ganglioside--gd1a and Encephalitis

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.

Topics: Adult; Ataxia; Autoantibodies; Brain Stem; Diagnosis, Differential; Electroencephalography; Encephalitis; G(M1) Ganglioside; Gangliosides; Glasgow Coma Scale; Humans; Male; Ophthalmoplegia

Clinical and immunological evaluation of 'incomplete' Bickerstaff brainstem encephalitis (BBE).. We studied two patients with postinfectious brainstem syndromes who presented at National University Hospital Singapore. Laboratory work-up included measurement of antiganglioside antibodies.. Both patients displayed hypersomnolence and cerebellar-like ataxia in the absence of external ophthalmoplegia and carried high serum titres of IgG anti-GQ1b antibodies, strongly indicative of BBE.. Ophthalmoplegia can be absent or incomplete in BBE, and the absence of this clinical feature should not exclude BBE from the clinicians' differential. Such cases of incomplete BBE could be defined as 'ataxic hypersomnolence without ophthalmoplegia'.

Topics: Adult; Autoantibodies; Brain Stem; Cerebellar Ataxia; Disorders of Excessive Somnolence; Encephalitis; Female; Follow-Up Studies; G(M1) Ganglioside; Gangliosides; Humans; Immunization, Passive; Middle Aged; Neurologic Examination; Ophthalmoplegia