Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Neurology > Toxicities to the CNS > Flashcards

Toxicities to the CNS Flashcards

1
Q

How to recognize a metabolic disorder?

A
  • -presentation is abnormal and does not respond to conventional therapy
    • chronic metabolic disorders are typically only obvious during physical stress
    • the longer treatment is delayed the more irreversible damage is done
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

A build up of Gangiosidoses in neurons is caused by what?

A

Hexoaminidase A Defect – Tay-Sachs Disease

Hexosaminidase B Defect – Sandhoff Disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are characteristic findings of Tay-Sachs Disease?

A
  • Normal at birth, progresses to psychomotor retardation by 6 months of age
  • Blindness
  • Cherry Red Spot in Macula
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What are the differences between Tay-Sachs and Sandhoff Disease?

A

Tay-Sachs most affects CNS

Sandhoff mostly affects organs and liver, progresses faster than Tay-Sachs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is common histology found with Tay-Sachs Disease?

A
  • Enlarged Neuron filled with abnormal accumulation of gangiosides
  • menbranous cytoplasmic bodies
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

If a patient is found to have a Galactocerebroside-B-galactosidase deficiency, what does it cause and why?

A

Globoid Cell Leukodystrophy (Krabbe’s Disease)

- accumulation of psychosine, damaging Oligodendrocytes causing rapid motor function deterioration with tonic spasms.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

If a patient is screen to be positive for Krabbe’s Disease, what is the next step?

A

Umbilical Cord/Bone Marrow Transplant

– earlier the better, the damage is irreversible

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is a histologic finding of Krabbe’s Disease?

A
  • Atrophic White Matter
  • Globoid Cells in the White Matter
  • Globoid Macrophages with straight crystalloids
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

If there is a build up of sulfatides and myelin degeneration in an infant with metachromasia staining what might be the cause?

A

Metachromatic Leukodystrophy

– Aryl Sulfatase A deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

A 7 year old male presents with progressive dementia, visual/hearing loss, seizures, with grey discoloration of white matter with PAS-positive macrophages?

A

Adrenoleukodystrophy

– Decreased activity of very long chain fatty acyl-Coa synthetase (peroxisomes)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is a common effect of chronic liver disease in alcoholics?

A

–Hepatic Encephalopathy

Build up of ammonemia in the CNS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are the common symptoms of Hepatic Encephalopathy?

A
  • -inattentiveness and short term memory impairment
  • -**Asterixis
    • confusion
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

If on histology a Alzheimer Type 2 Astrocyte is revealed, what might be the etiology?

A

Hepatic Encephalopathy

    • “Astrocyte with empty or cleared out nucleus”
    • Not related to Alzheimer’s Disease
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is the best way to diagnose a Mitochondrial Disease?

A

Broad spectrum of testing to see the general consensus if most are abnormal to confirm diagnosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the most common cause of MELAS?

A

Heteroplasmic point mutation of mt-tRNA-leu

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are the clinical manifestations of a mitocondrial mutation causing deficiency converting pyruvate to ATP?

A

Leigh’s Disease

  • Patchy necrosis of the periventricular grey matter not consistant with normal vessel distribution
  • arrest of development, lactic acidemia, loss of skills
  • spongiform proliferation
17
Q

What conditions are concerning for chronic alcoholics and malnourished?

A
  • Wernicke Encephalopathy

- Korsakoff Syndrome

18
Q

What is a consistant finding with Wernicke Encephalopathy?

A
  • Ophthalmoplegia and Nystagmus
  • Ataxia / Confusion
  • Destruction of the Mammilary Bodies
19
Q

If a chronic alcoholic is being evaluated and is having difficulty with recent past events, but still seems to speak knowledgably and able to still perform learned tasks normally. Even though the patient is unable to remember they are able to put together a logical reasoning for the gap in time? What might be causing this?

A

Korsakoff Psychosis

–from repeated episodes of Wernicke’s Encephalopathy

20
Q

What are the effects of chronic B12 deficiency?

A
  • loss of ability to myelinate, affecting PNS/CNS
  • ataxia, spasticity
  • demyelination of lateral corticospinal tracts and posterior columns
    From Pernicious Anemia – lost Intrinsic Factor to absorb B12
21
Q

If you find necrosis of the globus pallidus and substantia nigra, what is the most likely cause?

A

Carbon Monoxide Poisoning

– These areas have the most iron present, so it binds there.

22
Q

What can chronic ethanol toxicity cause long term?

A

Cerebellar Degeneration

- truncal ataxia and nystagmus

23
Q

What are the common signs of Fetal Alcohol Syndrome?

A
  • Facial Deformities
  • epicanthal folds (at the corners of the eyes)
  • thin upper lip
  • microcephaly
  • cerebellar dysgenesis
  • upturned nose
  • flat nasal bridge
    (even if no facial features can have cerebellar defects)
24
Q

What is an effect of Phenytoin toxicity?

A
  • atrophy of the cerebellar vermis and loss of Purkinje cells

Neurology (11 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions