Demyelinating disorders Flashcards
Characteristics of MS
Chronic inflammation
Demyelination
Gliosis
Neuronal Loss
Characteristic finding on CSF analysis in MS
Oligoclonal bands
Gene associated with predisposition for MS
HLA-DRB1 gene
General immunologic pathogenesis of MS
Autoreactive T lymphocytes (type IV hypersensitivity)
B cell activation and antibody response
Helper T cell types associated with MS
Th1
Th17
CSF abnormalities in MS
Mononuclear cell pleocytosis
Oligoclonal bands (increased IgG)
Histology shows perivenular cuffing by inflammatory mononuclear cells predominantly with T cells and macrophages
Active MS plaque
Histology shows loss of oligodendrocytes with astrocyte proliferation and gliosis
Inactive MS plaque
How areas of demyelination in MS appear on MRI
High-signal on T2 and FLAIR
Treatment for acute attack of MS
Corticosteroids
Humanized monoclonal antibody against alpha-4 integrin used in treatment of remitting relapsing MS
Natalizumab
Reduces the transmission of immune cells into the CNS by interfering with the alpha-4-beta-1 integrin receptor molecules
Natalizumab
Risk associated with Natalizumab
Multifocal leukoencephalopathy
Acts by suppressing the activity of T cells, B cells, and macrophages on the myelin sheath
Mitoxantrone
General mechanism of cladribine
Depletes B cells
Mimics the structure of myelin basic protein to act as a decoy for T cells
Glatiramer
Shifts population of T cells from proinflammatory Th1 T cells to regulatory Th2 T cells to suppress inflammatory response
Glatiramer
Increases cytotoxicity of NK cells and increases phagocytic activity of macrophages to treat MS
Beta interferons (beta-1a)
Selective sphingosine-1-phosphate receptor modulators
Fingolimod
Siponimod
Drugs that sequester lymphocytes in lymph nodes to prevent them from contributing to an autoimmune reaction, used to treat MS
Fingolimod
Siponimod
Multifocal areas of demyelination of varying size in the brain, sparing the spinal cord and optic nerves
Progressive multifocal leukoencephalopathy
Cause of progressive multifocal leukoencephalopathy
Reactivation of latent JC virus
Demyelination without inflammation in the base of the pons with relative sparing of axons and nerve cells
Osmotic demyelination syndrome/central pontine myelinolysis
Tracts affected in osmotic demyelination syndrome
Bilateral corticospinal and corticobulbar tracts
Caused by rapid correction of hyponatremia
Osmotic demyelination syndrome
Characterized by massive astrocyte death
Osmotic demyelination syndrome
Clinical features of osmotic demyelination syndrome
Quadriparesis
Pseudobulbar palsy –> dysarthria, dysphagia, and dysphonia
Locked-in syndrome
First manifestation of MS
Impaired vision associated with optic neuritis and internuclear ophthalmoplegia
Name for MRI finding in MS
Dawson’s fingers
