The PSP Associationassets.simplewebfiles.com/t/h/e/p/Study Days/PSP_presentation_Gina... · Working for a world free of PSP The PSP Association. Registered charity 1037087 The PSP

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

11


Content;Content;

• Aetiology• Symptoms• Differential diagnosis• Management• Summary• Video• The PSP Association• Questions ?


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


PSP PSP –– 1857 ?1857 ?


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


PSP PSP –– PathologyPathology


SymptomsSymptoms

• Rigidity• Bradykinesia• Loss of balance-with early falls• Dysphasia/dysphagia• Slowness of eye movements-downward

gaze• Possible personality changes/behavioural

problems


Clinical features in 187 cases of PSPClinical features in 187 cases of PSP

0102030405060708090

100

gaze palsyfalls

dysarthriadysphagiaapathy

tremor

dystonia

Presenter

Presentation Notes

Prev largest study 52 patients. Recorded versus examined only 35% versus 65% worse for apraxia. Common and underrecorded Examined eyes 62% dipl, 43% photpho and eyelid apraxia, treatable with botox. Dipl within 1 year or swall within 2years assocd worse prognosis.


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


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


Sitting En BlocSitting En Bloc


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


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)


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)


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


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


Eye ExaminationEye Examination


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


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


Cognitive Changes (Continued)Cognitive Changes (Continued)

Management;

• Need for families to understand

• Support for families/carers to recognise emotions

• Allow respite periods

• Antidepressants


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


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


PSP PSP –– Palliative carePalliative care

• Progressive condition, deterioration is individual

• Immobile• Unable to communicate• Unable to eat/drink• Restricted vision• Incontinent• Intellect intact


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


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.”


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


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


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


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


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


PSP PSP -- Dudley Moore 1935Dudley Moore 1935--20022002



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

http://www.pspeur.org/

mailto:[email protected]



Documents

The PSP Associationassets.simplewebfiles.com/t/h/e/p/Study Days/PSP_presentation_Gina... · Working for a world free of PSP The PSP Association. Registered charity 1037087 The PSP