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Working for a world free of PSPThe PSP Association. Registered charity 1037087
The PSP AssociationThe PSP AssociationPresentation on the symptoms, care and Presentation on the symptoms, care and
support support of patients with PSPof patients with PSP
Presented by:Presented by:Samantha Pavey, PSP Nurse SpecialistSamantha Pavey, PSP Nurse Specialist
and and Gina Rutterford, Development OfficerGina Rutterford, Development Officer
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Working for a world free of PSPThe PSP Association. Registered charity 1037087
Content;Content;
• Aetiology• Symptoms• Differential diagnosis• Management• Summary• Video• The PSP Association• Questions ?
Working for a world free of PSPThe PSP Association. Registered charity 1037087
PSP PSP oror Steele Richardson Olszewski Steele Richardson Olszewski SyndromeSyndrome
•1950s Richardson noticed unusual symptoms
•Steele & Olszewski studied pathological changes
•1964 first clear description of condition
Working for a world free of PSPThe PSP Association. Registered charity 1037087
PSP PSP –– 1857 ?1857 ?
Working for a world free of PSPThe PSP Association. Registered charity 1037087
PSP PSP –– AetiologyAetiology
• Degenerative progressive neurological condition• 6 per 100,000 of population (as MND)• Cause Unknown• Affects male/females equally• Age of onset 60 plus, rarely under 40• No effective treatment• No cure• Life expectancy 5-8 years
Working for a world free of PSPThe PSP Association. Registered charity 1037087
PSP PSP –– PathologyPathology
Working for a world free of PSPThe PSP Association. Registered charity 1037087
SymptomsSymptoms
• Rigidity• Bradykinesia• Loss of balance-with early falls• Dysphasia/dysphagia• Slowness of eye movements-downward
gaze• Possible personality changes/behavioural
problems
Working for a world free of PSPThe PSP Association. Registered charity 1037087
Clinical features in 187 cases of PSPClinical features in 187 cases of PSP
0102030405060708090
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gaze palsyfalls
dysarthriadysphagiaapathy
tremor
dystonia
Working for a world free of PSPThe PSP Association. Registered charity 1037087
PSP PSP –– Differential diagnosisDifferential diagnosis
• No current definitive diagnostic tool• Non levodopa responsive• No presenting tremor• Rarely affects people under 40• Falls are often backwards• Steady deterioration• Restricted eye movement• Neurofibrilliary tangles not Lewy Bodies
Working for a world free of PSPThe PSP Association. Registered charity 1037087
Postural InstabilityPostural Instability
• Falls-backwards, often without warning• Shuffling gait• Stiff arched neck, axial rigidity• Eye problems increase difficulties, cannot
look down• Tiredness, light-headed• Change of direction-loss of balance• Sitting ‘en bloc’/ Rocket sign
Working for a world free of PSPThe PSP Association. Registered charity 1037087
Sitting En BlocSitting En Bloc
Working for a world free of PSPThe PSP Association. Registered charity 1037087
Postural Instability Postural Instability Management;Management;
Refer to OT• Grabrails• Adaptation to home/equipment (Stair lift)• Bring bed downstairsRefer to Physio• Advice/exercise• Built up shoes• Weighted walking frame• Wheelchair
Hoist
Working for a world free of PSPThe PSP Association. Registered charity 1037087
PSP PSP –– SwallowSwallow• Reduced swallow• Overfill mouth • Weak cough reflex-Choking• SialorrheaManagement;-• Refer to SaLT• Technique for safe swallow• Softened diet/thickened fluids• Videofluroscopy• Atropine drops for saliva-
orally/glycopyrrolate/botox (hyoscine)
Working for a world free of PSPThe PSP Association. Registered charity 1037087
Swallow (Continued)Swallow (Continued)
Refer to dietician
• Weight loss• Supplements• PEG feeding
(Issues re end of life discussed early on so patients wishes are known)
Working for a world free of PSPThe PSP Association. Registered charity 1037087
PSP PSP –– SpeechSpeech
• Hypokinetic Dysarthria – (quiet, fast repetition of sounds (pallilalia) -like PD)
• Spastic Dysarthria – (slow and slurred, voice harsh or strained)
• Ataxic Dysarthria – (slurred, imprecise, sounding drunk, difficulty with co-ordination of tongue, lips, voice)
• Delay in response; word search (library)ManagementRefer to SaLT• Exercises may help• Communication aids: picture/alphabet boards,
amplifiers, electronic aids
Working for a world free of PSPThe PSP Association. Registered charity 1037087
Visual ImpairmentVisual Impairment
• Slow up/down gaze• Mona Lisa syndrome• Dolls eye movement• Blurred, tunnel vision• Slow saccades• Blepharospasm, apraxia of eye opening• Sore, gritty inflamed eyes• Photophobia
Working for a world free of PSPThe PSP Association. Registered charity 1037087
Eye ExaminationEye Examination
Working for a world free of PSPThe PSP Association. Registered charity 1037087
Management;Management;
• Referral to orthoptist (new lenses may not be beneficial)
• Prisms• Eye crutches/tapes• Wrap around sunglasses• Botulinum toxin injections• Register blind/partially sighted
Working for a world free of PSPThe PSP Association. Registered charity 1037087
PSP PSP –– Cognitive changesCognitive changes
• Vague changes to personality• Intellect largely intact• Loss of higher executive function • Short term memory loss• Impaired judgement• Irritability, aggression, apathy• Emotional lability• Depression
Working for a world free of PSPThe PSP Association. Registered charity 1037087
Cognitive Changes (Continued)Cognitive Changes (Continued)
Management;
• Need for families to understand
• Support for families/carers to recognise emotions
• Allow respite periods
• Antidepressants
Working for a world free of PSPThe PSP Association. Registered charity 1037087
PSP PSP –– PainPain
• Rigidity of muscles (particularly neck)• Cramp in muscles• Possible contracturesManagement;• Ask GP to rule out other causes• Muscle relaxants• Warmth, analgesia• Massage• Complementary therapies; aromatherapy,
reflexology, acupuncture
Working for a world free of PSPThe PSP Association. Registered charity 1037087
PSP PSP –– Continence IssuesContinence Issues
• Bladder/bowel incontinence• Nocturnal eneuresis• Difficulty initiating flow• UTI’s
Constipation
Management;• Refer to Continence Nurse Advisor• Ultrasound• Bottles/pads/conveens/commodes• High fibre diet/adequate fluids• Suppositories, enemas, laxatives• Intermittent /permanent catheterisation
Working for a world free of PSPThe PSP Association. Registered charity 1037087
PSP PSP –– Palliative carePalliative care
• Progressive condition, deterioration is individual
• Immobile• Unable to communicate• Unable to eat/drink• Restricted vision• Incontinent• Intellect intact
Working for a world free of PSPThe PSP Association. Registered charity 1037087
Palliative care (continued)Palliative care (continued)
Management;Refer to specialist in palliative care• QOL• Patient choice, patient preferred place of care• Continuing care assessment• Hospice respite care, outreach team• Analgesia, antibiotics as required• Carer support
Working for a world free of PSPThe PSP Association. Registered charity 1037087
PSP PSP -- A patients viewA patients view
“I have 2 strong legs that I can movebut when I walk I fall;I have a strong voicebut when I talk no-one understands;I have good visionbut I cannot see – I cannot read;My throat is healthybut I cannot eat without choking.”
Working for a world free of PSPThe PSP Association. Registered charity 1037087
PSP PSP –– Registered Charity:Registered Charity:--
• To promote research worldwide into the cause, effective treatment and cure
• To provide information and support to afflicted families across Europe
• To engender awareness
Working for a world free of PSPThe PSP Association. Registered charity 1037087
PSP PSP –– Sara Sara KoeKoe Research Research CentreCentre
• Set up January 2002
• Sponsored by the PSP Association
• Part of the Institute of Neurology, National Hospital, Queen’s Square, London
• PSP Brain Tissue Bank
Working for a world free of PSPThe PSP Association. Registered charity 1037087
PSP PSP –– AchievementsAchievements
• Funding almost £2m of research worldwide• Held 6 International Medical Workshops devoted to
PSP• Established 24 hour telephone counselling service• Provides 3 Specialist Nurses• Set up 16 different Local Support Groups across
UK• Establishing 6 Development Officers
Working for a world free of PSPThe PSP Association. Registered charity 1037087
PSP PSP –– Nurse SpecialistsNurse Specialists
• Operate 24/7 telephone helpline• Attend and advise each Support Group 3
times a year• Consult at Specialist PSP Clinics in London,
Cambridge, Newcastle, Newport and Manchester
• Give talks to Professionals, Multidisciplinary teams etc
Working for a world free of PSPThe PSP Association. Registered charity 1037087
PSP PSP –– SubscribersSubscribers
• Telephone Counselling Service• Local Support Groups• PSP Matters• Web Site Forum• Annual Symposium – September 2009 Cardiff• Carers’ Information Pack• Information on current research• Database
Working for a world free of PSPThe PSP Association. Registered charity 1037087
PSP PSP -- Dudley Moore 1935Dudley Moore 1935--20022002
Working for a world free of PSPThe PSP Association. Registered charity 1037087
For more information:For more information:• Please look at our website: www.pspeur.org
• or Telephone: 01327 322410
• or Email: [email protected]
• or Write to us at: The PSP AssociationPSP House167 Watling Street WestTowcester, Northants, NN12 6BX
• Nurse Specialist Samantha Pavey- South East: 0208 150 6250 [email protected]
• Development Officer Gina Rutterford 0790 181 [email protected]
• Registered Charity Number 1037087