Martin: Demyelinating/Neurodegenerative Diseases/Toxic and Acquired Metabolic Diseases Flashcards
Demyelinating diseases of the CNS are acquired conditions characterized by preferential damage to ________ w/ relative preservation of ______.
Demyelinating diseases of the CNS are acquired conditions characterized by preferential damage to myelin w/ relative preservation of axons.
Which disease is characterized by distinct episodes of neuro deficits separated in time due to white matter lesion that are separated in space
Multiple Sclerosis
Which MHC halotype increases risk for developing MS?
HLA-DR2
Which T cells are the major players in causing damage to the myelin in persons w/ MS?
CD4+ TH1 and TH17
Which demyelinating disease is associated with lesions that are firmer than the surrounding white matter and contain circumscribed, depressed, glassy, grey-tan, irregularly shaped plaques?
MS
What is seen morphologically in an active plaque of a patient with MS?
- Abundant macrophagescontaininglipid-rich, PAS-positive debris
- Perivascular (small veins) inflammatory infiltrate (mononuclear) at outer edge of plaqe
- Relative preservation of axons within plaque and depletion of oligodendrocytes
A 40 yo woman who presents with chief complaint of unilateral disturbance should raise red flags for which disease until proven otherwise?
MS
What is a frequent initial manifestation of MS?
Unilateral visual disturbances due to involvement of the optic nerve (optic neuritis, retrobulbar neuritis)
The mnemonic for MS is SINS, what are each of these clinical findings?
S = scanning speech
I = intention tremor (incontinence and INO)
N = nystagmus
Which Ig is found in increased levels in the CSF of patients with MS?
What is seen on immunoelectrophoresis?
- IgG
- Oligoclonal IgG bands
Infarction of which artery is associated with contralateral homonymous hemianopia?
Posterior Cerebral Artery
Infarction of which artery is associated with UMN-type weakness and cortical-type sensory loss; contralateral hemiplegia initially?
Anterior Cerebral Artery (ACA)
Genes for which interleukin receptors have been associated with an increased risk in developing MS?
IL-2 and IL-7
What are the CSF findings in MS?
- Midly elevated protein
- Moderate pleocytosis in 1/3 cases
- IgG increased
Which condition is characterize by synchronous bilateral optic neuritis and spinal cord demyelination?
Neuromyelitis optica (aka Devic disease)
Neuromyelitis optica is due to antibodies against?
Major channel of which cell?
Aquaporin-4; major water channel of astrocytes
What is commonly found in the CSF of patients with Neuromyelitis Optica?
White cells, often including neutrophils
Although similar to MS, how does Acute Disseminated Encephalomyelitis (ADEM) differ?
When does it occur and what are the clinical manifestations?
- Occurs in younger patients w/ an abrupt onset and may be rapidly fatal
- Is a DIFFUSE monophasic demyelinating disease occuring after a viral infection or viral immunization (rare)
- Signs and symptoms develop 1-2 weeks after the antecedent infection as headache, lethargy, and coma
- In contrast to MS, all of the lesions look similar – monophasic
*MS has focal findings w/ considerable variance in the size of lesions
Acute necrotizing hemorrhagic encephalomyelitis (AKA acute hemorrhagic leukoencephalitis of Weston Hurst) is almost invariable preceded by a recent episode of?
Who is most at risk?
- Upper respiratory infection (URI)
- Young adults and children
Central pontine myelinolysis (aka osmotic demyelination disorder) most commonly arises when?
- 2-6 days after rapid correction of hyponatremia
- Low to high, the pons will die
Which neurodegenerative disorder is characterized by loss of myelin in a roughly symmetric pattern involving the basis pontis and portions of the pontine tegmentum, including myelin loss WITHOUT evidence of inflammation?
Central pontine myelinolysis (aka osmotic demyelination disorder)
What is the clinical presentation of Central pontine myelinolysis (aka osmotic demyelination disorder)?
- Rapidly evolving quadriplegia, which may be fatal
- “Locked-in” syndrome, in which patients are fully conscious yet unresponsive
Which chromosome is the gene encoding APP located on and why is this significant?
- Chromosome 21
- Lies in the down syndrome region; patients with down syndrome usually develop Alzheimers around age 40
Generation of which peptide aggregates are the critical initiating event for the development of AD?
Aβ first and then tau






