Neuro - Week 6 > Metabolic Neurologic Disorders > Flashcards

Metabolic Neurologic Disorders Flashcards

(89 cards)

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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
Why does Wernicke encephalopathy cause memory loss?
Hemorrhage in thalamus and mamillary bodies
26
In Wernicke encephalopathy, hemorrhage in these two structures can cause memory loss
Thalamus and mamillary bodies
27
Is Wernicke encephalopathy slow or rapid onset?
Rapid
28
This is the classic triad of Wernicke encephalopathy
Confusion with memory loss (delirium) Ophthalmoplegia Gait ataxia
29
This is the chronic form of Wernicke-Korsakoff Syndrome
Korsakoff syndrome
30
Is Korsakoff syndrome reversible?
No
31
Korsakoff syndrome is due to chronic, repetitive injury to this structure
Mamillary bodies (and possibly cerebellum/PNS)
32
Encephalopathy seen in end-stage liver disease
Hepatic encephalopathy
33
Hepatic encephalopathy occurs with liver disease, but also may be induced with this procedure
Porto-systemic shunting
34
Pathophysiology of Hepatic encephalopathy is unclear, but is related to this condition
Hyperammonemia
35
This type of encephalopathy is related to Hyperammonemia
Hepatic encephalopathy
36
What organ metabolizes ammonia?
Liver
37
Encephalopathy involving this organ will show Alzheimer type II astrocytes
Liver
38
This cell type is the pathologic hallmark of Hepatic encephalopathy
Alzheimer type II astrocyte
39
High levels of this compound results from hemoglobin degradation and is seen in liver failure
Bilirubin
40
Is bilirubin low or high in hepatic encephalopathy?
High
41
Is albumin low or high in hepatic encephalopathy?
Low
42
Is prothrombin time (PR) low or high in hepatic encephalopathy?
High
43
Is NH3 low or high in hepatic encephalopathy?
High
44
Asterixis is sudden loss of muscle tone ("Flapping tumor") and is seen in this type of encephalopathy
Hepatic encephalopathy
45
This is the characteristic physical sign of Hepatic encephalopathy
Asterixis (sudden loss of muscle tone) "Flapping tumor"
46
Porto-systemic shunt can be a precipitating factor to this type of encephalopathy
Hepatic encephalopathy
47
GI bleed, dehydration, constipation and bacterial infections can be precipitating factors to this type of encephalopathy
Hepatic encephalopathy
48
Condition characterized by fatty liver and cerebral edema associated with viral infection
Reyes syndrome
49
Reyes syndrome involves fatty liver and cerebral edema, and is associated with this
Viral infection (especially influenza and varicella)
50
Does Reyes syndrome occur in pediatrics or adults?
Pediatrics (almost exclusively) (becoming very rare)
51
Reyes syndrome is associated with use of this drug
Aspirin
52
Wilson disease is a disorder of metabolism of this
Copper
53
This is a disorder of copper metabolism AKA Hepatolenticular degeneration
Wilson disease
54
What is the inheritance pattern of Wilson disease?
Autosomal recessive
55
What is the age of onset of Wilson disease?
15-30 years
56
This gene is involved in Wilson disease
ATP7B (encodes Copper transporting ATPase 2)
57
ATP7B is a gene involved in Wilson disease, which encodes for this protein
Copper transporting ATPase 2
58
Copper transporting ATPase 2 is involved in Wilson disease, and is encoded by this gene
ATP7B
59
In Wilson disease, copper can deposit in CNS, primarily in this structure
Basal ganglia (globus pallidus, caudate/putamen)
60
In Wilson disease, does CNS dysfunction or liver cirrhosis usually occur first?
Cirrhosis (not always though)
61
Why can there be increased AST/ALT and PT in Wilson disease?
Because of hepatic copper deposition causing liver dysfunction and cirrhosis
62
Does Wilson disease involve hypokinetic or hyperkinetic symptoms?
Hyperkinetic (because basal ganglia is often affected)
63
In Wilson disease, copper deposition in cornea can present as this
Keyser-Fleischer Ring
64
What is Keyser-Fleischer Ring?
Copper deposition in cornea Seen in Wilson disease
65
Keyser-Fleischer ring is a deposition of this in the cornea
Copper (seen in Wilson disease)
66
What is the treatment for Wilson disease?
Chelation therapy
67
In Wilson disease, is urine copper increased or decreased?
Increased
68
In Wilson disease, is serum copper increased or decreased?
Decreased
69
In Wilson disease, is ceruloplasmin (copper transport protein) increased or decreased?
Decreased
70
High AST/ALT, high PT, low albumin, and low ceruloplasmin indicate this condition
Wilson disease
71
Why can Alzheimer type II astrocytes be seen in Wilson disease?
Due to liver failure
72
Can there be cerebral atrophy in Wilson disease?
Yes
73
Can there be neuronal loss and gliosis in Wilson disease?
Yes
74
These three areas of the brain are most vulnerable to ischemic injury, and are thus most affected by hypoglycemia and carbon monoxide
Pyramidal cortical neurons Sommer's sector (of hippocampus) Purkinje cells of cerebellum
75
The main issue of encephalopathy due to hyperglycemia is imbalance of this
Water
76
How do you treat encephalopathy caused by hyperglycemia?
Decrease glucose and hydrate, SLOWLY (too quickly can cause swelling and herniation)
77
Carbon monoxide can cause necrosis of this part of the brain
Globus pallidus
78
Carbon monoxide can cause late demyelination of this
White matter
79
Can hypothyroidism or hyperthyroidism lead to cretinism, encephalopathy, and neuropathy?
Hypothyroid / Myxedema
80
Can hypothyroidism or hyperthyroidism lead to psychosis?
Hyperthyroid
81
Krabbe disease results in an inability to metabolize this compound, which is a myelin component
Galactosylceramide
82
Morphology of this condition involves brain atrophy, diffuse myelin loss and severe gliosis Multinucleated globoid cells in white matter
Krabbe disease
83
Spherical cytoplasmic granules in Schwann cells / Oligodendrocytes are seen in this condition
Metachromatic leukodystrophy
84
Spherical cytoplasmic granules in Schwann cells and oligodendrocytes are seen in Metachromatic leukodystrophy, and stain red-brown with these stains
Cresyl violet or Toluidine blue
85
Red-brown granules seen on Cresyl violet or Toluidine Blue stains are seen in this condition
Metachromatic leukodystrophy
86
Adrenoleukodystrophy leads to very high levels of this
Very long chain fatty acids
87
In Adrenoleukodystrophy, do CNS manifestations or adrenal insufficiency occur earliest?
CNS manifestations (3-10 years) (adrenal sufficiency later) (Progressive to death by 2-4 years after onset)
88
What is the prognosis of Adrenoleukodystrophy?
Poor Progressive to death by 2-4 years after onset
89
Very high levels of very long chain fatty acids, demyelination and axon loss, and adrenal atrophy are seen in this condition
Adrenoleukodystrophy

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