Metabolic Disorders in CNS
Terms
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- Nystagmus, ataxia, and mental confusion seen in alcoholics.
- Wernicke encephalopathy
- Retrograde and anterograde amnesia with confabulation seen in alcoholics.
- Korsakoff psychosis
- Wernicke-Korsakoff is due to a deficiency in ___.
- thiamine
- Discoloration is seen in ____ in Wernicke-Korsakoff.
- mammillary bodies, hypothalmus, and periaqueductal gray matter
- Mild form of fetal alcohol syndrome
- fetal alcohol effect
- FAS is associated w/ ?
- growth retardation, facial abnormalities, and intellectual impairment, behavioral problems, mircrocephaly
- Alcohol effects which development epoch?
- Neurogenesis for microcephaly, migration for the other lesions
- Important CNS feature of alcoholism
- cerebellar degeneration
- Patient comes in w/ hyponatremia and you normalize patient's sodium too fast.
- Central pontine myelinolysis
- Demyelination of corpus callosum seen in Italian men who drank cheap red wine.
- Marchiafava-Bignami Disease
- Subacute combined degeneration is seen in deficiencies of _____
- B12 (most common) and folate
- Folate deficiecy in pregnancy can lead to ____
- neural tube defects
- Organellar reactions to heavy metals:
-
1. mitochondria initiate apoptosis
2. protein syntheis lost in ER
3. nuclear proteins are altered - Heavy metals are more pathogenic in adults/children?
- children b/c they can concentrate the heavy metals more
- Pathogenesis of mercury
- alters enzymatic reactions
- Fetal mercury exposure causes
- microcephaly and abnormality in neuronal migration
- Presenting symptom of methanol poisoning
- blindness
- Methanol is metabolized to
- formic acid
- Formic acid causes lesions in the ?
- retinal cells, cerebral cortex, and putamen
- Carbon monoxide causes lesions in
- globus pallidus
- Seen in people w/ cirrhosis and liver failure and have hyperammonemia
- Hepatic Encephalpathy
- Histolic finding in hepatic encephalopathy
- Alzheimer type 2 astrocytes
- Wilson's disease is a disease of ____ metabolism
- coppper
- Copper deposits in eye
- Kaiser Fleischer ring
- Comatose, fatty liver, strong association w/ aspirin
- Reye's syndrome
- "Mad as a hatter"
- Mercury poisoning
- Degeneration of posterior columns extending from thoracic cord to nucleus gracilis is seen in this deficiency.
- Vitamin E
- Most enzyme deficiencies are inherited in a ____ fashion
- Autosommal Recessive
- Lysosomal storage disease that occurs in the neuronal cell body.
- Neuronal lipidosis
- Common histological finding in mucopolysaccharidoses
- zebra bodies
- Histological finding in Tay Sachs
- membranous cytoplasmic bodies
- Problem w/ storage of sphingolipids
- Gangliosidoses
- Storage diseases are primarily ___ matter diseases and present with ___ and ____.
- gray, seizures and loss of cognitive ability
- Tay Sachs is a deficiency in hexosaminnidase A and results in accumulation of ____.
- GM2 gangliosides
- Symptoms of Tay Sachs
- dementia, blindness (retinal degeneration)
- A arylsulfatase A deficiency causes an accumulation of myelin sulfatide in both oligodendroglia and Schwann cells.
- Metachromic Leukodystrophy