PARKINSONIAN SYNDROMES MiMDA Annual Meeting September, 2016 Diane Parsons, MD indigo Palliative Care and Neurology Consultant
Disclosures I have no financial disclosures. I may discuss off label use of medications.
Objectives Review features of tremor Discuss Parkinsons Disease Discuss atypical parkinsonisms Red Flags, Mimics Management of symptoms
Parkinsonian Syndromes Multisystem disorders BRADYKINESIA, akinesia Rigidity Resting tremor Postural instability, +/- falls Motor and non-motor symptoms
Diagnosing PD and parkinsonisms No blood, CSF, or imaging markers Diagnosis based on clinical symptoms and signs Medication review
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
Parkinsons Disease Bradykinesia, resting tremor, rigidity, postural instability (cardinal features) Onset: Often in 7 th decade, deceasing incidence after age 75 Survival 10-20 years Most common form of parkinsonian syndrome: 85% Non-motor symptoms often most debilitating aspect in later stages
Parkinsons Disease REM Sleep behaviors Micrographia Chronic constipation Hypophonia Loss of sense of smell Masked facies Fatigue Dizziness/ fainting Gait changes Dementia Neuropsychiatric issues
Mimics Drug- induced PD Lower-half Parkinsons PD vs. parkinsonism Alzheimer s normal aging Essential tremor
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
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
Medications that cause extrapyramidal syndromes Antipsychotics Metaclopramide/compazine Reserpine Some calcium channel blockers Stimulants; amphetamines, cocaine Usually resolves after offending agent discontinued
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
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
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
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
PSP Wheelchair sign Rocket sign Applause sign No pharmacologic treatment for PSP Supportive treatment
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
MSA-C Gait and balance impairments Limb ataxia Staccato speech or dysarthria Nystagmus May evolve to resemble MSA-P Olivo-ponto-cerebellar atrophy
Other MSA Signs and Symptoms Sleep disturbances Autonomic failure Respiratory dysfunction Orthostatic hypotension Early neurogenic bladder symptoms Pisa syndrome, camptocornia
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
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
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
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
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
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
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
Advanced PD Hoehn and Yahr 3.0-4.0 3.0: 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
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?
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
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
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
Guidelines: Off Time Level A: entacapone, rasagiline Level B: pramipexole, ropinerole Level C: apomorphine subq, selegiline
Guidelines: Dyskinesia Level C: amantadine Level B: DBS subthalamic nucleus Level U: DBS globus pallidus interna, DBS ventral intermediate nucleus of thalamus