hereditary motor & sensory neuropathy

hereditary motor & sensory neuropathy

A group of conditions dignified by eponym and subdivided as follows.

hereditary motor & sensory neuropathy

Type I Charcot-Marie-Tooth disease, hypertrophic form An AD–type 1a, type 1b, or X-linked condition that is the most common form of HMSN, characterized by a slowly progressive disease of childhood onset with predominantly motor symptoms, pes cavus, calf atrophy, very slow motor impulse conduction with segmental demyelination and remyelination with 'onion-bulb' formation

Type II Roussy-Levy syndrome, or Charcot-Marie-Tooth disease, neuronal form An AD condition characterized by slowly progressive disease of adolescent onset with predominantly motor symptoms, clubfoot deformity, calf atrophy, mild reduction in impulse transmission and 'onion-bulb' formation

Type III Dejerine-Sottas disease A rare relentlessly progressive AD condition of infant onset with short stature, scoliosis, pes cavus, calf atrophy, very slow conduction of motor impulses with segmental demyelination and remyelination 'onion-bulb' formation. See Hereditary sensory neuropathy.

McGraw-Hill Concise Dictionary of Modern Medicine. © 2002 by The McGraw-Hill Companies, Inc.