Usmle Step 1 Fa Neurology-Basal Ganglia

Front 1

Cause of Parkinson's disease

Back 1

Loss of substantia nigra pars compacta (loss of dopaminergic neurons)

Front 2

Histology seen in Parkinson's disease

Back 2

Loss of pigmented dopaminergic neurons; Lewy bodies (intracytoplasmic eosinophilic inclusions containing alpha synuclein)

Front 3

Clinical manifestations of Parkinson's disease

Back 3

Bradykinesia/akinesia
Cogwheel rigidity
Postural instability (stopped posture)
Shuffling gait
Pill rolling (resting) tremor
Masked faces
Depression
Dementia

Front 4

Known causes of parkinsonism

Back 4

Infections
Vascular
Toxic insults (MPTP)

Front 5

Huntington's disease: genetics/etiology

Back 5

AD trinucleotide repeat disorder (codes for huntingtin protein) leading to atrophy of the caudate nucleus (loss of GABA ergic neurons)
Shows anticipation and genomic imprinting

Front 6

Imaging of Huntington's disease

Back 6

Enlarged lateral ventricles on CT (due to atrophy of the caudate)

Front 7

Clinical manifestations of Huntington's disease

Back 7

Chorea (multiple, rapid, random, movements)
Athetosis (slow writhing movements)
Personality changes
Dementia

Front 8

Typical age of presentation in Huntington's disease

Back 8

20-40YO

Front 9

Hemiballismus
(a) clinical manifestations
(b) cause

Back 9

(a) sudden wild flailing of 1 arm
(b) characteristic of contralateral subthalamic nucleus lesion; loss of inhibition through globus pallidus

Front 10

Chorea

Back 10

Sudden, jerky, purposeless movements

Front 11

Athetosis

Back 11

Slow, writhing movements, esp of fingers

Front 12

Treatment of Huntington's disease

Back 12

Antipsychotic agents, benzodiazepines, anticonvulsants

Front 13

Wilson's disease: genetic defect

Back 13

Autosomal recessive defect in copper transport

Front 14

Neurologic manifesations of wilson's disease

Back 14

Tremor, asterixis, parkinsonian symptoms, chorea, neuropsychiatric symptoms

Front 15

Treatment of Wilson's disease

Back 15

Penicillamine (chelator)
Zinc acetate (blocks absorption)

Front 16

Essential/postural tremor (description, genetics)

Back 16

Action tremor
Autosomal dominant

Front 17

Treatment of essential/postural tremor

Back 17

Alcohol (decr tremor)
Beta blockers

Front 18

Resting tremor descriptions

Back 18

Resting tremor; pill rollingl most noticeable distally

Front 19

Intention tremor (associated w/what region of the brain)

Back 19

Slow, zigzag movement when pointing toward a target
Associated w/cerebellar dysfct