Asymmetrical polyneuropathy with a stepwise progressive course and well-demarcated areas of demyelination

A female patient was 12 years old when she presented with hemiatrophy and m uscle weakness on the right side of her body. Then a stepwise worsening occ urred, and at 19 years of age sensory symptoms were also noticed, as well a s a mild involvement of the left part of her body. The cerebrospinal fluid (CSF) protein level was elevated without cells. The main electrophysiologic al abnormality was a marked temporal dispersion of the compound muscle acti on potentials (CMAPs). Motor nerve conduction velocities were moderately re duced. A superficial peroneal nerve biopsy revealed well-demarcated areas o f demyelination with prominent Schwann cell hyperplasia. Neither deletion n or duplication of the PMP22 gene nor mutation of the PO or connexin 32 gene s was found by molecular genetic investigations. Immunotherapy was administ ered, and over the next 6 years the symptomatology fluctuated. This unusual disorder seems to be a variant of chronic acquired demyelinating polyneuro pathy and may be immunologically mediated. (C) 1999 John Wiley & Sons, Inc.