Movement Disorders Flashcards
Movement disordered are classified as____
bradykinetic
or
hyperkinetic
Examples of bradykinetic disorders/ akinetic rigid syndromes
MOST COMMON: parkinsonism
idiopathic parkinson’s disease
postencephalitic
toxin-induced
drug-induced
MPTP
Describe Idiopathic Parkinson’s Disease
depletion of dopamine in nigrostriatal system–> disrupts balance of dopamine and Ach
Cardinal features of Idiopathic Parkinson’s Disease
Tremor
–> resting “pill-rolling”, unilateral at first
Rigidity
–> increased resistance to passive movement, “cogwheel”
Bradykinesia
–> slow movement, difficulty initiating movement
Other, noncardinal features of Idiopathic Parkinson’s Disease
postural instability
hypophonia
masked face, decreased eye blink
festinating gait (slow, small steps that accelerate)
dysautonomia (orthostatic hypotension, incontinence, impotence, anhydrosis)
dementia
What has loss of voluntary control of eye movements, especially vertical gaze, with bradykinesia and rigidity?
Progressive Supranuclear Palsy (PSP)
What has PRONOUNCED autonomic dysfunction with bradykinesia and rigidity?
Multiple Systems Atrophy (MSA/Shy-Drager Syndrome)
What is characterized by cortical and basal ganglionic dysfunction, has bradykinesia and rigidity, with cortical sensory loss, apraxia, myoclonus or aphasia?
Cortical Basal (Ganglionic) Degeneration (CBD)
Why do you give antiparkinsonian meds to akinetic rigid syndromes?
they have little effect but no other therapy exist for PSP, MSA or CBD
General antiparkinsonian treatment
dopamine agonist levodopa COMT inhibitor anticholinergics MAOB inhibitor amantadine surgery (deep brain +)
Describe movements seen in hyperkinetic movement disorders
chorea
–> irregular, brief, dance-like (may incorporate into purposeful movements d/t embarrassment)
athetosis
–> writhing, snake-like movements that often occur w/ chorea
dystonia
–> sustained muscle contractions that produce twisting and repetitive movements, abnormal postures
ballism
–> large amplitude, flinging movement from proximal extremity
tic
–> brief, rapid, repetitive muscle movement
Disorders with hallmark chorea
Huntington’s Disease
Sydenham’s Chorea
Disorders with hallmark dystonia
Idiopathic Torsion Dystonia
Focal Torsion Dystonia
Describe Huntington’s Disease
gradual onset and progression of chorea and dementia d/t CAG repeats on Chr 4
AD, onset 30-50 with 15 year lifespan
Describe Sydenham’s Chorea
mainly in children and adolescents as complication of group A strep
unilateral chorea movements
–>when mild, confused for restlessness or fidgeting
behavioral changes
treatment: bedrest and abx
Describe Idiopathic Torsion Dystonia
dystonic movements and postures
AD, AR or X-linked
childhood or later onset but remains throughout life
Clinical findings in Idiopathic Torsion Dystonia
Torticollis (cervical dystonia)
Blepharospasm (spontaneous closure of eyelids)
Oromandibular dystonia
Arm hyperpronated with wristed flexed and fingers extended
Leg extended with pronation and inversion of foot
Treatment for Idiopathic Torsion Dystonia
low dose levodopa anticholinergics benzos neuroleptic drugs baclofen carbamazepine
Describe Focal Torsion Dystonia
dystonia confined to a focal area
same sx as idiopathic except get writer’s cramp (when performing certain tasks)
botox may help (used in focal because not realistic to inject when it’s all over like idiopathic)
Describe Wilson’s disease
AR disorder of copper metabolism–> NEURO and HEPATIC dysfunction
Chr 13
decreased binding of copper to ceruloplasmin–> free copper deposited in tissues
childhood or young adult
Clinical findings of Wilson’s disease
both brady and hyperkinetic: resting/postural tremor chorea movements rigidity bradykinesia dysarthria/phagia ataxia personality/behavior changes dementia psychosis/hallucinations
DX
-increased copper in urine, decreased serum ceruloplasmin
-Kayser-Fleischer Rings on eye exam
Treatment of Wilson’s disease
Penicillamine (copper chelating agent)
restriction of dietary copper
Disorders with hallmark of tic
Gilles De La Tourette’s Syndrome
Describe Gilles De La Tourette’s Syndrome
chronic multiple motor and vocal tics before age 21
males
can see vulgar/obscene speech, parroting speech of others, imitating others’ movements, repetition of words or phrases
tmt: clonidine, haloperidol, phenothiazines
Describe essential tremor/ benign familial tremor
BL postural/kinetic tremor (can involve head/voice)
usually begins later in life
slow progression
usually doesn’t result in significant disability, but social embarrassment
Alcohol decreases tremor temporarily
tmt: beta blocker, primidone, benzos, topiramate
