PATH: Disorders of Myelin Flashcards
Causes of Primary Myelin Loss?
What determines the outcome of demyelnation?
Demyelinating Disorders?
- MS
- Transverse Myelitis (Inflammation of spinal cord (lower back)
- Neuromueleitis Optica
- Progressive Multifocal Leukoencephalopathy (PML): VIRAL or Iatrogenic
- Central Pontine Myelonolysis (IV Saline given in too high concentration => osmotic balance of brainstem disrupted => demylation)
- Toxic Demylenation (Hexachlorophane, Cyanide, CO, Solvent Vapor Abuse)
- PNS Disorders (Guillain- Barre Syndrome, CIDP, Chacot Marie Tooth Disease)
Acquired, autoimmune demyelinating disorder of CNS (brain/cord) characterized by distinct episodes of neurologic deficit separated by time?
- Characteristics?
- Appearance (Acute/Chronic)?
Epidemiology?
MS Pathogenesis?
MS Prognosis? How can this be observed bedside?
Presentation/Diagnosis of MS?
Progression of MS?
Treatment of MS?
□ _________________ prolongs action potentials (Doesn’t affect underlying immune problem)
□ Relapse treatment: _____________ (feel better quickly: turn off inflammation/cytokines)
□ Remission maintenance:
- ___________________: Suppresses lymphocyte activity
- ___________________: Competitor antigen soaks up lymphocyte activity
- ___________________: Suppresses trafficking and TH17 activity
- _______________ (vessels), and _______________ (nodes): Frustrate lymphocyte trafficking
- __________________: Suppresses lymphocyte proliferation (Topoisomerase Inhibition)
Treatment of MS?
□ K-channel blocker (Fampyra) prolongs action potentials (Doesn’t affect underlying immune problem)
□ Relapse treatment: Steroids (feel better quickly: turn off inflammation/cytokines)
□ Remission maintenance:
- Immunoglobulin: Suppresses lymphocyte activity
- Glatiramer acetate: Competitor antigen soaks up lymphocyte activity
- Interferon Beta 1: Suppresses trafficking and TH17 activity
- Natalizumab (vessels), and Fingolimod (nodes): Frustrate lymphocyte trafficking
- Mitoxantrone: Suppresses lymphocyte proliferation (Topoisomerase Inhibition)
Role of Clinically Isolated Syndrome in the Diagnosis of MS?
MS Diagnosis must consist of 2 or more attacks affecting the CNS disseminated in time and space
Acute, Monophasic, demylenating inflation of the spinal cord resulting in severe neck/back pain and subacute weakness below a certain level?
Antibody-mediated demyelinating disease common in Asia?
Acute brain/spinal cord inflammation 5-15 days post-viral illness resulting in ataxia, headache, and ataxia?
Immune/compliment activation mediated Ephaphilitis mostly occurring in children following URTI?
Demyelinating disease in which the JC Polyomavirus targets oligodendrocyte [viral inclusions]
=> cell lysis => rapid demyelination?
Concerns with MS Treatment?
Demylenating condition which results from vigorous correction of low serum sodium levels? leading to rapid onset tetraplegia and coma?
Abnormalities in genes related to the generation, turnover and maintenance of Myelin?
Example Diseases?
Leukodystrophies
Autosomal recessive disease resulting from deficiencies in galactocerbroside B galactosidase manifesting in early infancy (3-6 Months) with rapidly progressive severe motor/sensory and cognitive deficits?
- Treatment?
X-Linked disease in which abnormal ATP-BindingCassete-D1 (ABCD1) => Abnormal peroxisome transport => VLC Fatty Acids can’t be transported and build up => damage to myelin and adrenal glands?
- Treatment?
Autosomal recessive diseases resulting from deficient lysosomal arylsulfatase A => Buildup of toxic sulfatides
- Treatment?
