Acute Bulbar Palsy-Plus Variant of Guillain-Barré Syndrome in a 3-Year-Old Girl

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.

Keywords: areflexia; cranial neuropathy; inflammatory neuropathy; influenza vaccine; stridor; vocal cord paralysis.

Figure 1.

Axial heavily fluid weighted T2 (A), postcontrast FLAIR (B), precontrast T1 (C) and postcontrast T1 (D) weighted MR images at the pontomesencephalic junction reveal symmetric, smooth enhancement of the oculomotor nerves. Axial heavily fluid weighted T2 (E), postcontrast FLAIR (F), precontrast T1 (G) and postcontrast T1 (H) weighted MR images at the lower pons reveal symmetric, smooth enhancement of the facial nerves involving the meatal and labyrinthine segments.

Figure 2.

Axial precontrast (A) and postcontrast (B) T1-weighted MR images of the spine at the C5-6 level show symmetric, bilateral, smooth anterior and posterior nerve root enhancement. Axial precontrast (C) and postcontrast (D) T1-weighted MR images of the cauda equina reveal smooth nerve root enhancement without nodularity. Coronal T1 postcontrast reconstruction (E) of the cauda equina reveals diffuse nerve root enhancement in long axis.