Severe Locked-In-Like Guillain-Barré's Syndrome: Dilemmas in Diagnosis and Treatment

Area of Science:

• Pediatric Neurology
• Neuroimmunology
• Clinical Case Studies

Background:

• Childhood Guillain-Barré syndrome (GBS) presents diagnostic and therapeutic challenges due to atypical disease courses.
• Early and intensive immunomodulatory treatment is standard for severe GBS.
• Protracted and severe GBS can mimic other neurological conditions, complicating management.

Observation:

• A 13-year-old boy developed acute GBS, progressing over 40 days to a severe locked-in-like syndrome despite initial immunomodulatory therapy.
• The patient experienced a 3-month plateau phase with fluctuating inflammatory activity.
• Atypical features at onset and profound neurological deficits were noted.

Findings:

• Repeated and maintenance immunomodulatory treatment was initiated due to persistent inflammatory signs.
• This delayed treatment strategy resulted in continuous and near-complete functional recovery.
• The case demonstrates a late but substantial response to prolonged immunomodulatory intervention.

Implications:

• This case underscores the need to consider atypical presentations and protracted courses in childhood GBS.
• It suggests that prolonged or repeated immunomodulatory treatment may be beneficial in severe, refractory GBS cases.
• Revisiting diagnostic and therapeutic dilemmas in severe, atypical GBS is crucial for optimizing patient outcomes.