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Weakness in both lower limbs for 1 week and blepharoptosis for 3 days in a boy aged 1 year and 7 months
Chang-Hui Lang1, Mao-Qiang Tian1, Xiao-Mei Shu1
1Department of Pediatrics, Children's Hospital of Guizhou Province/Affiliated Hospital of Zunyi Medical University, Zunyi, Guizhou 563003, China.
Insights
A rare pediatric case of Guillain-Barré syndrome (GBS) overlapping with Miller-Fisher syndrome and Bickerstaff brainstem encephalitis presented with limb weakness and ptosis. Early H reflex testing is crucial for diagnosing these complex GBS overlap syndromes in children.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Clinical Neuroscience
Background:
- Guillain-Barré syndrome (GBS) and its overlap syndromes present significant diagnostic challenges in children.
- Anti-GD1b antibody-related GBS overlap syndromes exhibit unique clinical and neurophysiological features.
- Clinical heterogeneity in pediatric GBS overlaps stems from combined peripheral nerve and brainstem involvement.
Purpose of the Study:
- To report a pediatric case of GBS overlapping with Miller-Fisher syndrome and Bickerstaff brainstem encephalitis.
- To highlight the diagnostic difficulties and clinical manifestations of anti-GD1b antibody-related GBS overlap syndromes.
- To emphasize the importance of specific neurophysiological tests in diagnosing pediatric GBS variants.
Main Methods:
- Case presentation of a 1-year-7-month-old boy with progressive neurological deficits.
- Neurological examination including muscle strength assessment and reflex evaluation.
- Cerebrospinal fluid analysis, H reflex testing, and serum anti-GD1b IgG antibody testing.
Main Results:
- The patient exhibited lethargy, blepharoptosis, limb weakness (upper limbs grade 4, lower limbs grade 3), and absent reflexes.
- Laboratory findings included albuminocytological dissociation, absent H reflex, and positive anti-GD1b IgG.
- Diagnosis of Guillain-Barré syndrome overlapping with Miller-Fisher syndrome and Bickerstaff brainstem encephalitis was confirmed.
Conclusions:
- Anti-GD1b antibody-related GBS overlap syndromes in children are clinically heterogeneous and neurophysiologically complex.
- Nerve conduction velocity tests, particularly the H reflex, are essential for diagnosing pediatric GBS variants with limb weakness and ptosis.
- Prompt diagnosis and treatment, including immunoglobulin and plasma exchange, led to the patient's recovery.
Abstract:
A boy, aged 1 year and 7 months, was hospitalized due to weakness in both lower limbs and blepharoptosis, which showed progressive aggravation and developed into irregular breathing. Neurological examinations showed lethargy, blepharoptosis, grade 4 muscle strength of both upper limbs, grade 3 muscle strength of both lower limbs, and disappearance of tendon reflex. Laboratory tests revealed albuminocytological dissociation in cerebrospinal fluid, disappearance of H reflex, and positive serum anti-GD1b IgG. The boy was finally diagnosed with Guillain-Barré syndrome (GBS) overlapping with Miller-Fisher syndrome and Bickerstaff brainstem encephalitis. He recovered and was discharged after treatment including immunoglobulin, plasma exchange, and respiratory support. The GBS overlap syndromes in children have strong clinical heterogeneity due to the injury of both peripheral nerve and brainstem, among which anti-GD1b antibody-related GBS overlap syndromes have special clinical manifestations and complex neuroelectrophysiological changes and are thus difficult to diagnose. Nerve conduction velocity tests, especially H reflex test, should be performed for children with weakness in both lower limbs and blepharoptosis.
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