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Neuro Round 2 wk 2+ > Demyelinating Dz JB > Flashcards

Demyelinating Dz JB Flashcards

1
Q

In the CNS, compacted myelin is produce by… and contains what two proteins?

A
  • oligodendrocytes
  • Proteolipid protein (PLP)
  • Myelin Basic Protein (MBP)
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2
Q

In the PNS, myelin is produced by… and is made of what two proteins?

A
  • Schwann Cells
  • Myelin protein zero (MPZ)
  • Peripheral myelin protein -22 (PMP-22)
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3
Q

An arrest in myelin production would be considered…

Examples of diseases like this would be…

A

hypomyelinating

rare peds dz - Pelizaeus Merzbacher, vanishing white matter dz

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4
Q

Diseases that cause abnormal myelin production are considered….

Examples include

A

Dysmyelinating

  • adenoleukodystrophy
  • Alexander’s Disease
  • Metachromatic leukodystrophy
  • Krabbe’s
  • Canavan
  • Phenylketonuria
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5
Q

Disease that cause destruction of existing myelin are considered…

A

Demyelinating

  • MS
  • Acute disseminated encephalomyelitis (ADEM)
  • AIDP/GBS
  • CIDP
  • Charcot-Marie-Tooth
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6
Q

Alexander’s Disease has features of a GFAP mutation and Rosenthal fibers. An infant might present with megalencephaly. What type of disease is it, and where does it localize to?

A

Dysmyelinating Disease

Frontal Leukodystrophy

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7
Q

A lysosomal frontal leukodystrophy is…

This is characterized by a deficiency in what? This deficiency causes accumulation of…. and ultimately defective CNS and PNS myelination (dysmyelinating).

What are unique ssx for an adult?

A

Metachromatic leukodystrophy

arylsulfatase A deficiency causing cerebroside sulfate accumulation

adult form is dementia with peripheral neuropathy and cholecystitis

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8
Q

A galactocerebrosidase beta-galatosidase deficiency could lead to…

with ssx of…

A

Krabbe - posterior leukodystrophy

optic atrophy/blindness, sz, psychomotor retardation, peripheral neuropathy; infantile, juvenile and adult forms

kids most often

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9
Q

High plasma VLCFAs and a childhood onset behavior abnormalities and adrenal insufficiency is making me think…

A

adrenoleukodystrophy - posterior leukodystrophy

dysmyelinating disease

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10
Q

An autoimmune disorder with autoreactive lymphocytes is…

The primary phases of this dz are…

A

MS

  • inflammation
  • demyelination/axon loss of gray and white matter
  • gliosis/neurodegeneration of brain and spinal cord (Disability)
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11
Q

Location wise, who has an increased risk of getting MS?

A

increased distance from the equator, decreased sun exposure

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12
Q

85% of people with MS present with…

A

clinically isolated syndrome - solitatry demyelinating event with ssx lasting more than 24 hours

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13
Q

Optic neuritis is a common presentation of MS. This pt will be… and have ssx like…

A
  • female 30-40
  • rapid visual loss over hours to days
  • pain on EOM movement
  • relative afferent pupillary defect on exam
  • fundus is often normal
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14
Q

What are some factors a/w aggressive MS?

A
  • male
  • older than 40
  • AA
  • motor involvement
  • cerebellar involvement
  • poor recovery from relapses
  • low vit D
  • smoker
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15
Q

Diagnosis of MS must satisfy the McDonald Criteria, which includes…

A
  • Exclusion of other dx
    • optic neuritis from infection or compressive
    • transverse myelitis - spinal dural AV fistula, B12/Cu deficiency, tumor or parneoplastic syndrome, Alexander’s Dz
    • MRI lesion: tumor, stroke, vascular malformation
  • Time and Space Criteria
    • dissemination in time: more than 1 attack
    • dissemination in space: at least one lesion in two of 4 areas
      • juxtacortical, cortical, periventricular, posterior fossa/SC
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16
Q

What am I looking at?

A

Dawson’s fingers, characteristic of MS

17
Q

What are some ways to tx RRMS?

A
  • beta interferons
  • fingolimoid
  • dimethyl fumerate
  • natalizumab
  • rituzimab
  • Ocrelizumab gets both PPMS and RRMS
18
Q

What is a risk of a few tx of MS?

A

risk PML caused by JC virus

19
Q

What qualifies as an MS flare/lapse?

What is the tx for this?

A

lapse lasts >24 hours and cannot be attributed to infection or heat exposure

methylprednisolone 1g IV per day for 3 days followed by prednisone taper

ACTH for pt that cannot tolerate steroids

plasma exchange is another option

20
Q

Neuromyelitis Optic specturum disorders are caused by autoimmune factors like..

A

antibodies to aquaporin-4

a water channel on astrocytic foot processes, exposed in optic nerves, brain and SC

21
Q

What are clinical features of neuromyelitis optica?

A
  • F>M 9:1
  • optic neuritis, usually bilateral and severe
  • longitudinally extensive transverse myelitis (3 vertebral segments involved), involves central cord, can be complete; v painful
  • area postrema syndrome
    • hiccups, nausea, vomiting
22
Q

How do you tx neuromyelitis Optica?

A

rituximab for maintenance, IVMP or plasma exchange for acute exacerbations

23
Q

contrasting MS, NMO lesions are often..

A

large and follow ependymal lining

24
Q

Clinical presenation of sensory loss, pain, weakness, ataxia, bowel or bladder dysfunction

with ssx progressing overing hours, days, and weeks - peaking at 4 weeks if left untx’d

consider dx of …

A

transverse myelitis

25
Q

Acutely, in transverse myelitis, I am going to see what on exam? What should I be looking for?

A

flaccid weakness with decreased reflexes

must look for SC sensory level

26
Q

kid aged 5-8 yo, just had a viral illness, super sleepy, getting more difficult to arouse…

dx?

What are some key features?

A

acute disseminated encephalomyelitis (ADEM)

  • monophasic>>>>> multiphasic
  • encephalopathy is a prominent feature
  • bilateral asymmetric large T2 hyperintense white matter lesions
  • T1 hypointense lesions are rare (common in MS or NMO)
  • oligoclonal bands are usually absent
27
Q

What will I see on imaging of the brain in ADEM?

A

bilateral, large, asymmetric, multifocal lesions with poorly demarcated borders

28
Q

What is the MC ssx of MS?

A

fatigue

29
Q

What is the MC AE at the onset of tx with interferons for tx of MS?

A

flu-like ssx

Neuro Round 2 wk 2+ (16 decks)

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