By Craig Brooker, M.D.

Parkinsonism Tremor Myoclonus Chorea Tics Dystonia Stereotypies RLS/PLMS

Idiopathic Parkinson’s Disease Dementia with Lewy Bodies Multiple System Atrophy Progressive Supranuclear Palsy Corticobasal Degeneration Vascular Parkinsonism Post-Traumatic Parkinsonism Secondary (Toxin, Meds, Metabolic)

Indian Medicine ~2,500BC: Kampavata “kampa” (shaking) “vata” (lack of muscle movement); tx’d w/ Mucuna plant, has L-dopa in it

1817 James Parkinson “Essay on Shaking Palsy”

Tretiakoff 1919 noted loss of neurons of substantia nigra

Usually asymmetric Cardinal Signs

Tremor (resting, “pill rolling”) Rigidity (cogwheel) Bradykinesia Postural instability/Gait abnormalities

L-dopa responsive Later findings: depression, dementia

Often tremor Sometimes just poor gait or falls Usually > 70 yo; 1% of pop > 60yo; Only

4% of parkinson’s patients dx’d <50yo Males > females + family hx in 25%; 13 different “PARK”

genes, auto dom, recessive, & x-linked

Exam: Do full neuro exam (www.NeurologyExam.com)

Also look for decreased eye blinking, mask face, clumsiness of alternating movement, bradykinesia, slow talking, hypophonia, cogwheeling rigidity, micrographia, difficulty rising without pushing, freezing, stooped posture, reduced arm swinging, shuffling gait, en bloc turning, festination/retropulsion, & + glabellar tap.

Also do tremor exam, expect pill rolling tremor at rest > posturally or action, usu ok water pouring (unlikely essential tremor)

Conservative mgt ok until disability Dopamine & its agonist:

L-dopa & carbidopa (sinemet); often therapeutic trial

Ropinirole (Requip) Pramipexole (Mirapex)

Some argue L-dopa sparing treatment early or <60yo

“Wearing off” phenomenon: increase frequency

Decreased efficacy with time: increase dose

Dyskinesias: extra movements L-dopa has most dyskinesias but is also

most effective

Deep Brain Stimulation: subthalamic nucleus & Globus Pallidus Only for patients responsive to L-dopa Will never be more effective than L-dopa Useful for patients who have SE on L-dopa (e.g. esp

dyskinesias) Often reserved for those < 70yo or so & good surgical

candidates Complications:

Brain surgery Bleed Infections acutely or chronically Device needs adjustments esp early on, very specialized

care Some still require medications, though usu lesser dose Can’t get MRIs after DBS

“Differentiated” by hallucinations, usually visual

Dementia may occur sooner than PD Differentiation is somewhat arbitrary May be L-dopa responsive

Differentiated from PD: early Autonomic failure (low BP) Poorly responsive to L-dopa Cerebellar signs and/or long tract signs Often little or no tremor Less cognitive problems usually(Change in nomenclature: MSA now includes

Shy-Drager Syndrome, Olivo-ponto-cerebellar Syndrome, and Striato-Nigral Syndrome)

Differentiated from PD: Vertical supranuclear palsy with down gaze

abnormalities Early falls Symmetric bradykinesia & rigidity; usu no

tremor “pseudobulbar palsy” w/ dysarthria &

dysphagia Dementia has frontal lobe features Poor response to dopamine

Drugs: antipsychotics, metoclopramide, MPTP

Toxins: CO poisoning, cyanide, organic solvents, manganese

Trauma: e.g. boxing (Mohammad Ali) Structural effecting striatonigral pathways

Hydrocephalus, tumor Metabolic: Wilson’s, low parathyroid Infections: encephalitis, PML, toxoplasmosis Stroke: “vascular parkinsonism”

Asymmetric but with poor response to L-dopa

Ideomotor Apraxia, Alien limb Lack of tremor

Common, usu family history May start at very young age, but not

always Usu symmetric, postural and/or action

component >> resting Often with more subtle head, jaw, voice

tremors May be disabling, often respond to EtOH No other features of parkinsonism Tx: Propranolol, primidone; Gabapentin;

DBS (Thalamus)

Usu symmetric in arms, may involve voice

Generally worse based on environmental factors (e.g. social situations, stress, anxiety, stimulants/caffiene, lack of sleep, alcohol withdrawal, many different medications, etc)

Can otherwise be hard to differentiate from essential tremor and they are often both present

Not a tremor Sudden, shock-like muscle contractions Focal, multifocal, or generalized Usu not rhythmic Epileptic or non-epileptic Hypnic jerks (physiological) Juvenile Myoclonic Epilepsy (pathological) Anoxic injury

Chorea: “Dance-like movement” -excessive spontaneous movements -irregular, random, brief, and abrupt -non-repetitive Usu distal predominance; may disguise in

other voluntary movements

Causes: -Post-infectious: Sydenham’s, Group A

strep mimicry; usu 1-8 mo after infection, self-limited

-Huntington’s, MRI: caudate atrophy

Sx: chorea, psychosis, dementia, death Dx: genetic testing, CAG repeat >38,

autodom MRI brain: caudate atrophy Tx: symptomatic

Chorea: tetrabenazine, neuroleptics Psychosis: antipsychotics

No known disease modifying therapy

Semi-voluntary (i.e. suppressible), rapid, non-rhythmic movements and/or sounds

Simple (e.g. blinking, grimacing, coughing) or complex

May have OCD too Tourette’s: vocal and motor tics 96% dx by age 11, most resolve by 18yo Tx: Tetrabenazine to decrease dopamine

(or antipsychotics) or CBT for Tics

Involuntary muscle contractions that cause slow repetitive movements or abnormal postures

E.g. writer’s cramp, cervical dystonia, blepharospasm, musicians; Focal, generalized,

Usu idiopathic, rarely DYT1 or 2ndary Tx: botox, Benztropine (anti-Ach),

dopamine sometimes useful, benzos; occasionally DBS (Gpi)

Repetitive, purposeless movements (no urge unlike tics)

Mostly peds; esp with autism, MR, Common: head nodding, rocking, arm

flapping, etc. Etiology: believed psychological, self

reward/stim

4 criteria for dx: Unpleasant sensation(“creeping, crawling,

pulsing”) Urge to move legs Worse at night (while awake) or at rest Improved by movement

Reversible causes: low ferritin (<50mg/dl), SSRIs

Other causes: hereditary, idiopathic, 2ndary to neuropathy

Tx: dopamine agonists, gabapentinComplications: dopamine agonists (augmentation)

Involuntary movements of legs during sleep

May be comorbid with RLS Debatable as to whether it causes

excessive daytime sleepiness (EDS) unto itself

Not usually treated unless no other cause for EDS can be found & patient is desperate

Often a 1st sign of Parkinson’s Incomplete loss of tone during REM Classically, acting out dreams (usu being

chased or fighting) Men > women Diff Dx: “Pseudo-RBD” (i.e. OSA), 2ndary

RBD (SSRIs, EtOH withdrawal, sedative withdrawal)

Tx: padded sleep environment, correct 2ndary causes, consider clonazepam, gabapentin

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