficulty looking down at plate Mouth stuffing or rapid drinking Poor self-feeding due stiffness or hand contracture Restricted head & neck posture or hyperextension may impair airway protection Impaired coordination between feeding, swallowing, and breathing Occasional difficulty opening mouth Lack of awareness of difficulties 13

14 PSP Speech and communication problems Early involvement of Speech and Language Therapist Exercises for speech Delay in response, word search not dementia Reduced facial expression poor eye contact Palilalia, stuttering Communication aids: Picture, alphabet boards, amplifiers, electronic aids Three types of speech pattern: Hypokinetic Dysarthria like PD, quiet, repetition of sounds Spastic Dysarthria strained, slow and slurred Ataxi Dysarthria slurred and imprecise, sounding drunk 14

15 PSP Cognitive Change Need for families to understand Vague changes in personality Intelligence largely intact Loss of higher executive function Signe d applause Short term memory problems Impaired judgement or reasoning Irritability, aggression, apathy Emotional lability Depression, general lack of interest Support for families, recognising emotions Antidepressant drugs 15

16 PSP Bladder & Bowel Problems Incontinence Continence advice nurse Bottle, Commode Pads, sheath Catheter either intermittent or permanent Nocturnal enuresis Difficulty with initiating flow U.T.I Constipation diet, roughage, laxatives Suppositories and enemas 16

17 PSP Palliative Care Improving with awareness of Neurological illness Referral to specialist in Palliative care Quality of life, patient choice, preferred place of care Continuing Health Care Assessment Hospice respite care, outreach team Increasing immobility, stiffness, contractures Pain control, antibiotics U.T.I. Respiratory infections Aspiration Pneumonia Ongoing carer Support to choose best place of care 17

18 PSP Professionals Access to our specialist nurses for support/advice Professional Membership CIP Leaflets/booklets/literature Study Sessions Reassurance that your patient is receiving expert advice Web access professionals section 18

19 PSP Nurse Specialist S/West Jill Lyons Helpline Talks/Study sessions Support Groups 6 in Southwest Clinics London Cambridge Newcastle Newport Manchester Edinburgh Hove 19

20 PSP - Summary Completely immobile Restricted vision Unable to communicate Often fed via PEG Incontinent Intelligence largely intact Very individual illness 20