Nutritional disorders

Question 1

Name the Vitamin deficiency associated with the following finding (s):

Hemorrhagic or shrunken maxillary bodies

Answer 1

Thiamine (B1) deficiency

Wernicke-Korsakoff syndrome

Question 2

Name the Vitamin deficiency associated with the following finding (s):

Dermatitis, diarrhea and dementia
Prominent chromatolysis of Betz cells and dentate nuclei (cerebellum)

Answer 2

Nicotinic acid (B3) deficiency

Pellagra

Question 3

Name the Vitamin deficiency associated with the following:

Treatment with isoniazid

Answer 3

Pyridoxine (B6) deficiency

Question 4

Name the Vitamin deficiency associated with the following finding (s):

Demyelination of the dorsal columns and lateral corticospinal tracts

Answer 4

Vitamin B12 deficiency

Question 5

Name the nutritional deficiency associated with the following finding (s):

Neural tube defects

Answer 5

Folic acid

Question 6

Name the Vitamin deficiency associated with the following finding (s):

Dry, hyperkeratotic skin, night blindness and corneal keratinization

Answer 6

Vitamin A deficiency

Question 7

Name the Vitamin deficiency associated with the following finding (s):

Myopathy and increased risk of neuropsychiatric disorders, neurodegenerative disease and multiple sclerosis

Answer 7

Vitamin D

Question 8

Name the Vitamin deficiency associated with the following finding (s):

Degeneration of the posterior spinal , spinocerebellar and corticospinal tracts
Axonal swellings in the gracile funiculi

Answer 8

Vitamin E (alpha-tocopherol) deficiency

Question 9

Name the Vitamin deficiency associated with the following disorders (s):

Ataxia with isolated vitamin ___ deficiency
Bassen-Kornzweig syndrome (abetalipoproteinemia)

Answer 9

Vitamin E deficiency

Question 10

Which area (s) of the hippocampus is most vulnerable to hypoxia?

Answer 10

CA1 and subiculum

Question 11

Which area of the hippocampus is least vulnerable to hypoxia?

Answer 11

CA2

Question 12

Which layer (s) is/are most vulnerable to hypoxia in the neocortex?

Answer 12

Layers 3, 5 and 6

Question 13

Which area the deep nuclei are most vulnerable to hypoglycemia? in infants?

Answer 13

Caudate nucleus and putamen

Infants - dentate nuclei

Question 14

Name the syndrome characterized by pyrexia, encephalopathy and autonomic instability

Answer 14

Neurolepic malignant syndrome

Due to a reaction to neuroleptic and other antidopaminergic drugs

Question 15

Condition associated with a hyper metabolic response to inhalation anesthetics and depolarizing muscle relaxants

Answer 15

Malignant hyperthermia

Question 16

Receptor affected in malignant hyperthermia

Answer 16

Ryanodine receptors calcium-release channel (gene on chromosome 19q13)

Question 17

Name the metabolic derangement associated with osmotic demyelination syndrome

Answer 17

Hyponatremia

Question 18

Brain structure most commonly affected in osmotic demyelination syndrome

Answer 18

Pons (central pontine myelinolysis)

Question 19

Condition associated with extensive idiopathic calcification of the basal ganglia

Answer 19

Fahr disease

Question 20

Condition associated with extensive idiopathic calcification of the basal ganglia and neurofibrillary tangles

Answer 20

Diffuse neurofibrillary tangles with calcification (DNTC)

Question 21

Most common cause of hypercalcemic encephalopathy

Answer 21

Primary hyperparathyroidism

Question 22

Metabolic derangement associated with posterior reversible encephalopathy syndrome (PRES)

Answer 22

Hypercalcemia

Question 23

Syndrome associated with posterior cortical and subcortical lesions

Answer 23

Posterior reversible encephalopathy syndrome (PRES)

Question 24

Alzheimer type II astrocytes are commonly associated with which organ dysfunction?

Answer 24

Liver failure

Hepatic encephalopathy

Question 25

What is the common morphologic of Alzheimer type II astrocytes seen in the cerebral cortex, striatum, thalamus and hypothalamus?

Answer 25

Round

Question 26

What is the common morphologic of Alzheimer type II astrocytes seen in the palladium, subthalamic, dentate nucleus and brainstem

Answer 26

Irregularly shared nuclear borders

Question 27

Name the 2 conditions commonly associated with the presence of Opalski cells

Answer 27

Hepatic encephalopathy

Wilson’s disease (hepatolenticular degeneration)

Question 28

A mutation in ATP7B is associated with which metabolic disease

Answer 28

Wilson’s disease

Chromosome 13q14.3

Question 29

Which portion of the putamen particular appears brown and shrunken in Wilson’s disease?

Answer 29

Middle portion

Imparts a “Cupid’s bow” appearance

Question 30

Where are Opalski cells most prominent in Wilson’s disease?

Answer 30

Globus pallidus

Question 31

Disorder due to loss of ferroxidase activity of ceruloplasmin

Answer 31

Aceruloplasminemia

Question 32

What is the CNS presentation of Reye syndrome?

Answer 32

Acute NON-inflammatory encephalopathy

Question 33

Name the 5 porphyrias that can cause neurologic disease

Answer 33

1. ALA dehydratase porphyria (ALA dehydratase)
2. Acute intermittent porphyria (HMB synthase)
3. Porphyria cutanea tarda (Uroporphyrinogen)
4. Hereditary coproporhyria (Coproporhyrinogen)
5. Varigate porphyria (Protoporphyrinogen)

Question 34

What is the most consistent neuropathologic abnormality seen in celiac disease?

Answer 34

Cerebellar atrophy with loss of Purkinje cells, Bergmann gliosis and loss of loss of granule cells

Question 35

Which cells are often prominent in uremic encephalopathy?

Answer 35

Alzheimer type II astrocytes

Question 36

Encephalopathy associated with multiple foci of necrosis with calcification predominantly in the white matter

Answer 36

Multifocal necrotizing leukoencephalopathy