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Published on: November 3, 2016
[Guillain-Barré syndrome in a child with normal tendon reflexes]
S Tellier1, A Gerdelat-Mas, C Karsenty
1Service de neurologie pédiatrique, hôpital des enfants, CHU de Toulouse, 330, avenue Grande-Bretagne, 31059 Toulouse cedex 9, France.
Insights
This case study highlights acute motor axonal neuropathy (AMAN) Guillain-Barré syndrome (GBS) in a child with normal reflexes. Early diagnosis and treatment with IVIg led to a favorable outcome, proving GBS can occur with preserved reflexes.
Area of Science:
- Neurology
- Immunology
Background:
- Guillain-Barré syndrome (GBS) is an autoimmune disorder affecting the peripheral nervous system.
- The acute motor axonal neuropathy (AMAN) subtype is characterized by motor axonal degeneration.
- Typically, GBS presents with diminished or absent tendon reflexes.
Observation:
- A 10-year-old child developed distal limb weakness 6 days post-gastroenteritis.
- The child presented with preserved tendon reflexes, elevated CSF protein, and no cells.
- Nerve conduction studies confirmed motor axonal degeneration consistent with GBS.
Findings:
- The patient's serum tested positive for IgG antibodies against gangliosides GM1 and GD1b.
- This case demonstrates GBS, specifically the AMAN form, can occur in children with normal tendon reflexes.
- Unlike previously reported adult cases, this pediatric case expands the understanding of AMAN presentations.
Implications:
- This case challenges the traditional diagnostic criteria for GBS, emphasizing that preserved reflexes do not exclude the diagnosis.
- It underscores the importance of considering AMAN in pediatric patients with unexplained limb weakness and normal reflexes.
- Early identification and treatment with intravenous immunoglobulins (IVIg) can lead to positive clinical outcomes in pediatric GBS.
Abstract:
We describe the case of a 10-year-old child with the acute motor axonal neuropathy (AMAN) form of Guillain-Barré syndrome (GBS) with preserved tendon reflexes, 6 days after a bout of gastroenteritis. The child quickly showed weakness of the distal muscles of his four limbs, with preserved tendon reflexes and a raised CSF protein concentration with no cells. Nerve conduction studies showing motor axonal degeneration confirmed the diagnosis of GBS in spite of preserved tendon reflexes. The serum was positive for IgG antibodies to gangliosides GM1 and GD1b. The child received intravenous immunoglobulins, which resulted in a favorable progression. This case proves that GBS with normal tendon reflexes exists. The other cases of SGB with preserved tendon reflexes already described in the literature were the AMANs form with antibodies to gangliosides in the serum and only adults were affected.
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