Document Detail


Clonazepam responsive opsoclonus myoclonus syndrome: additional evidence in favour of fastigial nucleus disinhibition hypothesis?
MedLine Citation:
PMID:  20094737     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Vimal Kumar Paliwal; Satish Chandra; Ritu Verma; Jayantee Kalita; Usha K Misra
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2010-01-22
Journal Detail:
Title:  Journal of neural transmission (Vienna, Austria : 1996)     Volume:  117     ISSN:  1435-1463     ISO Abbreviation:  J Neural Transm     Publication Date:  2010 May 
Date Detail:
Created Date:  2010-04-22     Completed Date:  2010-07-29     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9702341     Medline TA:  J Neural Transm     Country:  Austria    
Other Details:
Languages:  eng     Pagination:  613-5     Citation Subset:  IM    
Affiliation:
Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India. dr_vimalkpaliwal@rediffmail.com
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MeSH Terms
Descriptor/Qualifier:
Autoantibodies / metabolism
Cerebellar Cortex / immunology,  metabolism,  physiopathology
Cerebellar Nuclei / drug effects*,  metabolism,  physiopathology*
Clonazepam / administration & dosage*
Efferent Pathways / immunology,  metabolism,  physiopathology
GABA Modulators / administration & dosage
Humans
Infant
Male
Neural Inhibition / drug effects*,  physiology
Neuroblastoma / complications,  immunology,  surgery
Neurosurgical Procedures
Opsoclonus-Myoclonus Syndrome / drug therapy*,  metabolism,  physiopathology*
Pelvic Neoplasms / complications,  immunology,  surgery
Purkinje Cells / immunology,  metabolism,  pathology
Treatment Outcome
gamma-Aminobutyric Acid / metabolism
Chemical
Reg. No./Substance:
0/Autoantibodies; 0/GABA Modulators; 1622-61-3/Clonazepam; 56-12-2/gamma-Aminobutyric Acid

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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