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Journal of Child Neurology, Vol. 23, No. 2, 228-230 (2008)
DOI: 10.1177/0883073807307973

Steroid-Responsive Chronic Cerebellitis With Positive Glutamate Receptor {delta}2 Antibody

Masaya Kubota, MD, PhD

Department of Pediatrics (Pediatric Neurology), Metropolitan Hachioji Children's Hospital, Hachioji-city, Tokyo, Japan, mkmegped@opal .plala.or.jp

Yukitoshi Takahashi, MD, PhD

Department of Pediatrics (Pediatric Neurology), Metropolitan Hachioji Children's Hospital, Hachioji-city, Tokyo, Japan

We report the clinical course of a 4-year-old girl with chronic cerebellitis (onset 2 days after diphtheria-pertussis-tetanus vaccination at 1 year and 7 months old) associated with anti-glutamate receptor {delta}2 antibody, who improved dramatically with steroid therapy (methylprednisolone pulse therapy plus oral prednisolone). Recently, it has been reported that the anti-glutamate receptor {delta}2 selectively expressed at the post-synaptic site of parallel fiber—Purkinje cell synapses has an important role in cerebellar function in the developing brain. The present case suggests that anti-glutamate receptor {delta}2 antibody plays a primary role in an immune-mediated process causing chronic cerebellar symptoms, and the lesion site seems to be localized to the parallel fiber—Purkinje cell synapse. Because the cerebellum is strongly involved in language acquisition as well as motor development, treatment must facilitate time for language learning while reducing the side effects of the corticosteroid therapy.

Key Words: chronic cerebellitis • glutamate receptor {delta}2


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