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Published on: January 9, 2018
Steroid-responsive chronic cerebellitis with positive glutamate receptor delta 2 antibody
Masaya Kubota1, Yukitoshi Takahashi
1Department of Pediatrics (Pediatric Neurology), Metropolitan Hachioji Children's Hospital, Hachioji-city, Tokyo, Japan. mkmegped@opal .plala.or.jp
Insights
A 4-year-old girl with chronic cerebellitis, linked to anti-glutamate receptor delta 2 antibodies after vaccination, showed significant improvement with steroid therapy. This case highlights the role of these antibodies in immune-mediated cerebellar dysfunction.
Area of Science:
- Neuroimmunology
- Pediatric Neurology
Background:
- Chronic cerebellitis is a rare neurological disorder affecting cerebellar function.
- Anti-glutamate receptor delta 2 antibodies are implicated in cerebellar disorders, particularly in the developing brain.
Observation:
- A 4-year-old girl developed chronic cerebellitis following diphtheria-pertussis-tetanus vaccination.
- The patient presented with symptoms associated with anti-glutamate receptor delta 2 antibodies.
Findings:
- The patient experienced a dramatic clinical improvement following methylprednisolone pulse therapy and oral prednisolone.
- The findings suggest that anti-glutamate receptor delta 2 antibodies play a key role in an immune-mediated process affecting the parallel fiber-Purkinje cell synapse.
Implications:
- This case underscores the potential of steroid therapy for antibody-mediated chronic cerebellitis.
- Effective treatment is crucial for optimizing motor development and language acquisition in affected children, while managing corticosteroid side effects.
Abstract:
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.
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