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Metabolic Neurologic Disorders Flashcards

1
Q

Subacute combined degeneration is caused by a deficiency of this

A

B12

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2
Q

Homocystine and Methylmalonyl CoA are elevated in this condition

A

Subacute combined degeneration

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3
Q

Do symptoms of Subacute combined degeneration (typically paresthesias) first occur proximal or distally?

A

Distal / peripheral extremities first (feet, hands)
then ascends

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4
Q

These 2 spinal tracts are affected in Subacute combined degeneration

A

Dorsal columns and Corticospinal tracts

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5
Q

Is hemoglobin low or high in subacute combined degeneration?

A

Low

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6
Q

Low hemoglobin, macrocytic anemia, and hypersegmented neutrophils are seen in this condition

A

Subacute combined degeneration

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7
Q

Anterior superior cerebellar atrophy and Sensorimotor peripheral neuropathy are caused by this

A

Ethanol toxicity (long term abuse)

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8
Q

Thiamine is this vitamin

A

B1

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9
Q

Thiamine deficiency is most common in people with this in the Western hemisphere

A

Alcoholism

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10
Q

Thiamine deficiency is most common in people with this in the non-western hemispheres

A

Nutritional deficiency
(especially with “exclusive” rice diet)

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11
Q

Exclusive rice diet can result in a deficiency of this

A

Thiamine (B1)

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12
Q

Beri Beri disease is due to deficiency in this

A

Thiamine

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13
Q

Main disorder resulting from thiamine deficiency that involves PNS and cardiac muscle

A

Beri Beri

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14
Q

Main disorder resulting from thiamine deficiency that involves CNS

A

Wernicke-Korsakoff

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15
Q

Does Beri Beri disease involve the CNS or PNS?

A

PNS (and cardiac muscle)

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16
Q

Condition characterized by PNS manifestations of thiamine deficiency

A

Beri Beri

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17
Q

“Wet” beri beri occurs when there is involvement of this

A

Heart failure

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18
Q

Beri Beri involves paresthesias/pain beginning in this part of the body

A

Feet

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19
Q

Does Beri Beri disease involve sensory or motor problems?

A

Sensory
(distal sensory loss, paresthesias, pain)

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20
Q

CNS dysfunction due to thiamine deficiency

A

Wernicke-Korsakoff Syndrome

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21
Q

This is the acute form of Wernicke-Korsakoff Syndrome

A

Wernicke encephalopathy

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22
Q

Wernicke encephalopathy is reversible with this

A

Thiamine replacement

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23
Q

Wernicke encephalopathy involves hemorrhage around these structures

A

Cerebral aqueduct, 3rd/4th ventricles
(= causes ophthalmoplegia)
Also thalamus and mamillary bodies
(= causes memory loss)

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24
Q

Why does Wernicke encephalopathy cause ophthalmoplegia?

A

Hemorrhage around aqueduct, 3rd/4th ventricles affect CN III

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25
Q

Why does Wernicke encephalopathy cause memory loss?

A

Hemorrhage in thalamus and mamillary bodies

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26
Q

In Wernicke encephalopathy, hemorrhage in these two structures can cause memory loss

A

Thalamus and mamillary bodies

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27
Q

Is Wernicke encephalopathy slow or rapid onset?

A

Rapid

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28
Q

This is the classic triad of Wernicke encephalopathy

A

Confusion with memory loss (delirium)
Ophthalmoplegia
Gait ataxia

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29
Q

This is the chronic form of Wernicke-Korsakoff Syndrome

A

Korsakoff syndrome

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30
Q

Is Korsakoff syndrome reversible?

A

No

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31
Q

Korsakoff syndrome is due to chronic, repetitive injury to this structure

A

Mamillary bodies
(and possibly cerebellum/PNS)

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32
Q

Encephalopathy seen in end-stage liver disease

A

Hepatic encephalopathy

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33
Q

Hepatic encephalopathy occurs with liver disease, but also may be induced with this procedure

A

Porto-systemic shunting

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34
Q

Pathophysiology of Hepatic encephalopathy is unclear, but is related to this condition

A

Hyperammonemia

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35
Q

This type of encephalopathy is related to Hyperammonemia

A

Hepatic encephalopathy

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36
Q

What organ metabolizes ammonia?

37
Q

Encephalopathy involving this organ will show Alzheimer type II astrocytes

38
Q

This cell type is the pathologic hallmark of Hepatic encephalopathy

A

Alzheimer type II astrocyte

39
Q

High levels of this compound results from hemoglobin degradation and is seen in liver failure

40
Q

Is bilirubin low or high in hepatic encephalopathy?

41
Q

Is albumin low or high in hepatic encephalopathy?

42
Q

Is prothrombin time (PR) low or high in hepatic encephalopathy?

43
Q

Is NH3 low or high in hepatic encephalopathy?

44
Q

Asterixis is sudden loss of muscle tone (“Flapping tumor”) and is seen in this type of encephalopathy

A

Hepatic encephalopathy

45
Q

This is the characteristic physical sign of Hepatic encephalopathy

A

Asterixis (sudden loss of muscle tone)
“Flapping tumor”

46
Q

Porto-systemic shunt can be a precipitating factor to this type of encephalopathy

A

Hepatic encephalopathy

47
Q

GI bleed, dehydration, constipation and bacterial infections can be precipitating factors to this type of encephalopathy

A

Hepatic encephalopathy

48
Q

Condition characterized by fatty liver and cerebral edema associated with viral infection

A

Reyes syndrome

49
Q

Reyes syndrome involves fatty liver and cerebral edema, and is associated with this

A

Viral infection
(especially influenza and varicella)

50
Q

Does Reyes syndrome occur in pediatrics or adults?

A

Pediatrics
(almost exclusively)
(becoming very rare)

51
Q

Reyes syndrome is associated with use of this drug

52
Q

Wilson disease is a disorder of metabolism of this

53
Q

This is a disorder of copper metabolism
AKA Hepatolenticular degeneration

A

Wilson disease

54
Q

What is the inheritance pattern of Wilson disease?

A

Autosomal recessive

55
Q

What is the age of onset of Wilson disease?

A

15-30 years

56
Q

This gene is involved in Wilson disease

A

ATP7B
(encodes Copper transporting ATPase 2)

57
Q

ATP7B is a gene involved in Wilson disease, which encodes for this protein

A

Copper transporting ATPase 2

58
Q

Copper transporting ATPase 2 is involved in Wilson disease, and is encoded by this gene

59
Q

In Wilson disease, copper can deposit in CNS, primarily in this structure

A

Basal ganglia
(globus pallidus, caudate/putamen)

60
Q

In Wilson disease, does CNS dysfunction or liver cirrhosis usually occur first?

A

Cirrhosis
(not always though)

61
Q

Why can there be increased AST/ALT and PT in Wilson disease?

A

Because of hepatic copper deposition causing liver dysfunction and cirrhosis

62
Q

Does Wilson disease involve hypokinetic or hyperkinetic symptoms?

A

Hyperkinetic
(because basal ganglia is often affected)

63
Q

In Wilson disease, copper deposition in cornea can present as this

A

Keyser-Fleischer Ring

64
Q

What is Keyser-Fleischer Ring?

A

Copper deposition in cornea
Seen in Wilson disease

65
Q

Keyser-Fleischer ring is a deposition of this in the cornea

A

Copper
(seen in Wilson disease)

66
Q

What is the treatment for Wilson disease?

A

Chelation therapy

67
Q

In Wilson disease, is urine copper increased or decreased?

68
Q

In Wilson disease, is serum copper increased or decreased?

69
Q

In Wilson disease, is ceruloplasmin (copper transport protein) increased or decreased?

70
Q

High AST/ALT, high PT, low albumin, and low ceruloplasmin indicate this condition

A

Wilson disease

71
Q

Why can Alzheimer type II astrocytes be seen in Wilson disease?

A

Due to liver failure

72
Q

Can there be cerebral atrophy in Wilson disease?

73
Q

Can there be neuronal loss and gliosis in Wilson disease?

74
Q

These three areas of the brain are most vulnerable to ischemic injury, and are thus most affected by hypoglycemia and carbon monoxide

A

Pyramidal cortical neurons
Sommer’s sector (of hippocampus)
Purkinje cells of cerebellum

75
Q

The main issue of encephalopathy due to hyperglycemia is imbalance of this

76
Q

How do you treat encephalopathy caused by hyperglycemia?

A

Decrease glucose and hydrate, SLOWLY
(too quickly can cause swelling and herniation)

77
Q

Carbon monoxide can cause necrosis of this part of the brain

A

Globus pallidus

78
Q

Carbon monoxide can cause late demyelination of this

A

White matter

79
Q

Can hypothyroidism or hyperthyroidism lead to cretinism, encephalopathy, and neuropathy?

A

Hypothyroid / Myxedema

80
Q

Can hypothyroidism or hyperthyroidism lead to psychosis?

A

Hyperthyroid

81
Q

Krabbe disease results in an inability to metabolize this compound, which is a myelin component

A

Galactosylceramide

82
Q

Morphology of this condition involves brain atrophy, diffuse myelin loss and severe gliosis
Multinucleated globoid cells in white matter

A

Krabbe disease

83
Q

Spherical cytoplasmic granules in Schwann cells / Oligodendrocytes are seen in this condition

A

Metachromatic leukodystrophy

84
Q

Spherical cytoplasmic granules in Schwann cells and oligodendrocytes are seen in Metachromatic leukodystrophy, and stain red-brown with these stains

A

Cresyl violet or Toluidine blue

85
Q

Red-brown granules seen on Cresyl violet or Toluidine Blue stains are seen in this condition

A

Metachromatic leukodystrophy

86
Q

Adrenoleukodystrophy leads to very high levels of this

A

Very long chain fatty acids

87
Q

In Adrenoleukodystrophy, do CNS manifestations or adrenal insufficiency occur earliest?

A

CNS manifestations (3-10 years)
(adrenal sufficiency later)
(Progressive to death by 2-4 years after onset)

88
Q

What is the prognosis of Adrenoleukodystrophy?

A

Poor
Progressive to death by 2-4 years after onset

89
Q

Very high levels of very long chain fatty acids, demyelination and axon loss, and adrenal atrophy are seen in this condition

A

Adrenoleukodystrophy

Neuro - Week 6 (7 decks)

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