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BMB W11 > 10-21 L1 Demyelinating and Degenerative disease > Flashcards

10-21 L1 Demyelinating and Degenerative disease Flashcards

1
Q

Name the 6 Demyelinating Diseases

A
  • Remyelination
  • GBS (Guillain-Barre syndrome)
  • MS (Multiple Sclerosis)
  • CPM (Central Pontine Myelinolysis)
  • PML (Progressive multifocal Leukoencephalopathy)
  • ADEM (Acute Disseminated Encephalomyelitis)
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2
Q

Name the 4 Degenerative Diseases?

A
  • CMT (Charcot Marie Tooth Disease)
  • FA (Freidreich’s Ataxia)
  • HD (Huntington’s Disease)
  • AML (Amyotrophic Lateral Sclerosis)
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3
Q

What primary demyelination is direct damage to what?

A

Direct damage to the oliodendroglia or schwann cells with relative preservation of axons.

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4
Q

Name the 3 causes that demyelination can cause clinical symptoms because of conduction block:

A
  • Voltage-gated sodium channels exist only at nodes of ranvier
  • Demyelinated strect of axon cannot produce action potentials (no voltage-gated Na channels)
  • Demyelinated axons have terrible cable properties
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5
Q

Remyelination

reverses clinical symptoms by what mechanism?

A
  • restores cable properties (sufficiently for action potentials to be triggered at nodes of Ranvies)
  • Conduction velocities of remyelinated axons are slow (diagnostic nerve conduction velocites)
  • time lag is clinically insignificant
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6
Q

Name key symptoms of GBS (guillain-Barre Syndrome)

A
  • Demyelinating peripheral neuropathy
  • Ascending paralysis
  • Loss of reflexes
  • Respiratory failure is most common cause of death (preventable)
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7
Q

What causes weakness in GBS?

What is elevated in CSF?

A
  • weakness is caused by a conduction block
  • Spinal fluid protein is elevated (otherwise CSF is normal)
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8
Q

What is used to treat pts w/GBS?

A
  • Intravenous immunoglobulin (IVIG)
  • plasmapheresis
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9
Q

Desribe the epidemiology of MS (multiple sclerosis)

  • Def.
  • incidince
  • gender
  • geneitic (hereditary)
  • regional observations
A
  • **Def.: **autoimmune disease of young adults (95% of cases are from 15-50)
  • incidince: 2nd most common cause of neurological disability (1st is trauma)
  • gender: 2F/1M
  • geneitic (hereditary): concordance is 1/3 in identical twins, 1/15 fraternal twins)
  • regional observations: latitidue (temperature zone is high, tropics low) and adolescence.
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10
Q

MS has a wide range of varited subacute presentations, what are they?

A
  • Monocular loss of vision, diplopia, walking trouble, bladder dysfunction are relatively common
  • Psychiatric or behavioral changes reflect frontal lobe involvement.
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11
Q

Describe the Intranuclear Opthalmoplegia (seen in pts w/MS)

A
  • Lesion of MLF
  • Impaired adduction of contrallateral eye
  • Nystagmus in abducting eye
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12
Q

What are common findings on pts with MS?

Assuming that you have made sure lumbar puncture is safe

A
  • Modest pleocytosis (increase in CSF white blood cells).
  • Elevated CSF index
  • Presence of oligoclonal bands
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13
Q

What are som imitators of MS?

A
  • Mass lesions (CNS lymphoma)
  • SLF (young women)
  • Neurosyphilis
  • Sarcoidosis
  • HIV-related CNS disease
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14
Q

Which two diseases can be treated with IV methylprednisone?

A
  • MS (Multiple sclerosis)
  • ADEM (Acute disseminated Encephalomyelitis)
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15
Q

Name the 6 medications used for MS.

A
  • IV methylprednisone
  • Beta-interferon (30-40% reduction in relapse)
  • Glatiramer acetate (30-40% reduction)
  • Natalizumab
  • Fingolimod (oral)
  • Dimethyl fumarate & teriflunomide (other oral agents)
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16
Q

beta interferon (30-40% reduciton in relapse rate)

A

used to treat MS

  • inhibits pro-inflammatory cytokines, T-cell proliferation, CNS trafficking
  • myalgias chills, SQ & IM
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17
Q

Glatiramer acetate (30-40% reduction)

A

used in MS

  • synthetic polypeptide SQ
  • Similar efficacy and side effects beta interferons
18
Q

Natalizumab

A
  • desinger antibody (binds to integrin- cellular adhesino molecule)
  • blocks lymphocytes-endothelial binding/penetrating BBB
  • IV monthly, causes PML (0.2%)
19
Q

Fingolimod

A

(oral, $$$)

  • block egress of lymphocytes from lymph nodes and spleen
  • heart block, oral (first case of PML reported 08-29-213)
20
Q

CPM

(Central pontine myelinolysis)

A
  • Demyelination of the ventral pons
  • caused by: rapid correctino of hyponatremia (malnutrition and alcohol may increase risk)
  • May present as a locked-in state or quadraplegia (more extensive lesions cause coma)
  • has the tridant sign
21
Q

An immunocompromised pt, informs you of initial complaints of aphasia, visual field defects, hemiparesis.

The pt family member informs you of instances of confusional states and seizures.

What’s your diagnosis?

A

PML (progressive multifocal leukoencephalopathy)

22
Q

What are theclinical findings of PML:

on MRI?

on CT?

A
  • MRI is excellent
    • non-enhancing
    • no mass effects
  • CSF is usually normal
    • PCR: JCV
    • serum JC antibodies (60-80% of population)
  • IRIS (immune reconstitution inflammation syndrome)
23
Q

What is ADEM?

A
  • Acute disseminated Encephalomyelitis: asymmetrical swelling of the brain
  • follows viral infection or vaccination
24
Q

What is used to diagnosis it?

A
  • CT shows cerebral edem and mass effects
  • MRI shows gadolinium enhacement of white matter
25
Q

What is in the CSF?

A
  • CSF shows moderate lymphocytic pleocytosis (50-200 cells) elevated protein (and less commonly oligoclonal bands)
26
Q

What is used to treat it?

A
  • IV methylprednisolone
  • hemicraniectomy: to prevent herniation and death
27
Q

What is a length dependent sensorimotor peripheral neuropathy?

A

**CMT **

Charcot marie tooth disease

28
Q

CMT1 vs CMT2

A
  • CMT1: has very slow motor conduction velocities (dysmyelinating)
  • CMT2: has normal motor conduciton velocities (axonal)
    • more complicated genetics and less obvious molecular biology/pathophysiology connections
29
Q

CMT1A

A

PMP22 duplication, 75% of all CMT1

30
Q

CMT1B

A

Myelin protein zero 20% of all CMT1

31
Q

CMT1X

A

lools like CMT1 but never has male-to-male inheritance (connexin 32)

32
Q
A
33
Q

Freidreich’s ataxia

A
  • most common hereditary ataxis (>50% of total)
  • autosomal recessive
  • insidious onset adolescence/young adult (up to 30’s)
34
Q

What is the triad of Freidreich’s Ataxia?

(95% of pts have this)

A
  • bilateral babinski responses (spasticity)
  • absent ankle reflexes (peripheral neuropathy)
  • dysmetria (ataxia)
35
Q

What are some multisystem probles commonly seen with Freidreich’s ataxia?

A
  • Hypertrophic cardiomyopathy (majority)
  • Diabetes (1:4)
  • Scoliosis (1:4)
36
Q

What is the genetic mutation in Freidreich’s Ataxia?

A
  • frataxin gene (expanded GAA repeat, 9q13)
    • Mitochondrial protein
    • Disturbance of iron homeostatsis
37
Q

What are the 3 things that raise suspicion of huntington’s disease?

A
  • Psychiatric/cognitive problems
  • Chorea
  • Dominant inheritance
38
Q

Huntington’s Disease

  • Onset:
  • MRI findings
  • genomic tests
A
  • 5-70 yo (usually around 25-45)
  • MRI: caudate atrophy/diffuse atrophy
  • expanded CAG repeats (chromosome 4)
39
Q

Describe ALS (Amyotrophic lateral sclerosis)

A
  • UMN & LMN findings
  • Cognitively normal (a subset has frontotemporal dementia)
  • Does not affect sensation, bladder and bowel function or eye control
40
Q

Riluzole

  • Incidence
  • MOA
A
  • Incidence: ALS
  • MOA: inhibits glutamate release and slows the disease slightly

BMB W11 (9 decks)

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