Axonal CMT with Axonal GBS Research - from Japan

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Abstract from the Journal of Neurological Science 2003 Jul

15;211(1-2):93-7

Axonal Guillain-Barre syndrome associated with axonal

Charcot-Marie-Tooth disease.

Odaka M, Yuki N, Kokubun N, Hirata K, Kuwabara S.

Department of Neurology, Dokkyo University School of Medicine,

Kitakobayashi 880, Mibu, Shimotsuga, 321-0293, Tochigi, Japan

We report the first case of axonal Guillain-Barre syndrome (GBS)

associated with axonal Charcot-Marie-Tooth disease (CMT). A 30-year-old

Japanese man, who had suffered leg atrophy and foot deformity since

childhood, developed acute weakness in his four limbs ollowing an upper

respiratory tract infection. Nerve conduction studies showed low

compound muscle action potential (CMAP) and sensory nerve action

potential (SNAP) amplitudes in all the nerves tested. Serial studies

showed a rapid increase in CMAP amplitude, but no significant change in

SNAP, which indicates that the acute event selectively involved motor

axons and was superimposed on a baseline motor-sensory axonal

neuropathy, probably CMT Type 2. Elevated serum IgG antibodies against

GM1 and GM1b, an increase in CSF protein, and rapid clinical and

electrophysiological recovery after plasma exchange support the

diagnosis of a pure motor axonal form of GBS, acute motor axonal

neuropathy. The association may be coincidental, but a particular

susceptibility to axonal damage of CMT2 cannot be excluded.