Toxic/Metabolic/Nutritional Disease

Question 1

What is the acute cerebral effect of excessive alcohol?

Answer 1

Cerebral edema

Question 2

What are the chronic cerebral effects of excessive alcohol? (many of them)

Answer 2

Meningeal fibrosis, cortex white matter volume loss, neuronal loss and dendritic reduction

Question 3

What are the chronic cerebellar vermis effects of excessive alcohol?

Answer 3

Folia crests most affected. Granule cell neuronal loss > Purkinje cell neuronal loss.

Question 4

Which toxin plays the biggest role in hepatic encephalopathy?

Answer 4

Ammonia

Question 5

In hepatic encephalopathy, grey matter astrocytes transform into which type of astrocytes and what is the effect of this?

Answer 5

“Alzheimer II” astrocytes - metabolically active astrocytes. This happens especially in the deep cerebral cortex and subcortical structures (globus pallidus especially). Astrocytes become overwhelmed and degenerate leading to encephalopathy.

Question 6

What is Wilson’s disease and what age-group does it affect?

Answer 6

Hepatic failure - disorder of copper metabolism. Young children.

Question 7

What is the inheritance pattern of Wilson’s disease? Which chromosome?

Answer 7

Autosomal recessive. Chromosome 13.

Question 8

What physical exam finding is more predominant than neurologic signs in Wilson’s disease?

Answer 8

Jaundice

Question 9

Wilson’s disease is fatal without treatment. What is the treatment?

Answer 9

Chelating agents?

Question 10

Wilson’s disease causes copper accumulation in the liver. What condition will this then lead to?

Answer 10

Hepatic encephalopathy

Question 11

What is Wernicke’s encephalopathy?

Answer 11

Thiamine (VitB1) deficiency

Question 12

What does the typical triad of Wernicke’s encephalopathy consist of? (in terms of symptoms and physical exam findings)

Answer 12

Ataxia, nystagmus, and confusion

Question 13

Alcoholics are very prone to deficiency of what vitamin?

Answer 13

Thiamine (Vitamin B1)

Question 14

Why can sudden glucose intake precipitate the problem of thiamine deficiency?

Answer 14

Thiamine is used as a cofactor in glucose metabolism

Question 15

What are the common anatomical brain sites affected by Wernicke’s encephalopathy (thiamine deficiency)?

Answer 15

Mammillary bodies > walls of 3rd ventricle > periaqueductal tissue > inferior colliculi > floor of 4th ventricle > thalamus

Question 16

What are the acute neuropathological findings of Wernicke’s encephalopathy?

Answer 16

Macro/micropetechial hemorrhages with capillary dilation, demyelination, microglial and macrophage influx, and fibrous gliosis

Question 17

What are the late neuropathological findings of Wernicke’s encephalopathy?

Answer 17

Neuronal loss, hemosiderin, mammillary body atrophy

Question 18

What is the treatment for Wernicke’s encephalopathy?

Answer 18

Administration of thiamine!

Question 19

Cobalamin (VitB12) is found in what foods?

Answer 19

Meat and dairy

Question 20

In the stomach, VitB12 is bound to what?

Answer 20

Intrinsic factor

Question 21

Cobalamin deficiency often causes what type of anemia?

Answer 21

Megaloblastic anemia

Question 22

Does cobalamin deficiency affect the brain or spinal cord?

Answer 22

Cord. Co[balamin]rd.

Question 23

Does spinal cord disease from cobalamin deficiency involve the motor or sensory tract?

Answer 23

Both!

Question 24

What are the initial symptoms of cobalamin deficiency?

Answer 24

Slight ataxia, numbness, tingling in lower extremities which develops into spastic weakness or even complete paraplegia.

Question 25

What is the initial path lesion in cobalamin deficiency?

Answer 25

Spongy vacuolization of cord white matter.

Question 26

What are later path lesions in cobalamin deficiency?

Answer 26

Demyelination, macrophage influx, and axonal degeneration.

Question 27

What is the cause of Central Pontine Myelinolysis (CPM)?

Answer 27

The rapid correction of hyponatremia (iatrogenic)

Question 28

CPM causes focal demyelinating lesions in which anatomical region of the CNS?

Answer 28

Pons