Motor nerve inexcitability in Guillain-Barré syndrome. The spectrum of distal conduction block and axonal degeneration.
We studied 34 patients with the Guillain-Barré syndrome (GBS) to clarify the clinical significance of inexcitable motor nerves and of low amplitude compound muscle action potentials (CMAPs). The patients were subdivided into two groups. Group 1 included eight patients who had electrically inexcitable motor nerves within 2 wks of the first symptom. (Two patients without extensive conduction studies had only one inexcitable motor nerve.) The outcome in this group at 1 yr varied from complete recovery (five patients) to severe motor sequelae (three patients). Group 2 included 26 patients who had two electrophysiological assessments, and in whom the serial changes in CMAP amplitudes were analysed and correlated to outcome. Fourteen of these 26 sets of serial studies were performed within 1 mth. Twelve of 26 patients in Group 2 showed decrease in the amplitude of CMAPs between serial studies; only six of these had a good outcome at 1 yr. Nine of 26 patients showed increase in CMAP amplitude between serial studies, of these eight had a good clinical outcome. Low-amplitude CMAPs or inexcitable motor nerves in the initial stages of GBS are due to distal pathology of the motor axons, either distal conduction block or axonal degeneration. The nature of these changes cannot be predicted by the results of the initial electrophysiological evaluation, including the presence or absence of active denervation. However, improvement of CMAP amplitude on sequential studies suggests a good outcome at 1 yr. We believe that, in the absence of a biological marker for GBS, individualization of an 'axonal variant' of the syndrome is not warranted at the present time.
Triggs
W J
WJ
Department of Neurology, Massachusetts General Hospital, Boston 02114.
Cros
D
D
Gominak
S C
SC
Zuniga
G
G
Beric
A
A
Shahani
B T
BT
Ropper
A H
AH
Roongta
S M
SM
D000200:Action Potentials; D000293:Adolescent; D000328:Adult; D000368:Aged; D001369:Axons; D002648:Child; D004576:Electromyography; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons; D009132:Muscles / Q000294:innervation; D009431:Neural Conduction; D010525:Peripheral Nerves / Q000503:physiopathology*; D011129:Polyradiculoneuropathy / Q000503:physiopathology*