Tuesday, 8-23-Clinical Conference #6-Stephens

Question 1

Unilateral lesions of the cerebellum result in __ deficits

Answer 1

ipsilateral

Question 2

midline lesions of the cerebellar vermis often affect ___

Answer 2

axial musculature

Question 3

Chronic ingestion of ethanol may cause a cortical atrophy of the __ of the cerebellum.

Answer 3

anterior lobe

Case 9239-Alcoholic cerebellar degeneration

Lecture: Vestibular system and cerebellum, p.28

Question 4

This cerebellar dysfunction is characterized by a broad-based staggering gait that initially develops in the lower limbs and progresses to the upper limbs over the course of a few years. Dysdiadochokinesia, dysmetria, nystagmus and intention tremor are present bilaterally. Death occurs 10-20 yrs after onset from cardiac or pulmonary complications

Answer 4

Friedrichs ataxia

Case 9237-Friedrichs ataxia

Lecture: Vestibular system and cerebellum, p. 28

Question 5

a 9 yr old boy has a CC of severe headache, double vision, trouble walking. Exam reveals bilateral papilledema, bilateral paresis of gaze, tilting of the head to the left, Romberg test positive to the left, and wide-based ataxia of the trunk musculature. CT and MRI confirms this diagnosis:

Answer 5

Medulloblastoma of the 4th ventricle

-Case 9212-Medulloblastoma of the 4th ventricle

Question 6

___ leads to LMN paresis and atrophy of the intrinsic muscles of the hands followed later by the arms and shoulder musculature. Pts can develop dysarthria, dysphagia, and paresis of tongue. Involvement of CST leads to spastic paralysis, hyperreflexia, and a Babinski sign. There are no sensory deficits

Answer 6

ALS

-Case 9216 and 9220-ALS

Question 7

What are signs and symptoms of unilateral lesions of the vestibular system?

Answer 7

• Postural impairment
• Eyes, head and body tend to turn toward the side of the lesion
• Pt tends to fall toward side of lesion
• Nystagmus toward side of lesion
• Vertigo
• Visceral disturbances, i.e., nausea, vomiting, pallor

Question 8

Nausea, vomiting, postural impairment, and nystagmus indicates __ involvement

Answer 8

vestibular

Question 9

dysmetria, ataxia, dysdiadochokinesia, and intention tremor indicates ___ involvement

Answer 9

cerebellar

Question 10

Babinski, spastic paralysis, decorticate rigidity indicate __ involvement

Answer 10

pyramidal

Question 11

dyskinesia, chorea, dystonia, rigidity, and tremors at rest indicate ___ involvement

Answer 11

extrapyramidal

Question 12

a 4 yr old girl presents with vomiting and has become “unsteady” in her walking. She has become increasingly irritable and over last several months has lost her balance and fallen frequently. The neuro exam reveals significant truncal ataxia and nystagmus. She is walking with her feet wide apart and staggering from side to side. The most likely dx?

Answer 12

Cerebellar medulloblastoma

-Case 9235-Cerebellar medulloblastoma

Question 13

Korsakoff syndrome involves bilateral destruction of the __ and the __ nucleus of the thalamus

Answer 13

mammillary bodies

dorsal medial

Question 14

a 50 yr old female present with trembling hands and general limb stiffness. During the exam, she has a masked facial expression and stare, shuffling gait, difficulty in initiating movements, pill-rolling tremors of both hands, and bilateral rigidity of the limbs. Most likely dx?

Answer 14

Parkinsons disease

-Case 9241-Parkinsons

Question 15

The neurons in this area of the substantia nigra are destroyed in parkinsons disease

Answer 15

pars compacta

Nigro striatal fibers are dopaminergic fibers that originate in the pars compacta of the substantia nigra and terminate in the caudate and putamen (striatum).

Question 16

These neurons are destroyed in Huntington’s chorea:

Answer 16

Inhibitory GABA-nergic fibers in the striatonigral fibers

Question 17

a 43 yr old former banker shows up with a CC of severe depression for about a year. As the pt walked to the couch, the doc notices that the pts balance seemed a little unsteady. As the pt is discussing his depression, he makes sudden jerky movements with his hands and displayed facial grimacing and rapid tongue movements. Examination between movements revealed a general hypotonia and hyporeflexia. When asked about any hx of depression in his family, the pt recalled an uncle at the age of 50 who divorced his wife, became a recluse, and a year later commit suicide. Most likely Dx?

Answer 17

Huntington’s chorea

-Case 9242-Huntington’s chorea

Question 18

a 69 yr old female widow presents with involuntary movements of her right arm. Upon examination, the doc observes violent flinging movements of her right upper limb, which originated at the shoulder and elbow. Her upper limb muscles appeared almost flaccid and hypotonic between movements. Most likely Dx?

Answer 18

Hemiballism

-Case 9240-hemiballism

Question 19

The principal areas of degeneration in Huntingtons chorea are ___

Answer 19

corpus striatum and cerebral cortex

Question 20

Parkinsons disease is due to degeneration of ___

Answer 20

Substantia nigra, globus pallidus, brainstem RF and postganglionic sympathetic neurons

Lecture: Motor system, p. 23

Question 21

Huntingtons disease is due to degeneration of ___

Answer 21

autosomal dominant disorder (chr 4). Degeneration of corpus striatum and cerebral cortex results in excessive dopaminergic influence in the degenerated striatum or loss of inhibition from the GABAnergic neurons of the striatonigral pathway

Lecture: Motor system, p. 23

Question 22

Hemichorea is unilateral choreiform movements due to degeneration of ___

Answer 22

vascular lesion in the basal ganglia

Lecture: Motor system, p. 23

Question 23

Athetosis is slow, involuntary, writhing movements of the limbs and face ad is due to degeneration of the ___

Answer 23

striatum

Lecture: Motor system, p. 23

Question 24

Hemiballism is contralateral violent, flailing movements of the proximal musculature of both the upper and lower extremities. It is due to degeneration of ___

Answer 24

unilateral lesions of the subthalamus resulting in a reduction or loss of glutamate inhibition upon the globus pallidus