RESUMO
The presence of inexcitable motor nerves early in the course of Guillain-Barré syndrome (GBS) identifies a subgroup of patients with more severe disease and delayed recovery. How frequently these electrodiagnostic findings reflect a primary axonal attack ("axonal" GBS) is controversial. We present two children with severe acute GBS, delayed recovery, and residual disability despite early treatment with human immunoglobulin. They had inexcitable motor nerves at days 6 and 7, and profuse fibrillations and positive waves on subsequent studies. Clinically and electrodiagnostically, both children's disease resembled the acute motor-sensory axonal variant of GBS (AMSAN). Sensory and motor nerve biopsies revealed severe macrophage-associated demyelination with axonal degeneration of variable severity. We conclude that clinical and electrodiagnostic features cannot discriminate between the "axonal" and demyelinating GBS. Early and severe demyelination with secondary axonal damage may mimic clinically and electrophysiologically the AMSAN variant of GBS.
Assuntos
Neurônios Motores/patologia , Nervo Fibular/patologia , Polirradiculoneuropatia/patologia , Nervo Sural/patologia , Biópsia , Criança , Eletromiografia , Feminino , Humanos , Imuno-Histoquímica , Masculino , Microscopia EletrônicaRESUMO
Thirteen children with severe Guillain-Barré syndrome were treated with human immune globulin. Patients received a mean total dose of 1.9 gm/kg of human immune globulin for 2 or 5 days. To evaluate the relationship between the response to human immune globulin and electrodiagnostic findings, we compared the clinical outcome of 3 groups of children. The first group consisted of 9 children with electrophysiologic evidence of a mean amplitude of the compound motor action potentials larger than 10% of the lower limit of normal. The second group of 4 children had inexcitable motor nerves. Children in the second group required longer periods to improve one functional grade (mean 67.3 days vs 18.8 days) and to reach grade 2 (219 days vs 32.7 days). Moreover, children in the second group were more disabled after 3 and 6 months, and they all remained with distal atrophy and weakness after 7 months of follow-up. Furthermore, the outcome of children in the second group was no different from that of a historic control of 5 untreated children with severe Guillain-Barré syndrome and similar electrophysiologic findings. Human immune globulin treatment in children with severe Guillain-Barré syndrome is safe, easy to administer, and does not increase the number of relapses. Nevertheless, it does not seem to benefit children with low mean compound motor action potential amplitude.