Journal of Neurology, Neurosurgery, and Psychiatry, 1995, Vol 58, 236-237
"Pseudo" hypertrophic neuropathy of childhood
M Baba, H Takada, H Miura, T Okushima and M Matsunaga
Department of Neurology, Hirosaki University School of Medicine, Japan.
A 9 year old boy had chronic progressive motor-sensory neuropathy that
started in early infancy. He had enlarged nerves and pes cavus deformity.
Motor conduction studies showed very dispersed, polyphasic compound muscle
action potentials with conduction velocities around 2 m/s. A sural nerve
biopsy showed severe loss of myelinated fibres. Two months of treatment
with corticosteroids restored muscle power. During this time the enlarged
nerves became normal and electrophysiological recovery was achieved.
Chronically acquired neuropathy in infancy is strikingly similar to
genetically determined neuropathy.
