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Neuroinflammatory Disorders

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Acute disseminated encephalomyelitis (ADEM)

Overview:

Widespread attack of inflammation in the central nervous system thought to be provoked by an infectious cause or occurring post vaccination

Features:

  • Multifocal areas of diffuse inflammation most often seen in the subcortical cerebellum, brainstem, and spinal cord
  • Usually monophasic but can be recurrent

Symptoms:

  • Encephalopathy: confusion, irritability, AMS (somnolence to coma)
  • Multifocal deficits, fever, meningismus (headache, photophobia, stiff neck)

Imaging:

  • Large (greater than 1 to 2 cm) multifocal, hyperintense, bilateral, asymmetric lesions in the supra-/infratentorial white matter on T2-weighted or MRI FLAIR images
  • Can be seen in gray matter, especially basal ganglia and thalamus

Resources:

Accute Optic Neuritis (AON)

Overview:

Optic neuritis is retrobulbar inflammation of the optic nerve that can occur spontaneously or be associated with other neurologic conditions such as MS, ADEM, NMO or lupus.

Features:

  • Usually unilateral
  • Dimished visual acuity, red color desaturation, afferent pupillary defect, and visual field deficits

Symptoms:

  • Headache and painful eye movements
  • Central visual blurring
  • Dim vision
  • Complete loss of vision

Imaging:

  • MRI often shows gadolinium enhancement of the optic nerve

Resources:

Transverse Myelitis

Features:

  • Spinal cord dysfunction typically owing to inflammatory lesion
  • Usually presents as a CIS, but may be associated with MS or ADEM

Symptoms:

  • Unilateral or bilateral motor or sensory deficits such as Paresthesias, weakness, sphincter dysfunction; can occasionally be more severe, including paraplegia and urinary retention

Imaging:

  • Gadolinium-enhancing lesions on MRI spreading over 1 or more segments

Resource:

Neuromyelitis Optica (NMO)

Overview:

Neuromyelitis Optica and neuromyelitis optical spectrum disorder (NMOSD) are inflammatory conditions of the central nervous system primarily targeting the optic nerve and spinal cord.

Features:

  • Optic neuritis
  • Acute myelitis
  • Postrema clinical syndrome
  • Acute brainstem syndrome
  • Symptomatic narcolepsy or acute diencephalic clinical syndrome with NMOSD-typical diencephalic MRI lesions
  • Symptomatic cerebral syndrome with NMOSD-typical brain lesions
  • More common in non-Caucasians, especially Asians
  • Important to rule out sarcoid, SLE, Sjogrens or other vasculitis

Symptoms:

  • Optic neuritis (see above)
  • Transverse myelitis (see above)
  • Intractable hiccups or nausea and vomiting
Resources:

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