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Lecture 18

Diseases of the Myelin

Definition of demyelinating diseases A disruption of normal myelin causing neurologic disease
Definition of dysmyelinating diseases A disruption of abnormal myelin causing neurologic disease
A neurologic disease characterized by a history of remissions and exacerbations of at least 2 separate neurologic abnormalities with multiple plaques found on MRI. Multiple sclerosis
What are the clinical forms of MS? (1) Relapsing-remitting MS (2) Primary progressive MS (3) Secondary-progressive MS
Pathologic characteristics of multiple sclerosis plaques (1) Demyelination with relative preservation of axons (2) Infiltration of lymphocytes and macrophages (3) Gliosis (4) Variable remyelination
A clinical type of MS characterized by acute attacks with full recovery or minor residual deficit upon recovery. Relapsing-remitting MS
A clinical type of MS characterized by progression of disability from the onset of the disease without remissions. This form more commonly affects males and has a later onset. Primary progressive MS
A clinical form of MS characterized by an initial course of relapsing-remitting MS, followed by progression of disability. Secondary-progressive MS
Common clinical symptoms of relapsing-remitting form of MS (1)Optic neuritis (2)sensory(less motor) symptoms (3)brain stem involvement (4)cerebellar involvement
What are the type of MS plaques? (1)Active plaque (2)Inactive plaque (3)Shadow plaque
Histologic characteristics of active MS plaques (1)Active myelin destruction with relative axonal preservation (2)Inflammatory reaction (T cells>>B cells) (3) Mild astrocytosis (4) Periphery of the plaque is hypercelluar with lymphocytes and macrophages
Histologic characteristics of chronic MS plaques (1)Demyelination with "naked" axons (2)Minimal inflammatory reaction (3)Dense gliosis (4)Periphery of the plaque is hypocellular
Histologic characteristics of shadow MS plaques (1)Myelin content is reduced but not totally absent (2)Remyelinated axons present with relatively thin myelin sheaths
Distribution of MS plaques (1)Periventricular areas (2)Cortical gray/white matter transition zone (3)Optic nerves, chiasm, tracts (4)Periaqueductal area (5)Cerebellum white matter (6)Cerebellar peduncles (7)Dorsal spinal cord (8)Subpial region of spinal cord (10)Pericentral canal
A very aggressive form of MS where the disease progresses rapidly to disability or death. Marburg type
A rare variant of MS characterized by lesions consisting of rings of demyelination alternating with rings of intact myelin. Balo type (Concentric sclerosis)
Treatment for an acute attack of MS High dose steroids
Treatment for relapsing forms of MS Interferon Beta (1a and 1b) or Natalizumab.
An acute demyelinating disorder characterized by widely scattered small foci of periventricular inflammation and demyelination most frequently associated with antecedent immunization or infection. Acute Disseminated Encephalomyelitis (ADEM)
Most common preceding infections of postinfectious encephalomyelitis Measles, mumps, chickenpox (varicella), rubella, influenza, mononucleosis (EBV)
Most common preceding vaccinations of postvaccinal encephalomyelitis Smallpox, rabies, influenza vaccination
Biochemical abnormality associated wtih X-linked Adrenoleukodystrophy (ALD) Leukodystrophy caused by abnormality in peroxisomal beta-oxidation resulting in an excess of very long chain fatty acid (VLCFA) esters in the plasma, cultured fibroblasts and affected tissues.
A rare inherited progressive neurologic disease with adrenal insufficiency. Childhood form of X-linked adrenoleukodystrophy
The distribution of lesions of X-linked adrenaleukodystrophy Lesions are bilateral and symmetrical and mainly limited to the white matter. The typical locations include occipital, parietal, and temporal lobes. Brain stem and the spinal cord are less involved.
A neurologic condition with a hallmark finding of needle-like trilaminar bodies in CNS macrophages, adrenal cells, Schwann cells, testes, and cultured fibroblasts. X-linked adrenaleukodystrophy
Pathogenesis of X-linked adrenoleukodystrophy Dysmyelination results from the myelin membrane insability induced by very long chain fatty chain acid (VLCFA) esters while the demyelination is initiated by cyotkines from an inflammatory response.
Criteria for prenatal diagnosis of X-linked adrenoleukodystrophy (1) very long chain fatty acid esters levels in chorionic villus samples (CVS) (2) needle-like trilaminar bodies in cultured fibroblasts of culture of amniotic fluid cells.
Treatment option for neurologically intact or verly mildly affected children in X-linked adrenoleukodystrophy Bone marrow transplant
Treatment options for X-linked adrenoleukodystrophy (1) X-linked adrenoleukodystrophy (2) Lorenzo's oil (3) glucocorticoid replacement for adrenocortical insufficiency
Created by: UVAPATH2