Acute disseminated encephalomyelitis in a female with hereditary neuropathy with susceptibility to pressure palsy

A 7-year-old female presented with fever, urinary incontinence, mental regr ession, gait disturbance, and lethargy after diarrhea, Magnetic resonance i maging revealed multifocal T-2-weighted hypersignal lesions supportive of a cute disseminated encephalomyelitis. Her mother had been diagnosed with her editary neuropathy with susceptibility to pressure palsy. The girl was also determined to have hereditary neuropathy with liability to pressure palsy, with a 1.5-Mb deletion in chromosome 17p11.2 encompassing the gene for per ipheral myelin protein 22 detected by fluorescent in situ hybridization, He reditary peripheral neuropathies may be a factor in triggering the autoimmu ne demyelinating disorder of the central nervous system. (C) 2000 by Elsevi er Science Inc. All rights reserved.