Demyelinating Diseases Flashcards
What is dysmyelination?
Flawed myelin manufacturing
What is demyelination?
Destruction of myelin
What is disruption of myelin metabolism?
Leukodystrophies
What cell types are shown within a multiple sclerosis lesion? What does this suggest?
Perivascular lymphocytes, macrophages, CD4+ helper T cells, CD8+ T cells; Suggests immune mechanism in MS
What process is occurring in the white matter at the arrows?
Loss of myelin
What is the hallmark of multiple sclerosis?
Demyelinated plaques
What is the essential clinical criterion for multiple sclerosis?
Dissemination of lesions in multiple separate areas of the CNS at different times
What are typical first time presentations of MS?
Lesions in optic nerve, brainstem or spinal cord; Blurred vision, internuclear ophthalmoplegia
What is transverse myelitis?
Acute demyelination within the spinal cord
What autoAb is produced in Neuromyelitis Optica?
Aquaporin 4
What is the basic mechanism of postinfectious and post vaccinal encephalomyelitis?
Immune responses to viral antigens (possible crossreactivity)
A patient was found to have an autosomal recessive disorder characterized by accumulation of a cerebroside and a deficiency in arylsulfatase A activity. What stains are used to identify this disorder? What is your Dx?
Cresyl violet and toluidine blue result in a metachromatic stain; Metachromatic Leukodystrophy
A 2 year old patient has a deficiency of galactocerebroside Beta-galactosidase and multinucleated globoid cells. The patient dies. What is your Dx? What is another name for your Dx? What are the globoid cells?
Krabbe Disease; Globoid Cell Leukodystrophy; Multinucleated macrophages containing undigested galactocerebroside
A 5 yo patient was found to have very long chain fatty acids in his serum and neurologic defects. What is the diagnosis? What other serious problems would this patient eventually develop? How is this disease transmitted?
Adrenoleukodystrophy; Adrenal atrophy and dysfunction; X-linked inherited disorder
