Monograph: Muscle Stiffness due to Neuromuscular Hyperexcitability-Article

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This monograph reviews pediatric Guillain–Barré syndrome (GBS), emphasizing how it differs from adult disease in presentation, diagnosis, management, and follow-up.<br /><br />GBS is an acute autoimmune polyradiculoneuropathy and a leading cause of acute flaccid weakness in children. Its incidence is slightly lower in children than adults, but it remains a key diagnostic consideration. In pediatrics, the earliest and most prominent symptoms are often pain, irritability, gait refusal, or refusal to bear weight rather than obvious weakness, which can delay diagnosis. Examining young children is often difficult, and sedation may be needed for lumbar puncture or electrodiagnostic testing.<br /><br />Diagnosis generally follows adult criteria, including Brighton criteria, but pediatric practice often relies more heavily on spinal MRI with gadolinium because it can support GBS diagnosis and help exclude mimics such as acute flaccid myelitis, myelopathy, CNS demyelination, or non-neurologic causes. CSF and electrodiagnostic studies remain useful, though early results may be normal or technically limited. Variant forms such as Miller–Fisher syndrome, Bickerstaff brainstem encephalitis, and pharyngeal–cervical–brachial variants are important in children.<br /><br />Supportive care is essential and includes respiratory monitoring, dysautonomia management, pain control, nutrition/swallow safety, rehabilitation, and selective consideration of venous thromboembolism prophylaxis. IVIG is the main disease-modifying therapy in children. Plasma exchange is effective but used less often because of access and logistical challenges. Corticosteroids are not recommended, and evidence for newer immunotherapies in children is lacking.<br /><br />Most children recover very well, with low mortality and good long-term function, though severe initial weakness and axonal injury predict slower recovery. A major follow-up issue is distinguishing monophasic GBS from acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), which can initially mimic GBS.

Keywords

pediatric Guillain-Barré syndrome

acute flaccid weakness

diagnosis

Brighton criteria

spinal MRI

cerebrospinal fluid

electrodiagnostic studies

intravenous immunoglobulin

plasma exchange

CIDP

Chronic Inflammatory Demyelinating Polyneuropathy