Demyelinating Diseases

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 Demyelinating Diseases
Central Nervous System Peripheral Nervous System

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CC (0): Andrew C

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 Demyelinating Diseases
• Multiple sclerosis
• Internuclear ophthalmoplegia
• Optic neuritis
• Transverse myelitis
• Neuromyelitis Optica

• Progressive Multifocal Leukoencephalopathy

CC (0): Andrew C
• Guillain Barre Syndrome

• Other Disorders

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 Multiple Sclerosis (MS)
• Presentation: Woman age 15-50 w/ ≥2 neurologic symptoms that are

disseminated in space and time

• Ophthalmologic (Internuclear Ophthalmoplegia, Optic Neuritis)
• UMN (Urge incontinence, Back spasms)
• LMN (weakness)
• Sensory loss/paresthesias (e.g. Lhermitte)
• Symptoms worse w/ heat (Uhthoff's phenomenon)

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 Multiple Sclerosis (MS)
• Courses:
• Relapsing-remitting (has episodes => completely recovers)
• Primary progressive (has episodes => completely recovers => then
progresses)
• Secondary progressive (progressively worsens, may have temporary recoveries)

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 Internuclear Ophthalmoplegia
• Presentation: Impaired adduction on conjugate gaze
• Nystagmus of contralateral eye
• Convergence and pupillary light reflex preserved
• Characteristically bilateral in MS (vs. CVA)

Leftward Gaze

Rightward Gaze

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 Internuclear Ophthalmoplegia
• Pathophysiology: Median Longitudinal Fasciculus (MLF) destruction

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 Optic Neuritis
• Presentation: Monocular vision loss + Pain w/ eye movement
• Loss of pupillary light reflex (Marcus-Gunn pupil)

• Pathophysiology: Optic nerve (CN II) lesion

Shine light in normal eye

Shine light in affected eye

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 Transverse Myelitis (TM)
• Presentation: Band-like sensation around the abdomen (“MS Hug”)
• Motor and sensory loss below the level of the lesion
• Flaccid => Spastic
• TM + Bilateral optic neuritis = Neuromyelitis Optica (NMO)

• Pathophysiology: Inflammation across width of the spinal cord

• If NMO => Anti-AQ4 antibodies

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 Multiple Sclerosis
• Pathophysiology: Autoimmune destruction of myelin in the CNS
• Locations: Periventricular, cerebellar, spinal cord
• ↑ prevalence w/ ↑ distance from the equator

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Domain

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 Multiple Sclerosis
• Dx: MRI => Dawson’s fingers, White matter lesions (old and new)
• If not definitive => Lumbar Puncture
• CSF => IgG Oligoclonal bands
=> Normal protein level and cell count

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 Multiple Sclerosis
• Mgmt: IFN-β, Glatiramer, Natalizumab
• Symptomatic control (e.g. Back spasms => Baclofen)
• If relapsing-remitting or primary progressive => Ocrelizumab (CD20)
• Acute flares => IV Methylprednisolone
• Plasmapheresis

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 Progressive Multifocal
Leukoencephalopathy (PML)
• Presentation: Ataxia, confusion, seizures
• Slowly progressive

• Pathophysiology: JC virus infection (Oligodendrocytes)

• HIV
• Natalizumab

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 Progressive Multifocal
Leukoencephalopathy (PML)
• Dx: MRI => Multifocal asymmetric white matter lesions
• No ring-enhancement or edema (vs. 1° CNS lymphoma)
• LP => CSF PCR
• Brain Biopsy (Rare)

• Mgmt: Highly Active Antiretroviral Therapy (HAART)

• HAART = 2 NRTIs + 1 Protease Inhibitor (OR 1 NNRTI)
• Supportive measures
CC (4.0): Hellerhoff

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 PML Toxoplasmosis 1° CNS Lymphoma

Mentzer, Alex, et al. "Is it all cerebral toxoplasmosis?." The

Lancet 379.9812 (2012): 286.
CC (4.0): Hellerhoff GNU (1.2): Tdvorak

• Multiple, asymmetric • Multiple • Single

• No ring-enhancement • Ring-enhancing • Ring-enhancing
• No edema • Edema • Edema

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 Guillain-Barré Syndrome (GBS)
• Presentation: Acute Inflammatory Demyelinating Polyneuropathy

(AIDP) is most common

• Infection followed by rapid ascending paralysis, ↓ DTRs
• Progresses over days to weeks => Respiratory/Facial/Bulbar involvement
• Paresthesias in the distal legs
• +/- Autonomic dysfunction (e.g. arrhythmia, orthostatic hypotension)

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 Guillain-Barré Syndrome (GBS)
• Pathophysiology: Autoimmune Schwann destruction (endoneurium)
• Campylobacter jejuni (most common)
Campylobacter jejuni
• Haemophilus influenzae
• Mycoplasma pneumoniae
• CMV
• Hepatitis
• HIV

CC (0): Arcardian CC (0): Haggstrom

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 Guillain-Barré Syndrome (GBS)
• Dx: Exam => ↓ DTRs
• Lumbar puncture => Albuminocytologic dissociation
• CSF => Normal cell count, ↑ Protein
• EMG => ↓ nerve conduction velocity
• Spirometry => Monitor FVC

• Mgmt: If FVC drops => Intubation

• Plasmapheresis; IVIG

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 GBS DDx
• If descending paralysis => Botulism

• If ascending paralysis, but tick present => Tick-induced paralysis

• If ascending paralysis, but tick absent => GBS

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 Other Demyelinating Diseases
• Rapid correction of hyponatremia => Central Pontine Myelinolysis

• Boy w/ Ataxia + Decreased cortisol => X-linked

Adrenoleukodystrophy

CC (0): Public Domain

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 Demyelinating Diseases
• Multiple sclerosis
• Internuclear ophthalmoplegia
• Optic neuritis
• Transverse myelitis
• Neuromyelitis Optica

• Progressive Multifocal Leukoencephalopathy

CC (0): Andrew C
• Guillain Barre Syndrome

• Other Disorders

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