Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
J Neural Transm (Vienna). 2010 May;117(5):613-5. doi: 10.1007/s00702-010-0366-y. Epub 2010 Jan 22.
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.
眼震-肌阵挛综合征是一种罕见的副肿瘤综合征，见于 50%的神经母细胞瘤患儿。眼震-肌阵挛的神经发生源尚不清楚，但有证据表明，小脑浦肯野细胞功能丧失导致 fastigial 核抑制解除在其中起作用。我们报告了一例副肿瘤性眼震-肌阵挛综合征患儿，该患儿对氯硝西泮反应良好。氯硝西泮的反应是 GABA 能神经回路参与眼震-肌阵挛综合征发生的证据，与 fastigial 核抑制解除假说一致。
