NERVE BIOPSY IN CHILDREN WITH SEVERE GUILLAIN-BARRE-SYNDROME AND INEXCITABLE MOTOR NERVES

The presence of inexcitable motor nerves early in the course of Guilla in-Barre syndrome (GBS) identifies a subgroup of patients with more se vere disease and delayed recovery. How frequently these electrodiagnos tic findings reflect a primary axonal attack (''axonal'' GBS) is contr oversial. We present two children with severe acute GBS, delayed recov ery, and residual disability despite early treatment with human immuno globulin. They had inexcitable motor nerves at clays 6 and 7, and prof use fibrillations and positive waves on subsequent studies. Clinically and electrodiagnostically, both children's disease resembled the acut e motor-sensory axonal variant of GBS (AMSAN). Sensory and motor nerve biopsies revealed severe macrophage-associated demyelination with axo nal degeneration of variable severity. We conclude that clinical and e lectrodiagnostic features cannot discriminate between the ''axonal'' a nd demyelinating GBS. Early and severe demyelination with secondary ax onal damage may mimic clinically and electrophysiologically the AMSAN variant of GBS.