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[Guillain-Barré syndrome in infancy: The importance of electroneuromyography]
M Vedrenne-Cloquet1, K Maincent2, T Billette de Villemeur3
1Service de neuropédiatrie, hôpital Armand-Trousseau, Assistance publique-Hôpitaux de Paris, 26, avenue Arnold-Netter, 75012 Paris, France.
Insights
Guillain-Barré Syndrome (GBS) is rare in infants, but diagnosis is possible with specific tests. Early treatment with intravenous immunoglobulins led to full recovery in an 8-month-old with atypical GBS.
Area of Science:
- Neurology
- Pediatrics
- Immunology
Background:
- Guillain-Barré Syndrome (GBS) is uncommon in infants, presenting diagnostic challenges.
- Differential diagnoses include congenital neuropathies, requiring careful evaluation.
Observation:
- An 8-month-old infant presented with acquired hypotonia and descending paralysis, notably in upper limbs.
- Severe, unexplained neutropenia accompanied the neurological symptoms.
Findings:
- Diagnosis of GBS was confirmed by albuminocytologic dissociation in cerebrospinal fluid and demyelinating sensomotor polyradiculoneuropathy on electroneuromyography.
- A single cycle of intravenous immunoglobulin therapy resulted in complete recovery after one year.
Implications:
- Highlights the existence of atypical GBS presentations in infants.
- Emphasizes the importance of prompt diagnosis, differentiation from congenital neuropathies, and timely treatment.
- Stresses the need for ongoing medical follow-up to detect potential relapses indicative of underlying genetic or chronic inflammatory conditions.
Abstract:
Guillain-Barré Syndrome (GBS) is rare in infancy, and the diagnosis of atypical forms is difficult in this age range. The main differential diagnoses include congenital neuropathy. Biological and electrophysiological investigations remain important to confirm diagnosis and start treatment quickly. We report the case of an 8-month-old boy who presented with acquired hypotonia due to progressive descending limb paralysis, predominant in the upper limbs, associated with unexplained severe neutropenia. GBS was diagnosed thanks to the association of albuminocytologic dissociation on cerebrospinal fluid and demyelinating sensomotor polyradiculoneuropathy on electroneuromyography. Only one cycle of treatment with intravenous immunoglobulins was sufficient to achieve complete recovery after 1 year. Physicians should know that atypical forms of GBS exist in infants, in order to recognize the syndrome, rule out differential diagnoses, and start treatment as soon as possible. Medical follow-up remains important before and after remission, especially in infants, to identify relapses, which might be the symptom of a genetic neuropathy or a chronic inflammatory disease.
