Systemic and Metabolic Disorders

Question 1

What will an EEG typically show with a toxic-metabolic encephalopathy?

Answer 1

slowing of the background rhythm

Question 2

What is the cardinal clinical feature of encephalopathy?

Answer 2

waxing and waning level of consciousness

Question 3

What is a frequent physical exam finding in encephalopathy?

Answer 3

asterixis, which is an example of negative myoclonus

Question 4

Sarcoidosis only affects the CNS in about 5% of cases. What is the most common presentation?

Answer 4

cranial neuropathy due to chronic basal meningitis with the facial and optic nerves most frequently affected

Facial neuropathy may occur due to parotid inflammation

visual changes are common due to direct involvement of the optic nerves

Question 5

What is the typical pathology of sarcoidosis?

Answer 5

noncaseating granuloma

Question 6

We usually don’t like to do brain biopsies to confirm neurosarcoid, but what will CSF analysis show?

Answer 6

elevated protein and lymphocytic pleocytosis

CSF ACE concentration is difficult to interpret, but serum ACE concentration will be high

Question 7

What is the most common neurological complication of diabetes?

Answer 7

peripheral neuropathy

Question 8

Describe the typical presentation of peripheral neuropathy

Answer 8

distal symmetric sensory polyneuropathy affecting temperature and pinprick sensation first (2/2 predilection for small myelinated and unmyelinated fibers)

Question 9

An associated autonomic neuropathy is common. What are some of the symptoms?

Answer 9

gustatory sweating, orthostatic hypotension, diarrhea and impotence

neurogenic bladder and gastroparesis occur less frequently

Question 10

What are some acute neurological effects of hyperglycemia?

Answer 10

seizures
transient painful peripheral neuropathy that resolves with correction of the hyperglycemia
chorea

Question 11

What is the clinical triad seen in Wernicke’s encephalopathy?

Answer 11

ophthalmoplegia
truncal ataxia
confusion

Question 12

Gait ataxia greater than limb ataxia and dysarthria in a long-time alcoholic usually is caused by what?

Answer 12

cerebellar degeneration

Question 13

Describe the vision change that occurs with tobacco-alcohol amblyopia?

Answer 13

an insidious and painless loss of vision in a centrocecal scotoma pattern

Question 14

Alcohol can cause a focal demyelination and necrosis of the corpus callosum leading to frontal-type dementia, seizures and pyramidal signs. What is this syndrome called?

Answer 14

Marchiafava-Bignami syndrome

Question 15

Subacute combined degeneration of the spinal cord results from deficiency of what?

Answer 15

Vitamin B12

Question 16

What happens in subacute combined degeneration of the spinal cord?

Answer 16

degeneration of the posterior and lateral white matter tracts of the spinal cord, so you get paresthesias in the hands and feet with eventual weakness and spasticity in the legs

often associated with a large-fiber peripehral neuropathy and macrocytic anemia

Question 17

What lab tests should you do to check for B12 deficiency?

Answer 17

B12 levels may be normal, so you need to measure levels of serum homocysteine and methylmalonic acid (which are the precursors of B12 and will thus be elevated when B12 is deficiency as the body tries to compensate)

Question 18

What are some of the CNS manifestations of SLE?

Answer 18

psychosis and depression are the most common

also seizures, stroke (bc of being hypercoagulable), transverse myelitis, headache and chorea

Question 19

What is the most common PNS manifestation of SLE?

Answer 19

distal sensory polyneuropathy

Question 20

What are the major features of antiphospholipid syndrome?

Answer 20

venous and arterial thrombosis (more often venous)
recurrent fetal loss
thrombocytopenia

Question 21

What is the management for antiphospholipid syndrome?

Answer 21

long-term warfarin to INR of 3-4