Variants of Multiple Sclerosis

Neuromyelitis optica or Devic's disease is characterized by an acute inflammatory demyelination confined to the optic nerves and spinal cord, often the cervical segments. The demyelination, in severe cases, progresses to necrosis and even cavitation (Fig. 8.13).

Charcot classic type MS shows the typical clinical features and course of MS.

Marburg type MS has an acute onset and rapid progression. Death usually occurs within 1 to 6 months of onset.

Balo's concentric sclerosis is characterized by concentric zones of demyelination alternating with zones of intact myelin, believed to represent remyelination

FIGURE 8.13

Neuromyelitis optica or Devic's disease. A 30-year-old woman presented with a history of headaches and rapidly progressing loss of vision in the left eye that began about 5 weeks following a difficult labor and a short febrile episode. Shortly after the onset of visual impairment, she developed paresthesias and weakness in all four extremities and urinary retention. After a short period of improvement, her condition deteriorated; she became quadriplegic and experienced respiratory difficulties. Six months after the onset of symptoms, she died. A. Extensive demyelination in left optic nerve. B. Extensive demyelination in the swollen cervical and thoracic cord (Weil stain). C. Massive perivascular lymphocytic infiltration (HE).

FIGURE 8.13

Neuromyelitis optica or Devic's disease. A 30-year-old woman presented with a history of headaches and rapidly progressing loss of vision in the left eye that began about 5 weeks following a difficult labor and a short febrile episode. Shortly after the onset of visual impairment, she developed paresthesias and weakness in all four extremities and urinary retention. After a short period of improvement, her condition deteriorated; she became quadriplegic and experienced respiratory difficulties. Six months after the onset of symptoms, she died. A. Extensive demyelination in left optic nerve. B. Extensive demyelination in the swollen cervical and thoracic cord (Weil stain). C. Massive perivascular lymphocytic infiltration (HE).

(Fig. 8.14). Schilder's disease refers to extensive demye-lination in the cerebral hemispheres, with sudanophilic breakdown products (Fig. 8.15). The disease affects both children and adults.

The association of MS with inflammatory demye-linating polyradiculopathy is rare. A few cases have been documented using MRI, electrophysiologic studies, and nerve biopsy.

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