[A case of acute disseminated encephalomyelitis with pathologically-proven acute demyelinating lesion in the peripheral nervous system].
We present a case of acute severe demyelination affecting both CNS and PNS with pathological evidence. A 62-year-old man presented with acute onset of coma preceded by common cold-like symptoms. He was diagnosed as acute disseminated encephalomyelopathy (ADEM), and died of brain herniation in two weeks. At autopsy, in addition to severe demyelination of the white matter of the central nervous system, there was widespread acute demyelinating process in the peripheral nervous system. Myelin destruction by macrophage was most conspicuous in the spinal nerve root with preservation of the axon cylinder. So far the association of hypertrophic demyelinating neuropathy was reported in cases of multiple sclerosis. However, there have not been any papers in which substantial involvement of both CNS and PNS in cases of ADEM except for the clinical report by Amit. Antigenic cross-reactivity between CNS and PNS against myelin proteins or other antigens like glycolipid may elicit similar immune responses producing demyelination.
Kinoshita
A
A
Department of Neurology, Neuropathology, Tokyo Metropolitan Neurological Hospital.
Kaseda
S
S
Yagi
K
K
Oda
M
M
Tanabe
H
H
D000208:Acute Disease; D003711:Demyelinating Diseases / Q000473:pathology*; D004673:Encephalomyelitis, Acute Disseminated / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010523:Peripheral Nervous System Diseases / Q000473:pathology*