Autoimmune disorders of the nervous system

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jknell
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193985
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Autoimmune disorders of the nervous system
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2013-01-20 17:55:01
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MBB II
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Autoimmune disorders of the nervous system
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  1. General Principles of Autoimmune disorders
    • 1. Direct (subacute)
    • -Cell mediated: multifocal, episodic
    • -Antibody mediated: diffuse, continuous or monophasic
    • 2. Vasculitic
    • -Large vessel injury: infarctions, acute
    • -Microvascular injury: indolent, diffuse, subacute or chronic
  2. Multiple Sclerosis
    - Clinical Presentation
    - Epidemiology
    • Clinical Presentation: Episodes of subacute onset (hours - days), with partial or full
    • resolution (weeks - months), affect any part of CNS
    • -UMN syndromes
    • -Cranial neuropathies
    • -autonomic dysfunction
    • -optic neuritis

    Multiple lesions in time and space
  3. Multiple Sclerosis
    -Pathophysiology
    -Diagnosis
    • Pathophysiology:
    • CD4 T cells react against myelin antigens and secrete cytokinesdemyelination, inflammationineffective remyelination impairs saltatory conductionDiagnosis:Imaging: T2 MRICSF: oligoclonal bands (IgG)Evoked potentials (slower conduction)
  4. Multiple Sclerosis
    -Treatment
    • Acute Relapse:IV glucocorticoids (decrease recovery time but don't change overall courseChronic Disease Modification:Glatiramer acetate
    • InterferonsNatalizumabFingolimodSymptomatic Therapy:Neuropathic pain (caramazepine, gapapentin, duloxetine)Spasticity (baclofen, tizanidine)Urinary Urges (oxybutinin)Fatigue (amantadine, modafinil, methylfenidate)

    Types:

    • 1. Relapsing-Remitting
    • 2. Secondary Progressive
    • 3. Primarily Progressive (older, male > female)


    • Epidemiology: more common in far northern and southern latitudes, strong
    • genetic component, 2-3x more common in women, ages 20-40
  5. Multiple Sclerosis

    -Pathophysiology
    -Diagnosis
    Pathophysiology:

    • CD4 T cells react against
    • myelin antigens and secrete cytokinesdemyelination, inflammationineffective remyelination
    • impairs saltatory conduction

    Diagnosis:

    Imaging: T2 MRICSF: oligoclonal bands (IgG)

    Evoked potentials (slower conduction)
  6. Multiple Sclerosis
    -Treatment
    Acute Relapse:

    • IV glucocorticoids (decrease
    • recovery time but don't change overall course

    Chronic Disease Modification:

    Glatiramer acetate InterferonsNatalizumabFingolimod

    Symptomatic Therapy:

    • Neuropathic pain (caramazepine,
    • gapapentin, duloxetine)Spasticity (baclofen,
    • tizanidine)Urinary Urges (oxybutinin)

    Fatigue (amantadine, modafinil, methylfenidate)
  7. Glatiramer acetate
    • MOA:
    • -synthetic peptide similar to MBP, exact mechanism unknown

    • Effects:
    • -decreases relapses by 1/3
    • -no neutralizing antibodies develop

    • Adverse Effects:
    • -delayed onset by months
    • -injection site reactions
  8. Interferons
    • MOA:
    • -decrease expression of pro-inflammatory cytokines
    • -reduce T cell migration across BBB
    • -increases production of NGF

    • Effects:
    • -similar to glatiramer

    • Adverse Effects:
    • -injection site reaction
    • -neutralizing antibodies
    • -flu-like symptoms
    • -liver dysfunction (rare)
    • -depression (rare)
  9. Natalizumab
    • MOA:
    • -monoclonal antibody that inhibits alpha-4 integrins
    • -prevents lymphocytes from entering CNS

    • Effects:
    • -decrease relapse by 60%
    • -decrease MRI lesions by 90%

    • Adverse Effects:
    • -1/1000 risk of progressive multifocal leukoencephalopathy (PML)
  10. Fingolimod
    • MOA:
    • -binds S1P receptors
    • -blocks lymphocytes from exiting LNs

    • Effects:
    • -only oral drug for MS
    • -more effective than IFN

    • Adverse Effects:
    • -bradycardia
    • -leukopenia
  11. Temporal Arteritis
    • Clinical Presentation:
    • -subacute to chronic HA, jaw claudication, nodular temporal artery, blindness


    • Epidemiology:
    • -most common in the elderly

    • Pathophysiology:
    • -chronic, granulomatous inflammation of large arteries (most often temporal
    • artery)
    • -initial T cell response against vascular wall antigen
    • -segmental regions of nodular intimal thickening with lumen narrowing


    • Diagnosis:
    • -Clinical suspicion
    • -ESR
    • -temporal artery bx

    • Treatment:
    • -high dose glucocorticoids (prednisone)
  12. Guillain Barre Syndrome
    • Clinical Presentation:
    • -subacute onset progressive ascending paralysis
    • -early mild sensory change
    • -painful
    • -typical LMN syndrome
    • -autonomic dysfunction
    • -out of degree areflexia
    • -85% recover (usually 2-3 mo, some 6-18 mo)

    • Epidemiology:
    • -60% preceeded by URI or GI illness
    • -relation to C. jejuni

    • Pathophysiology:
    • -T cells initiate response against myelin
    • -segmental demyelination throughout PNS
    • -the longer the nerves, the more lesions --> distal polyneuropathy

    • Treatment:
    • -ABCs
    • -Plasmapheresis
    • -IVIg
    • -Glucocorticoids are NOT effective
  13. Myasthenia Gravis
    • Clinical Presentation:
    • -fluctuating weakness
    • -weakness that worsens with exercise and improves with rest
    • -affects small, constantly used muscles first (EOMs, hands)

    • Epidemiology:
    • -third decade for women
    • -sixth decade for men
    • -associated with thymoma or thymic hyperplasia

    • Pathophysiology:
    • -antibodies block AChR
    • -increased degradation of AChRs
    • -Abs cause C' mediated degradation of postsynaptic folds

    • Diagnosis:
    • -serum antibodies
    • -decrementing response
    • -pharmacologic challenge (edrophonium or tensilon)

    • Treatment:
    • -Anticholinesterase drugs
    • -Chronic glucocorticoids
    • -plasmapheresis
    • -IVIg
    • -thymectomy
  14. Paraneoplastic Disorders
    -subacute to chronic progressive diffuse lesion

    • 1. Cerebellar degeneration
    • 2. Encephalomyelitis
    • 3. Opsoclonus-myoclonus-ataxia
    • 4. Subacute sensory neuropathy
    • 5. Lambert-Eaton myasthenic syndrome
  15. Cerebellar Degeneration
    • -onset: weeks to months
    • -symmetrical ataxia, dysarthria, nystagmus
    • -imaging: subtle enhancement of cerebellum
    • -associated with ovarian and lung cancer
  16. Encephalomyelatis
    • -subacute onset
    • -often bilateral
    • -medial temporal lobe
    • -confusion, agitation, amnesia, dementia
    • -associated with lung, prostate, lymphoid, ovarian, testicular CA
  17. Opsoclonus-myoclonus-ataxia
    • -predominantly childhood syndrome
    • -"bouncing eyes"
    • -associated with Neuroblastoma
  18. Subacute sensory neuropathy
    • -rapid onset polyneuropathy
    • -small cell lung cancer, lymphoma
    • -often rapidly leads to death, refractory to tx
  19. Lambert-Eaton myasthenic syndrome
    • -NMJ dysfunction due to failure of
    • vesicle fusion
    • -anti-voltage gated Ca channel antibodies
    • -improves with exercise (incrementing response)
    • -small cell lung cancer
  20. Acute demyelinating encephalomyelitis (ADEM)
    • -multifocal areas of myelin breakdown
    • -monophasic (all lesions enhance on MRI)
    • -typically follows an acute infection

    • Clinical Presentation:
    • -rapid subacute progression
    • -HA, confusion, fever, ataxia

    • Epidemiology:
    • -children more commonly affected in adults
    • -in adults can be associate with small pox vaccine
  21. Dermatomyositis
    • Clinical Presenation:
    • -muscle weakness
    • -heliotrope rash
    • -childhood variant is different

    • Pathophysiology:
    • -inflammatory infiltrates around small blood vessels and in perimysial CT
    • -B cell mediated
  22. Poliomyositis
    • Clinical Presentation:
    • -painless symmetrical weakness

    • Pathophysiology:
    • -inflammation in the endomesium scattered throughout the fascicles
    • -due to CD8 T cells

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