gq1b-ganglioside and Coma

A 25-years-old man experienced fever and diarrhea. Ten days later he noticed difficulty walking (day 1). On admission neurological examination revealed lethargy, dysarthria and weakness of limbs. Oculocephalic response was not be elicited and extensor toe signs were positive. In spite of treatment with aciclovir and methylprednisolone, he continued to show progressive deterioration developing to coma with decorticate posture. Autonomic symptoms (hyperhidrosis, hypersalivation and fever) and groaning were observed. Brain magnetic resonance image and brainstem evoked potential presented no abnormality, but electroencephalographic study showed a spindle pattern indicating spindle coma. Laboratory tests including cerebrospinal fluids showed no specific results. High-dose immunoglobulin was administered from day 6, and his consciousness level improved. External ophthalomoplegia and ataxic gait were observed after he became more alert. Because he had IgG type anti-GQ1b antibodies in the serum, a diagnosis was made of Bickerstaff's brainstem encephalitis (BBE). Six months after discharge he had complete resolution of his symptoms. This is the first report of spindle coma observed in a case of serologically confirmed BBE.

Topics: Adult; Autoantibodies; Biomarkers; Brain Stem; Coma; Decerebrate State; Drug Therapy, Combination; Electroencephalography; Encephalitis; Gangliosides; Humans; Immunoglobulin G; Immunoglobulins, Intravenous; Male; Methylprednisolone; Treatment Outcome

Bickerstaff brainstem encephalitis is characterized by the occurrence of ataxia, ophthalmoplegia, motor weakness with areflexia and central nervous system symptoms, with drowsiness, pyramidal syndrome and sensorial symptoms. Diagnosis is based on MR findings and GQ1b antibodies. Treatment is not well known.. We report a patient aged 39 years native of Laos who presented weakness, loss of reflexes, and drowsiness. Brain MR showed hyperintense signals in the brain stem. GQ1b antibodies were positive. The course was characterized by decrease of the weakness, normalization of MR and negativity of GQ1b antibodies.. This observation underlines common features of Bickerstaff brainstem encephalitis, Miller Fisher syndrome and Guillain Barre syndrome. A favorable course and GQ1b antibodies are shared by these syndromes.

Topics: Adrenal Cortex Hormones; Autoantibodies; Autoantigens; Blindness; Brain Stem; Coma; Combined Modality Therapy; Demyelinating Autoimmune Diseases, CNS; Electroencephalography; Encephalitis; Female; Gangliosides; Humans; Magnetic Resonance Imaging; Middle Aged; Optic Atrophy; Plasmapheresis; Quadriplegia; Reflex, Abnormal; Syndrome

A patient with Bickerstaff's brain stem encephalitis (BBE) associated with anti-GQ1b antibody developed coma, severe weakness, and respiratory distress. The patient required ventilatory support. After having failed to improve on steroids, she was treated with plasmapheresis. She improved concomitantly with the plasmapheresis treatment and made a complete recovery. BBE associated with anti-GQ1b antibody is generally considered to be benign, and specific treatments have not been established. The results with this patient suggest that the condition is not always benign, and plasmapheresis may be beneficial in this disorder.

Topics: Adult; Antibodies; Brain Stem; Coma; Electroencephalography; Encephalitis; Evoked Potentials, Somatosensory; Female; Gangliosides; Glucocorticoids; Humans; Immunoglobulin M; Infusions, Intravenous; Methylprednisolone; Muscle Weakness; Nerve Growth Factors; Plasmapheresis; Respiration, Artificial; Respiratory Insufficiency