Movement Disorders

Question 1

Progressive Supranuclear Palsy

Answer 1

Parkinsonian syndrome with supranuclear ophthalmoplegia, with limitation of vertical more than horizontal gaze; axial rigidity and neck extension, early falls as a consequence of impaired postural reflexes, neck extension, and an inability to look down.

Question 2

Corticobasal ganglionic degeneration

Answer 2

Parkinsonian syndrome with Apraxia, cortical sensory impairment, and alien limb phenomenon. Severe unilateral rigidity, stimulus-sensitive myoclonus.

Question 3

Diffuse Lewy Body Disease

Answer 3

Parkinsonian syndrome with Early dementia, prominent visual hallucinations, extreme sensitivity to extrapyramidal side effects of antidopaminergic neuroleptic drugs.

Question 4

Vascular parkinsonism

Answer 4

Parkinsonian syndrome with “Lower half” parkinsonism with rigidity in the legs and marked gait impairment; other evidence of diffuse vascular disease (corticospinal tract dysfunction, pseudobulbar palsy)

Question 5

Multiple System Atrophy

Answer 5

Parkinsonian syndrome with Early and prominent features of autonomic dysfunction; evidence of corticospinal tract dysfuntions, cerebellar signs, stimulus-sensitive myoclonus, vocal cord paresis.

Question 6

stiff person syndrome

Answer 6

characterized by chronic axial muscle rigidity and stiffness with superimposed painful muscle spasms. May be assc’d with anti-GAD antibodies.

Question 7

tremor

Answer 7

1) Resting– idiopathic parkinson’s disease, other parkinsonian syndromes.
2) Postural– essential tremor, physiological tremor, drugs (theophylline, beta-agonists), alcohol
3) action– cerebellum and cerebellar outflow tract dysfunction (eg, infarction, MS, tumor, Wilson’s disease, drugs)

Question 8

chorea

Answer 8

comprises involuntary abrupt and irregular movements that flow randomly from one body part to another.

Huntington’s disease, poststreptococcal infection, SLE, thyrotoxicosis, and pregnancy are among the more common causes of chorea.

Question 9

dystonia

Answer 9

sustained muscle contraction leading to repetitive twisting movements or abnormal postures.

Idiopathic torsion dystonia is a familial condition that may manifest as torticollis, writer’s cramp, blepharospasm, or spasmodic dysphonia.

Question 10

myoclonus

Answer 10

myclonus is a sudden, lightening-like movement produced by abrupt and brief muscle contraction.

Clonazepam is the most effective treatment for many patients.

Question 11

Tics

Answer 11

Abrupt, sterotyped, coordinated movements or vocalizations.

(tourette’s–common genetic disorder w/ onset of motor and vocal tics in childhood)

Dopamine antagonists are often the most effective therapy for tics.

Question 12

Wilson’s Disease

Answer 12

a disorder of copper metabolism.

Hyperkinetic and hypokinetic movement disorders as well as cognitive personality and mood disturbances are the most common neurologic manifestations of WD.

Kaiser-fleisher rings represent copper deposition in the cornea.

elevated serum copper levels, low cerulplasmin levels, and elevated 24 hour urinary copper are useful screening tests.