Abstract
Opsoclonus myoclonus syndrome is a rare paraneoplastic syndrome seen in 50% of children with neuroblastoma. Neural generator of opsoclonus and myoclonus is not known but evidences suggest the role of fastigial nucleus disinhibition from the loss of function of inhibitory (GABAergic) Purkinje cells in the cerebellum. We present a child with paraneoplastic opsoclonus myoclonus syndrome who responded well to clonazepam. Response to clonazepam is an evidence for the involvement of GABAergic neural circuits in the genesis of opsoclonus myoclonus syndrome and is in agreement with fastigial nucleus disinhibition hypothesis.
MeSH terms
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Autoantibodies / metabolism
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Cerebellar Cortex / immunology
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Cerebellar Cortex / metabolism
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Cerebellar Cortex / physiopathology
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Cerebellar Nuclei / drug effects*
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Cerebellar Nuclei / metabolism
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Cerebellar Nuclei / physiopathology*
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Clonazepam / administration & dosage*
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Efferent Pathways / immunology
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Efferent Pathways / metabolism
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Efferent Pathways / physiopathology
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GABA Modulators / administration & dosage
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Humans
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Infant
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Male
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Neural Inhibition / drug effects*
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Neural Inhibition / physiology
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Neuroblastoma / complications
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Neuroblastoma / immunology
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Neuroblastoma / surgery
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Neurosurgical Procedures
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Opsoclonus-Myoclonus Syndrome / drug therapy*
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Opsoclonus-Myoclonus Syndrome / metabolism
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Opsoclonus-Myoclonus Syndrome / physiopathology*
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Pelvic Neoplasms / complications
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Pelvic Neoplasms / immunology
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Pelvic Neoplasms / surgery
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Purkinje Cells / immunology
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Purkinje Cells / metabolism
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Purkinje Cells / pathology
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Treatment Outcome
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gamma-Aminobutyric Acid / metabolism
Substances
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Autoantibodies
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GABA Modulators
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gamma-Aminobutyric Acid
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Clonazepam