Parkinsonism and other movement disorders

1. Parkinsonism and other movement disorders PRM de Bittencourt www.unineuro.com.br

2. 1977: started classical neurology training 1982: first started with a large amount of clinical work 1985: depression definitely associated with Parkinson’s, imipramine replaced other anticholinergics It was thought Parkinson’s evolved without dementia, with depression, perhaps dementia at the end Initial surprise at the great number of cases “cured” of Parkinson’s disease The concept of Parkinson’s disease

3. Letter to CD Marsden in 1986 1988: Dr Marsden: diagnosis is really difficult Recommended a number of criteria 1988: Chouza e Melo-Souza, parkinsonism due to cinarizine and flunarizine 100. Cunha CA, Bittencourt PRM, Kohlscheen KL, Mercer LM. Reversible parkinsonism induced by cinarizine and flunarizine. Revista Médica do Paraná 50:13-16, 1993 The most frequent cause of parkinsonism in Curitiba in the 80s: “labirintitis”

4. Pathologically, patients with parkinsonism and dementia may be classified as tauopathies or sinucleinopathies, based on their aggregates of abnormal proteins There are no biologic markers at the moment that allow the diagnosis of the various disease that start with parkinsonism or dementia, and their clinical diagnosis may be a challenge Present vision (Litvan 2003)

5. Parkinsonism with dementia • tauopathies (PSP, Pick disease) • synucleinopathies (Parkinson, dementia with Lewy bodies • Drug-induced (combination of drugs, anti-cholinergics, or dopaminergics) • Infeccious (Creutzfeldt-Jakob, HIV) • Vascular

6. Parkinsonism with dementia • Toxic (Wilson, manganese) • Tumoral (primary, secondary, chronic subdural hematomas) • Normal pressure hydrocephalus • Post-traumatic (dementia pugilistica) • Sleep apnoea

7. synucleinopathies • Parkinson’s • akynesia; postural disturbance with axial involvemente, rigidity, response to L-PODA • Lewy body disease • Demential more proeminent • More rapidly progressive • N response to l-DOPA

8. Familial Frontotemporal lobe dementia with parkinsonism associated with chromosome- 17 Frontal behaviour (disinhibition, isolation, disfunction executive aphasia) parkinsonism tauopatias

9. Typical Parkinson’s patient • 60 year-old, male, non-smoker, brought by family or refered by clinician due to • Slowness • Lack of volition, apparent sadness • Motor difficulty with every day activities • Sleep disturbances

10. Typical Parkinson’s • Consults other physicians because • Labyrinth (dizzines, postural instability, gait difficulty, apparent lack of balance) • Vertebral column: lumbar pain, difficulty moving legs

11. Physical Examination • Posture: parkinsonian • Gait: parkinsonian • Slowness of movemento: rigidity • Lack of movement: akinesia • Tremor • Asymetric signs

12. On physical exam • Cardiovascular, respiratory, abdominal, head, neck, limbs: normal • Movement + thought: slow

13. Diagnosis: therapeutic test • Response to l-DOPA • Immediate • Dose-dependent • 3/3h • ¼ de 250mg

14. Medical diagnosis • Systemic investigation normal • Neuroimaging normal • CT • MRI

15. Diagnosis functional • Neuroimaging functional: normal • SPECT • PET • EEG with mapping of alpha at low normal

16. Functional diagnosis • IQ + Memory normal • WAIS • Weschler Memory Scale • Minimental

17. Natural history until 80s • 1-2 years before diagnosis • 5 years good response to L-DOPA • 5 years partial incapacity with multiple drugs • 2-3 years with terminal incapacity • Dysphagia + aspiration

18. Natural history after the 80s • 1-2 years before diagnosis • 5+ years good response to post-DA stimualtors : pramipexole • 5+ years good response to small doses of L-DOPA given at short intervals or SR + multiple drugs • 5 years partial incapacity with multiple drugs • 2-3 years with terminal incapacity • Dysphagia + aspiration

19. História natural após ano 2000 • 12 anos de diagnóstico, resposta a estimuladores pós sinápticos : pramipexole, pequenas doses de L-DOPA ou SR + múltiplos medicamentos • 5 anos de incapacidade parcial com múltiplos medicamentos ou estimulador de gânglios da base com retorno quase ao estado inicial, em pacientes com menos de 70 anos • 2-3 anos de incapacidade terminal • Disfagia + broncoaspiração

20. Multiple drugs • Tricyclics, venlafaxine, bupropione • entacapone • quetiapine • Avoid anticholinergic effect • Avoid depressive effect

21. Environmental treatment • Collection of cars versus mechanic • Ballroom dancing, snooker, tricot • Wedding invitations, model ships and airpplanes • Physical exercise • Extremely healthy life • Repetitive routine with novel fine and physical motor and mental acitvities

22. Essential tremor • Familial ou episodic • Rapid, action, symetric, diffuse • Propranolol, alcohol, phenobarbitone • Caffeine, dopaminergic substances • Benign • Cigarrete

23. Dystonias • Tardive dyskinesia • Psychogenic dyskinesia • Focal dystonia • Facial hemispasm • Generalized dystonia • Dystonic cerebral palsy

24. Choreas • Sydenham • pregnancy • Huntington • Drug induced • Antipsychotic • Metochlopramide • Fluoxetine • L-DOPA

25. Chorea, dyskinesia, dystonia • Botox • Anticholinergics • Mood stabilizers • DA blockers • Benzodiazepines