Bickerstaff's brainstem encephalitis associated with anti-GM1 and anti-GD1a antibodies

Abstract

Bickerstaff's brainstem encephalitis (BBE) is a Guillain-Barré syndrome (GBS) spectrum disorder associated with predominantly central nervous system predilection. Patients exhibit a variable constellation of depressed consciousness, bilateral external ophthalmoplegia, ataxia and long tract signs. Although the pathophysiology is not fully understood, it has been associated with anti-GQ1b antibodies in two-thirds of patients. We present a patient with clinical features consistent with BBE and positive anti-GM1 and anti-GD1a antibodies. A diagnostic approach to the acutely unwell patient with brainstem encephalitis is explored in this clinical context with a literature review of the aforementioned ganglioside antibody significance. Intravenous immunoglobulin therapy is highlighted in BBE using up-to-date evidence-based extrapolation from GBS.

Keywords: brain stem / cerebellum; cranial nerves; neuro ITU; neurology.

Figure 1

Bickerstaff’s encephalitis. (A) Axial fluid attenuated inversion recovery (FLAIR) MRI of the brain. (B) Axial T2 MRI of the brain. (C, D) Diffusion-weighted imaging (DWI) B1000 and apparent diffusion coefficient (ADC) map, respectively, obtained at the level of the mid-pons. No abnormal brainstem or cerebellar signal and no abnormal restricted diffusion. Normal MRI of the brain appearances.

Figure 2

Approach to the differential diagnosis of brainstem encephalitis. *Cocaine can erode the surrounding skull base and sinuses creating a pathway for all infections from the nasal cavity to the brainstem. ADEM, acute disseminated encephalomyelitis; BBE, Bickerstaff’s brainstem encephalitis; CMV, cytomegalovirus; CNS, central nervous system; EBV, Epstein-Barr virus; EV-71, enterovirus-71; Gly, glycine; HSV, herpses simplex virus; IgLon5, immunoglobulin-like cell adhesion molecule 5; NBS, neurobehçet’s syndrome; NMDA, N-methyl-D-aspartate; NMOSD, neuromyelitis optica spectrum disorder; SLE, systemic lupus erythematosus; TB, tuberculosis.