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Brain and Behavior > 6.2.3 Myelin Disorders > Flashcards

6.2.3 Myelin Disorders Flashcards

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1
Q
A
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2
Q

What are the main presentation features of central pontine myelitis?

A

Rapid quadriplegia, basis pontis lesion

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3
Q

What are the two main subtypes of myelin disorders?

A

Demyelinating disease and leukodystrophies

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4
Q

What are some of the unique features of acute hemorrhagic encephalomyelitis (Weston Hurst)?

A
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5
Q

Acquired chronic inflammatory disorder of CNS with distinct episodes of neurological deficits, separated in time, attributable to white matter lesions that are separated in space

A

Multiple sclerosis

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6
Q

What are the diagnostic criteria (main features) of NMO?

A

Optic Neuritis + Myelitis + NMO-IgG seropositivity

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7
Q

Name this condition

A

Acute hemorrhagic encephalomyelitis

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8
Q

What are some of the unique features of acute disseminated encephalomyelitis (ADEM)?

A
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9
Q

What gene is commonly associated with MS in most populations?

A

HLA-DR15 (a split of DR2)

Does not follow Mendelian genetics

Cytokine-receptor genes have been linked to MS susceptibility (IL2RA, IL7RA)

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10
Q

Which cranial nerve is commonly affected by MS and why?

A

Optic nerve (and chiasm) because the nerve is covered in meninges and still has central myelination

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11
Q

Name this condition and its characteristic cell that is being encircled

A

Neuromyelitis optica; Creutzfeldt cell

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12
Q

What are the key features of Krabbe’s?

A

Lysosomal Disease

Globoid cells

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13
Q

What virus is associated progressive multifocal leukoencephalopahty (PML)? What causes someone infected with this virus to progress to PML?

A

JC Virus; Becoming immunocompromised

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14
Q

What are leukodystrophies?

A

Progressive inherited diseases with abnormal synthesis or turnover of myelin; most are AR in inheritance

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15
Q

What are some of the unique characteristics of Acute MS?

A

Childhood to young adults, rapid progression, relatively unresponsive to steriods

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16
Q

What is the pathogenesis of MS?

A
17
Q

What is being shown in this image?

A

Inactive MS plaque (no blue due to destruction of myelin, few macrophages surrounding the blood vessels)

18
Q

Where is demyelination confined to in MS?

A

CNS

19
Q

What is the autoimmune target of neuromyelitis optica (NMO) that distinguishes it from MS?

A

NMO-Ab towards aquaporin-4

20
Q

What is the typical symptomalogy of PML? What is the typical course and end result?

A

Progressive visual, motor, dementia, and sensory symptoms

Short course (6-12 months) leading to death

21
Q

Who typically presents with adrenoleukodystrophy? Why?

A

Young boys; x-linked

22
Q

Name this condition

A

Adrenoleukodystrophy; black lines pointing at gemistocytic astrocytes

Parieto-occipital white matter preferentially affected

Perivascular CD8 T-lymphocytes

Macrophages, adrenal cortical cells, Schwann, Leydig cells: trilaminar lipid inclusions

23
Q

What is the inheritance of alexander’s dz?

A

AD due to de novo/mutation in GFAP gene

24
Q

What is shown in the center of this histological image?

A

Active MS plaque

Neurofilament: indicates the preservation of axons

Macrophages: presence indicates active destruction (active)

25
Q

What is the difference between an active and inactive plaque of MS?

A

Active plaque: Pink and swollen with presence of macrophages

Inactive plaque: inflammation and macrophages have cleared out, lack of myelin

26
Q

Name this condition

A

Alexander’s disease (more rosenthal fibers than any other disease)

27
Q

What are the key features of Metachromtic LD?

A

Metachromatic material

28
Q

Fill in the classification and key pathologic features of these leukodystrophies

A
29
Q

How will Alexander’s disease present in young children?

A

Seizures, spasticity, megalencephaly, developmental delay

30
Q

What are the key features of Adreno-LD?

A

Perivascular inflammation

Peroxisomal

31
Q

What is the most common form of MS?

A

Charcot, chronic relapsing and remitting

32
Q

What are the three arrows pointing at?

A

Orange: Schwann cell

Blue: corpora amylacea body

Red: oligodendrocytes

33
Q

What are some potential causes of central pontine myelitis?

A

Alcoholism, liver transplant, electrolyte imbalances

Rapid sodium correction association

34
Q

What gene is associated with adrenoleukodystrophy?

A

ABCD1 Transporter gene (Xq28)

35
Q

What is being shown in this histo slide?

A

Transition from central to peripheral myelin

36
Q

What category of diseases: Myelin damage with relative preservation of axons?

A

Demyelinating disease

37
Q

What is elevated in the CSF of MS?

A

mild elevation in protein (oligoclonal bands), lymphoctes

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