Term
| What is the clinically isolated syndrome and Benign MS |
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Definition
CIS: Single MS episode(commonly Isolated Optic Neuritis)
Benign MS: Returns to baseline function after an MS relapse |
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Term
| Describe the clinical presentation of MS |
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Definition
| Progressive lesions separated in time an space with periodic relapses and remissions |
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Term
| Diagnositic studies for MS |
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Definition
CSF Ig analysis: Oligoclonal banding
Electrophysiology: Delayed
MRI: Peri-ventricular Plaques
Hot Bath |
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Term
| Describe the histology of an MS plaque |
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Definition
Perivascular Lymphocytes
Myelin and Oligodendrocyte loss
Reactive astrocytes long-term
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Term
| What are the possible causes of MS |
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Definition
Virus(Canine Distemper Virus)
Climate before age 15
HLA antigens |
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Term
| Who gets Perivenous Encephalomyelitis, how does it present, and describe the pathologic categories |
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Definition
Who: Children
Pres: Headache, Lethargy, Coma, and Death
1.)Acute disseminated Encephalomyelitis with acute demyelination and perivenular lymphocytes
2.)Acute hemorrhage leukocephalitis (Acute disseminated Encelphalomyelitis with hemorrhage) |
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Term
| What is Central Pontine Myelinolysis and what is the prognosis |
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Definition
Demyelination of pontine base from rapid rise in saline
Poor prognosis |
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Term
| What is Metachromatic leukodystrophy and how is it diagnosed |
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Definition
Aryl Sulfatase-A mutation causing accumulation of galactosyl sulfatides
Dx: urine or blood Galactosyl levels |
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Term
| What is Krabbe's Disease and what is the pathology |
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Definition
| Galactocerebroside-galactosidase mutation causing galactocerebroside accumulation forming Globoid Cells(Mac with abnormal lipid) and demyelination |
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Term
| What is adrenalleukodystrophy |
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Definition
Peroxisomal defect in VLCFA transport causing accumulation
CNS demyelination and Addison's from adrenal insufficiency |
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Term
| How does Wernicke's Encephalopathy present and what is the most common cause in the hospital |
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Definition
Presentation: Delerium, Eye movement problems, ataxia
Glucose to boarderline deficient patients |
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Term
| What is Korsakoff's Psychosis |
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Definition
| Chronic Wernicke's with short-term memory loss and confabulation to hide it |
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Term
| What is the pathology of disorders Thiamine deficiency |
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Definition
Wernicke's: Petechial hemorrhages in Mamillary bodies, 3rd and 4th ventricular walls
Korsaoff's: Gliosis and vascular hyperplasia in Mamillary bodies, 3rd and 4th ventricular walls |
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Term
| How does B12 deficiency present and what is the pathology |
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Definition
Presentation: Subacute spastic paraparesis, ataxia, and paraesthesias of legs-> paralysis
Pathology: Degeneration of white matter in posterior/lateral areas of the spinal cord |
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Term
| What are the neurotoxic effects of Alcohol, Vinca Alkaloids, Methotrexate plus Irradiation, and Radiation |
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Definition
Alcohol: Peripheral neuropathy, Cerebellar vermis degeneration
Vinca Alkaloid: Peripheral neuropathy
Methotrexate+Irradiation: Periventricular white matter lesions causing leukoencephalopathy
Radiation: Direct damage especially when spinal cord is injured |
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Term
| What are the clinical signs of Hepatic failure neuropathy and what cell is common? |
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Definition
High Ammonia-> Asterixis
Cell: Alzheimer's Type II astrocytes
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Term
| What are signs and pathology of Wilson's disease? |
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Definition
Presentation: Kayser-Fleisher Rings(Copper in Cornea)
Hepatic failure
Psycosis, Dementia
Gross: Brown discoloration/atrophy of caudate, putamen, globus pallidus
Occasional putamen cavitations
Micro: Alzeimer's type II Astrocytes, Opalski Cells(large eccentric nuclei in basal ganglia) |
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Term
| What is leukodystrophy and what is the major clinical sign? |
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Definition
Error of metabolism of myelin causing abnormal formation and degradation
Signs: Spasticity and Weakness
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Term
| Cause and pathology of Guillain-Barre Syndrome? What is typically abnormal in the CSF fluid |
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Definition
Viral infection(most common)
Demyelinating autoimmune disease
Dramitically high protein in the CSF |
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