1. Parkinson’s Disease Dr Rachel Cary, Warwick Hospital

2. Overview  Hopefully not anything new (!)  Definition of Parkinson’s disease  Aetiology  Presentation  Investigations  Management: conservative, medical, surgical  Prognosis  Parkinson’s plus syndromes

3. Definition  Parkinson’s disease  The idiopathic syndrome of Parkinsonism  Described by Dr James Parkinson in 1817 – ‘the shaking palsy’  Parkinsonism  Symptom complex: characterised by bradykinesia, tremor and rigidity (classical triad)  Causes:  Drug induced – anti-dopaminergics e.g. clozapine, metoclopramide, domperidone  Post-encephalitis  Exposure to toxins: e.g. severe CO poisoning

4. How common is it?  65.6-125 per 100,000  Incidence rises steeply with age:  17.4 in 100,000 person years between age 50-59  93.1 in 100,000 person years between 70 and 79 years

5. Risk factors  Age  Male gender  Pesticide exposure  Genetic  ?born in the springtime

6. Aetiology and pathogenesis  Death of dopamine generating cells in the substantia nigra  Pathology characterised by accumulation of alpha-synuclein into inclusions  Lewy bodies  Insufficient formation and activity of dopamine in the midbrain

7. Presentation: symptoms + signs  Tremor 4-6Hz – unilateral onset  Rigidity  Slowness of movement  Postural instability  Gait: festinating, shuffling, reduced arm swing  Gait freezing  Turning en bloc  Hypomimia  Micrographia  Hypophonia

8. How will you elicit these in an OSCE?

9. Differential diagnosis  Benign essential tremor  Drug/toxin induced  Huntingon’s disease  Wilson’s disease  Parkinson’s plus syndromes (we’ll come to these later)  Lewy body dementia  CJD  Cerebellar tremor  Pick’s disease

10. Investigations  Bedside tests  Clinical diagnosis  Blood tests  Imaging  CT Head  Special tests: DAT  SPECT -> type of PET looking at striatal dopamine transporter

11. Associated diseases  Dementia in 20-40%  Depression – 45%

12. Management - conservative  Multidisciplinary management  Parkinson’s nurse  Physiotherapy  Avoid Zimmer frames unless they have wheels and a handbrake  Occupational therapy  Referral to other services as needed

13. Medical  Dopamine agonists e.g. ropinirole, bromocriptine  MAO inhibitors e.g. selegeline  Levodopa + decarboxylase inhibitor  COMT e.g. entacapaone  Continuous dopamine therapy  Supportive medication e.g. baclofen

14. Choice of drug  Depends on impact of improving motor disability vs risk of motor complications, and neuropsychiatric complications  Levodopa is the most effective drug: Sinemet, Madopar  Long term levodopa treatment associated with adverse motor effects that limit its use

15. Surgery  Bilateral subthalamic nucleus stimulation in patients refractory to medical treatment  Globus pallidus interna is also recommended by NICE, but rarely performed in the UK  Thalamic stimulation is an alternative for patients with sever tremor who are unsuitable for STN stimulation

16. Longer term problems  Motor fluctuations – “on” and “off”  Axial problems not responding to treatment  Balance  Speech  Gait disturbance  Parkinson’s disease dementia

17. Complications  Infections  Aspiration pneumonia  Pressure sores  Poor nutrition  Falls  Contractures  Bowel and bladder disorders

18. Prognosis  Slowly progressive  Mean duration 15 years  Large variation in progression

19. Parkinson’s plus syndromes  Features of Parkinson’s disease with additional features  Often symmetrical onset  Lack of/irregular resting tremor  Reduced response to dopaminergic drugs  Often more rapidly progressive  Multiple system atrophy  Autonomic dysfunction  Ataxia  Progressive supranuclear palsy  Supranuclear opthalmoplegia  Neck dystonia  Pseudobulbar palsy  Frequent falls, imbalance, and difficulty walking  Behavioural and cognitive impairment  Corticobasal degeneration  Alien hand syndrome  Apraxia  Aphasia

20. Summary  Parkinsonism  Rigidity, bradykinesia, tremor  Clinical diagnosis  Multidisciplinary team management  Drugs: levodopa and others  Problems associated with long-term usage of levodopa etc.  DBS  Complications and associated comorbidity  Prognosis