PARKINSONIAN SYNDROMES

Transcription

1 PARKINSONIAN SYNDROMES MiMDA Annual Meeting September, 2016 Diane Parsons, MD indigo Palliative Care and Neurology Consultant

2 Disclosures I have no financial disclosures. I may discuss off label use of medications.

3 Objectives Review features of tremor Discuss Parkinsons Disease Discuss atypical parkinsonisms Red Flags, Mimics Management of symptoms

4 Parkinsonian Syndromes Multisystem disorders BRADYKINESIA, akinesia Rigidity Resting tremor Postural instability, +/- falls Motor and non-motor symptoms

5 Diagnosing PD and parkinsonisms No blood, CSF, or imaging markers Diagnosis based on clinical symptoms and signs Medication review

6 Tremors Resting/Parkinsons Action/Essential Tremor Resting, rotatory, sustention With action, flexion/extension Resolves with action, reemergent Gait and postural changes Progression of signs and symptoms Arms, legs, chin, lips or tongue Asymmetric (Generally) resolves at rest Minimal/mild gait problems May be quite stable/although can be disabling Arms, voice, head, legs Usually symmetrical

7 Parkinsons Disease Bradykinesia, resting tremor, rigidity, postural instability (cardinal features) Onset: Often in 7 th decade, deceasing incidence after age 75 Survival years Most common form of parkinsonian syndrome: 85% Non-motor symptoms often most debilitating aspect in later stages

8 Parkinsons Disease REM Sleep behaviors Micrographia Chronic constipation Hypophonia Loss of sense of smell Masked facies Fatigue Dizziness/ fainting Gait changes Dementia Neuropsychiatric issues

9 Mimics Drug- induced PD Lower-half Parkinsons PD vs. parkinsonism Alzheimer s normal aging Essential tremor

10 Lower Half Parkinsons cc: difficulty walking H/o falls, looks fine seated Lacunar state/vascular parkinsonism NPH Primary gait disorder, gait ignition failure Cervical spondylosis/mimic, but with prominent pyramidal features

11 Drug Induced Parkinsons May look exactly like PD With buccal-lingual chorea, dystonia, or akathisia suspect neuroleptics or metaclopramide Resolves If still present in one year, assume idiopathic PD possibly exacerbated by medication

12 Medications that cause extrapyramidal syndromes Antipsychotics Metaclopramide/compazine Reserpine Some calcium channel blockers Stimulants; amphetamines, cocaine Usually resolves after offending agent discontinued

13 Parkinsonism of Normal Aging Community study; ~30% between ages 75-84, > 50% in ages over 85 Resembles Lower-half parkinsonism Caution with PD diagnosis at onset > 75, absence of rest tremor, symmetrical findings, and lack of responsiveness to levodopa

14 Atypical Parkinsonian Syndromes Progressive Supranuclear palsy Multisystem atrophy: MSA-P, MSA-C Corticobasal degeneration Dementia with Lewy bodies Clinical phenotypes overlap leading to delayed or misdiagnosis

15 Features of parkinsonisms Poor response to dopamine Early loss of balance Prominent cognitive features Rapid progression Significant postural hypotension Sphincter dysfunction/incontinence Little/no tremor

16 Progressive Supranuclear Palsy Onset > 50 yo, mean survival 5-8 years, 5/100k More rapid progression Early imbalance/falls, truncal rigidity Loss of eye movements Dysarthria and dysphagia Dementia occurs later May have brief response to dopamine

17 PSP Wheelchair sign Rocket sign Applause sign No pharmacologic treatment for PSP Supportive treatment

18 MSA- P Bradykinesia, autonomic dysfunction, ataxia, rigidity Initially difficult to distinguish from PD More rapid progression, mean survival 6-9 years Occasional poor/transient response to levodopa Red flag: dyskinesia and/or early orofacial dystonia on levodopa Shy-Drager, Striato-nigral Degeneration

19 MSA-C Gait and balance impairments Limb ataxia Staccato speech or dysarthria Nystagmus May evolve to resemble MSA-P Olivo-ponto-cerebellar atrophy

20 Other MSA Signs and Symptoms Sleep disturbances Autonomic failure Respiratory dysfunction Orthostatic hypotension Early neurogenic bladder symptoms Pisa syndrome, camptocornia

21 Cortico-basal Degeneration Least common, overlaps FTD, PPA, AD, PSP Onset > 60, mean survival time 7 years Rapidly progressive Asymmetric early bradykinesia, rigidity, limb dystonia, postural instability, language disturbance, disabling apraxia of affected limb, alien limb No effective treatment

22 Vascular parkinsonism A form of lower-half parkinson s Caused by multiple small strokes Gait changes more prominent than tremor Abrupt onset, >70-75 years of age Static course, or slow stepwise progression Dopamine not of great benefit

23 Dementia with Lewy Bodies Memory loss early/precedes or coincides with gait issues Prominent hallucinations Fluctuations in attention and cognition Cognitive changes; attention, executive function, visual-spatial difficulties Benefit from memory enhancing medications Sensitivity to neuroleptics

24 Treatment for Parkinsons levodopa: gold standard agonists: pramipexole, ropinerole anticholinergics for tremor amantadine selegilene or rasagiline/mao-b inhibitors, apomorphine DBS PT/OT/Speech Therapy

25 PD Treatment Dopaminergic treatment Avoid neuroleptics Non-motor symptoms need aggressive treatments Constipation Behavioral symptoms/quetiapine, antidepressants, anxiolytics Dopamine Dysregulation Syndrome: increases doses, impulse control disorders

26 Guidelines for Treatment of Autonomic Symptoms Sexual dysfunction: sildenafil citrate, Level C Orthostatic hypotension: Level U Urinary incontinence: Level U GI symptoms: PEG, Level C Sialorrhea: botox injections, Level B

27 Guidelines Depression: amitriptyline, Level C Psychosis: clozapine, Level B olanzapine should NOT be considered, Level B quetiapine, Level C Dementia: donepezil, Level B rivastigmine, Level B

28 Advanced PD Hoehn and Yahr : mild-moderate, postural instability, independent 4.0 severe disability, stand/ambulate unassisted 25% Medicare PD in LTC (2002) MoCA 23.5/30 12% on cognitive enhancing meds Support for spouse, caregiver, family

29 Advanced PD Optimize dopaminergic treatment (decrease?) Pain: pharm- and non-pharmacologic treatments Constipation: PEG po or PEG suppository Bladder issues: clock void, +/- Rx Orthostatic hypotension: 16 oz cold water AM and after meals, +/- meds Dysphagia: swallowing exercises, feeding tube?

30 Advanced PD Educate/support resident and family Palliative Care Advance Directives Consider Hospice when PPS 40% or less, FAST 7C or worse, unable to maintain nutritional status/losing weight, low albumin, recurrent infections, falls, repeated ER visits/hospital admissions

31 Summary Parkinsonisms/PD are multisystem disorders with significant morbidity and decreased survival time Motor and non-motor symptoms contribute to morbidity and mortality No curative treatment, but supportive care available and valuable Benefits to early Advance Directives discussions

32 Guidelines Sleep: Level U Excessive daytime sleepiness: modafinil, Level A RLS/PLMS: levodopa, Level B REM sleep behavior disorder: Level U Fatigue: methylphenidate, Level C Anxiety: Level U

33 Guidelines: Off Time Level A: entacapone, rasagiline Level B: pramipexole, ropinerole Level C: apomorphine subq, selegiline

34 Guidelines: Dyskinesia Level C: amantadine Level B: DBS subthalamic nucleus Level U: DBS globus pallidus interna, DBS ventral intermediate nucleus of thalamus