Neuro Demyelinating Disorders

Front 1

Key points of MS

Back 1

- autoimmune demyelinating disorder
- white matter lesions separated in TIME and SPACE

Front 2

Immune causality of MS

Back 2

-CD4 Th1 cells that react against self myelin antigens and secrete cytokines (i-gamma) that activate macrophages = eat the myelin

Front 3

Localizations of MS plaques

Back 3

near lateral ventricles, optic nerves/chiasm, brainstem tracts, cerebellum, spinal cord

Front 4

Active plaques

Back 4

Plenty of debris, macrophages, inflammatory infiltrate, perivascular cuff

Front 5

Inactive plaques

Back 5

No myelin, almost no oligodendrocytes, prominent gliosis

Front 6

Shadow plaques

Back 6

No sharp demarcation, either no progression or some recovery and remyelination of thin sheaths.

Front 7

Optic (retrobulbar) neuritis

Back 7

Sequelae of MS, unilateral vision loss early in disease

Front 8

CSF finding in MS (common, not diagnostic)

Back 8

Oligoclonal bands (and therefore elevated gamma globulin)

Front 9

Neuromyelitis Optica (Devic)

Back 9

MS variant involving bilateral optic tract and extreme spinal cord degen (even grey matter)

Front 10

Marburg MS

Back 10

Attacks the young, fulminant, swiftly fatal with prominent plaques

Front 11

ADEM

Back 11

(Acute Disseminating Encephalomyelitis) Monophasic, follows a viral infxn or immunization. Kills approximately 20% with widespread brain involvement. The rest recover fully

Front 12

ANHE

Back 12

(Acute Necrotizing Hemorrhagic Encephalomyelitis) Preceded by URI from mycoplasma pneumoniae. Considerably fatal.

Front 13

Loss of myelin in basis pontis

Back 13

Central pontine myelinolysis. Caused by rapid correction of hyponatremia. Rapid quadriplegia in setting of alcoholism, electrolyte imbalance, orthotopic liver transplantation

Front 14

What is a leukodystrophy, by definition?

Back 14

A genetically caused myelin disorder: abnormal synthesis or turnover

Front 15

Krabbe Disease

Back 15

Deficiency in galactocerebroside catabolism. Not toxic, the alternate pathway is deadly. Hits children after 3rd month, motor deficits followed by oligodendrocyte degeneration. Dead in 2 yrs.

Front 16

Morphology of Krabbe disease?

Back 16

Globoid cells around blood vessels.

Front 17

Metachromatic Leukodystrophy

Back 17

Accumulate sulfatides: infantile onset with death in 5-10 years. Adult disease is slower. Demyelination with gliosis, one color in most dye preps (hence, metachrome)

Front 18

Myelin loss and adrenal atrophy

Back 18

Adrenoleukodystrophy. In children it is rapidly fatal, in adults it is peripheral and takes decades. X-linked. VLCFA accumulation.

Front 19

Hepatic encephalopathy

Back 19

Liver disease leads to liver failure, astrocytes take on Alzheimer's 2 characteristics in basal ganglia and cortex

Front 20

Encephalopathies caused by alcoholism

Back 20

Cerebellar atrophy, cerebral atrophy, Wernick's encephalopathy, fetal alcohol syndrome

Front 21

Thiamine deficiency (due to alcoholism, starvation, IV feeding, etc) causes . . .?

Back 21

Wernicke's encephalopathy: degradation of hypothalamus/mamillary bodies: active psychosis

Front 22

Wernicke-Korsakoff syndrome

Back 22

Long-term sequelae of Wernicke's encephalopathy: opthamaloplegia, nystagmus, ataxia, confusion, coma, psychotic behavior, antegrade episodic memory loss, confabulation, amnesia

Front 23

Carbon monoxide poisoning breaks down what part of the brain?

Back 23

Globus Pallidus