Other Demyelinating Diseases of Unknown Pathogenesis
In addition to the genetic, immune-mediated, and metabolic myelinopathies, there are a number of other demyelinating diseases whose causes remain unknown. These diseases are briefly listed and characterized in Table 8.3.
Table 8.3 Rarer demyelinating diseases
Disease
Clinical manifestations
Special features
Concentric sclerosis (Baló sclerosis, periaxial encephalitis)
Acute disseminated encephalomyelitis (ADEM)
Neuromyelitis optica
(Devic disease)
Subacute myelo-optic neuropathy (SMON)
onset at any age, slow progression, associated with dementia acute multiple sclerosis; simultaneous appearance of symptomatic foci in the brain and spinal cord; acute onset, with fever; peripheral nervous system also involved simultaneous appearance of symptomatic foci in the optic nerve and spinal cord; mainly affects young women ascending paresthesiae and weakness of the lower limbs, appearing days or weeks after a gastrointestinal illness; the optic nerve is also involved in one-third of patients concentric bands of demyelination
MRI shows foci that arose simultaneously; his-topathological examination shows axonal injury as well; dramatic course; responds to cortisone and immunosuppressive drugs elevated CSF protein; no oligoclonal bands in CSF; inflammatory axonal damage most common in Japan; many patients have a prior history of oxyquinoline use
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