Movement Disorders

Question 1

Movement disordered are classified as____

Answer 1

bradykinetic
or
hyperkinetic

Question 2

Examples of bradykinetic disorders/ akinetic rigid syndromes

Answer 2

MOST COMMON: parkinsonism

idiopathic parkinson’s disease

postencephalitic

toxin-induced

drug-induced

MPTP

Question 3

Describe Idiopathic Parkinson’s Disease

Answer 3

depletion of dopamine in nigrostriatal system–> disrupts balance of dopamine and Ach

Question 4

Cardinal features of Idiopathic Parkinson’s Disease

Answer 4

Tremor
–> resting “pill-rolling”, unilateral at first

Rigidity
–> increased resistance to passive movement, “cogwheel”

Bradykinesia
–> slow movement, difficulty initiating movement

Question 5

Other, noncardinal features of Idiopathic Parkinson’s Disease

Answer 5

postural instability

hypophonia

masked face, decreased eye blink

festinating gait (slow, small steps that accelerate)

dysautonomia (orthostatic hypotension, incontinence, impotence, anhydrosis)

dementia

Question 6

What has loss of voluntary control of eye movements, especially vertical gaze, with bradykinesia and rigidity?

Answer 6

Progressive Supranuclear Palsy (PSP)

Question 7

What has PRONOUNCED autonomic dysfunction with bradykinesia and rigidity?

Answer 7

Multiple Systems Atrophy (MSA/Shy-Drager Syndrome)

Question 8

What is characterized by cortical and basal ganglionic dysfunction, has bradykinesia and rigidity, with cortical sensory loss, apraxia, myoclonus or aphasia?

Answer 8

Cortical Basal (Ganglionic) Degeneration (CBD)

Question 9

Why do you give antiparkinsonian meds to akinetic rigid syndromes?

Answer 9

they have little effect but no other therapy exist for PSP, MSA or CBD

Question 10

General antiparkinsonian treatment

Answer 10

dopamine agonist
levodopa
COMT inhibitor
anticholinergics
MAOB inhibitor
amantadine
surgery (deep brain +)

Question 11

Describe movements seen in hyperkinetic movement disorders

Answer 11

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

Question 12

Disorders with hallmark chorea

Answer 12

Huntington’s Disease

Sydenham’s Chorea

Question 13

Disorders with hallmark dystonia

Answer 13

Idiopathic Torsion Dystonia

Focal Torsion Dystonia

Question 14

Describe Huntington’s Disease

Answer 14

gradual onset and progression of chorea and dementia d/t CAG repeats on Chr 4

AD, onset 30-50 with 15 year lifespan

Question 15

Describe Sydenham’s Chorea

Answer 15

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

Question 16

Describe Idiopathic Torsion Dystonia

Answer 16

dystonic movements and postures

AD, AR or X-linked

childhood or later onset but remains throughout life

Question 17

Clinical findings in Idiopathic Torsion Dystonia

Answer 17

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

Question 18

Treatment for Idiopathic Torsion Dystonia

Answer 18

low dose levodopa
anticholinergics
benzos
neuroleptic drugs
baclofen
carbamazepine

Question 19

Describe Focal Torsion Dystonia

Answer 19

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)

Question 20

Describe Wilson’s disease

Answer 20

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

Question 21

Clinical findings of Wilson’s disease

Answer 21

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

Question 22

Treatment of Wilson’s disease

Answer 22

Penicillamine (copper chelating agent)

restriction of dietary copper

Question 23

Disorders with hallmark of tic

Answer 23

Gilles De La Tourette’s Syndrome

Question 24

Describe Gilles De La Tourette’s Syndrome

Answer 24

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

Question 25

Describe essential tremor/ benign familial tremor

Answer 25

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