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Published on: November 9, 2017
Acute Bulbar Palsy-Plus Variant of Guillain-Barré Syndrome in a 3-Year-Old Girl
Saihari S Dukkipati1, Daniel J Zhou1, Andria M Powers2
1Department of Neurological Sciences, University of Nebraska Medical Center, Omaha, NE, USA.
Insights
A child developed facial palsy, dysphagia, and ataxia after an influenza vaccine, diagnosed as a rare Guillain-Barré syndrome (GBS) variant. Prompt treatment with intravenous immunoglobulin led to a full recovery.
Area of Science:
- Neurology
- Immunology
- Pediatrics
Background:
- Guillain-Barré syndrome (GBS) is an autoimmune disorder affecting the peripheral nervous system.
- The acute bulbar palsy plus (ABPp) variant of GBS is rare, particularly in young children.
- Vaccine-associated neurological complications, though uncommon, warrant careful investigation.
Observation:
- A 3-year-old girl presented with rapid onset of bilateral facial palsy, dysphagia, dysphonia, areflexia, and ataxia post-influenza vaccination.
- Neuroimaging revealed enhancement of cranial nerves and spinal roots.
- Cerebrospinal fluid analysis showed elevated protein and white blood cell count, with negative infectious and autoimmune markers.
Findings:
- The patient's clinical presentation and diagnostic workup were most consistent with the ABPp variant of GBS.
- Negative serological testing for common anti-ganglioside antibodies.
- Exclusion of infectious and other autoimmune etiologies.
Implications:
- This case highlights the importance of considering rare GBS variants in pediatric patients with post-vaccination neurological symptoms.
- Early diagnosis and prompt treatment with intravenous immunoglobulin can lead to favorable outcomes.
- Further research into vaccine-associated GBS variants is needed to understand pathogenesis and optimize management.
Abstract:
We present a case of a 3-year-old girl who rapidly developed bilateral facial palsy, dysphagia, dysphonia, areflexia, and ataxia soon after receiving an influenza vaccine. Brain and spine Magnetic resonance imaging (MRI) scans with and without contrast showed enhancement of cranial nerves III, V, VII, and X, as well as the anterior and posterior cervical spinal and cauda equina roots. cerebrospinal fluid (CSF) studies showed white blood cell count of 19 cells/cm2, glucose 81 mg/dL, and protein 116 mg/dL, with negative infectious and autoimmune labs. Serum IgM and IgG antibodies against GM1, GD1a, GD1b, GM2, GT1A, GQ1b were negative. The patient was treated with intravenous immunoglobulin, which led to a full recovery. Upon three-month follow-up, her neurologic examination demonstrated normal cranial nerves, reflexes, and gait. Her presentation was most consistent with the acute bulbar palsy plus (ABPp) variant of Guillain-Barré syndrome (GBS), a rare and challenging diagnosis especially in her age group.
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