Brain & development · 2026
Pediatric GBS-myelitis overlap syndrome: Severe phenotype, treatment response, and neurological outcomes.
СтатьяНазвание и аннотация: Перевод в очереди
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Ye Fubiao, Zhang Yiyan, Li Yukun, Kang Jing, Chen Jiawei, Fu Shijie, Fang Qiong
Аннотация
PURPOSE: To characterize the clinical features, treatment responses, and outcomes of pediatric patients with Guillain-Barré syndrome (GBS) and concurrent myelitis.
METHODS: Clinical and auxiliary data from 22 pediatric patients (5 from local hospital and 17 by reviewing the previous literatures) with GBS and myelitis were analyzed. Parameters included demographics, neuroimaging, cerebrospinal fluid (CSF) findings, immunotherapy regimens, and outcomes stratified by the Hughes Functional Grading Scale.
RESULTS: In this pediatric cohort (median age: 11.0 years; male-to-female ratio ≈1:1), GBS with concurrent myelitis manifested with flaccid paralysis in 95.5% of cases, including quadriplegia (59.1%), accompanied by diminished deep tendon reflexes (90.9%). Sensory disturbances were documented in 77.3% of patients, while autonomic dysfunction featured urinary impairment (68.2%) and respiratory failure requiring ventilation (52.6%). Neurodiagnostic findings universally demonstrated long-segment spinal cord lesions (>5 vertebral segments) on MRI and albuminocytological dissociation in CSF analysis. First-line immunotherapy comprised intravenous immunoglobulin (IVIG) and corticosteroids. Severity stratification identified a severe group (Hughes Grade ≥ 3; n = 13) characterized by: tetraplegia (92.3%), respiratory failure (69.2%), extensive spinal cord edema (84.6%), prolonged hospitalization (median: 6.0 weeks), higher residual sequelae (69.2%). The majority of patients in this group (92.3%) necessitated combination therapy with IVIG and corticosteroids.
CONCLUSIONS: Pediatric GBS with myelitis frequently presents with severe neurological involvement. Although combination immunotherapy demonstrates efficacy, the severe subgroup shows significant residual morbidity. Early aggressive intervention is warranted to mitigate long-term disability.
METHODS: Clinical and auxiliary data from 22 pediatric patients (5 from local hospital and 17 by reviewing the previous literatures) with GBS and myelitis were analyzed. Parameters included demographics, neuroimaging, cerebrospinal fluid (CSF) findings, immunotherapy regimens, and outcomes stratified by the Hughes Functional Grading Scale.
RESULTS: In this pediatric cohort (median age: 11.0 years; male-to-female ratio ≈1:1), GBS with concurrent myelitis manifested with flaccid paralysis in 95.5% of cases, including quadriplegia (59.1%), accompanied by diminished deep tendon reflexes (90.9%). Sensory disturbances were documented in 77.3% of patients, while autonomic dysfunction featured urinary impairment (68.2%) and respiratory failure requiring ventilation (52.6%). Neurodiagnostic findings universally demonstrated long-segment spinal cord lesions (>5 vertebral segments) on MRI and albuminocytological dissociation in CSF analysis. First-line immunotherapy comprised intravenous immunoglobulin (IVIG) and corticosteroids. Severity stratification identified a severe group (Hughes Grade ≥ 3; n = 13) characterized by: tetraplegia (92.3%), respiratory failure (69.2%), extensive spinal cord edema (84.6%), prolonged hospitalization (median: 6.0 weeks), higher residual sequelae (69.2%). The majority of patients in this group (92.3%) necessitated combination therapy with IVIG and corticosteroids.
CONCLUSIONS: Pediatric GBS with myelitis frequently presents with severe neurological involvement. Although combination immunotherapy demonstrates efficacy, the severe subgroup shows significant residual morbidity. Early aggressive intervention is warranted to mitigate long-term disability.