Korean J Pediatr.  2010 Apr;53(4):607-611. 10.3345/kjp.2010.53.4.607.

A case of Bickerstaff's brainstem encephalitis in childhood

• Kim JY
• Kim YO
• Son YJ
• Woo YJ

• KMID: 2073591
• DOI: http://doi.org/10.3345/kjp.2010.53.4.607

Abstract

Bickerstaff's brainstem encephalitis (BBE) is a rare disease diagnosed by specific clinical features such as 'progressive, relatively symmetric external ophthalmoplegia and ataxia by 4 weeks' and 'disturbance of consciousness or hyperreflexia' after the exclusion of other diseases involving the brain stem. Anti-ganglioside antibodies (GM, GD and GQ) in the serum or cerebrospinal fluid (CSF) are sometimes informative for the diagnosis of BBE because of the rarity of positive findings in other diagnositic
METHODS
brain magnetic resonance imaging (MRI), routine CSF examination, motor nerve conduction study, and needle electromyography. We report a rare case of childhood BBE with elevated anti-GM1 antibodies in the serum, who had specific clinical symptoms such as a cranial polyneuropathy presenting as ophthalmoplegia, dysarthria, dysphagia, and facial weakness; progressive motor weakness; altered mental status; and ataxia. However, the brain MRI, routine CSF examination, nerve conduction studies, electromyography, somatosensory evoked potentials, and brainstem auditory evoked potentials were normal. BBE was suspected and the patient was successfully treated with intravenous immunoglobulins.

Keyword

Encephalitis; Brain stem; Child

MeSH Terms

Antibodies
Ataxia
Brain
Brain Stem
Child
Consciousness
Deglutition Disorders
Dysarthria
Electromyography
Encephalitis
Evoked Potentials, Auditory, Brain Stem
Evoked Potentials, Somatosensory
Humans
Immunoglobulins, Intravenous
Magnetic Resonance Imaging
Needles
Neural Conduction
Ophthalmoplegia
Polyneuropathies
Rare Diseases
Antibodies
Immunoglobulins, Intravenous