Clonazepam responsive opsoclonus myoclonus syndrome: additional evidence in favour of fastigial nucleus disinhibition hypothesis?
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.
Paliwal
Vimal Kumar
VK
Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India. dr_vimalkpaliwal@rediffmail.com
Chandra
Satish
S
Verma
Ritu
R
Kalita
Jayantee
J
Misra
Usha K
UK
D001323:Autoantibodies / Q000378:metabolism; D002525:Cerebellar Cortex / Q000276:immunology / Q000378:metabolism / Q000503:physiopathology; D002529:Cerebellar Nuclei / Q000187:drug effects* / Q000378:metabolism / Q000503:physiopathology*; D002998:Clonazepam / Q000008:administration & dosage*; D004525:Efferent Pathways / Q000276:immunology / Q000378:metabolism / Q000503:physiopathology; D018757:GABA Modulators / Q000008:administration & dosage; D006801:Humans; D007223:Infant; D008297:Male; D009433:Neural Inhibition / Q000187:drug effects* / Q000502:physiology; D009447:Neuroblastoma / Q000150:complications / Q000276:immunology / Q000601:surgery; D019635:Neurosurgical Procedures; D053578:Opsoclonus-Myoclonus Syndrome / Q000188:drug therapy* / Q000378:metabolism / Q000503:physiopathology*; D010386:Pelvic Neoplasms / Q000150:complications / Q000276:immunology / Q000601:surgery; D011689:Purkinje Cells / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D016896:Treatment Outcome; D005680:gamma-Aminobutyric Acid / Q000378:metabolism
D001323:Autoantibodies; D018757:GABA Modulators; D005680:gamma-Aminobutyric Acid; D002998:Clonazepam