DOPA RESPONSIVE DYSTONIA: A CASE REPORT
Authors
Abstract:
Dopa responsive dystonia (DRD) is a dystonic syndrome of childhood, usually affecting gait and subsequently developing into parkinsonism with a dramatic therapeutic response to levodopa. At the best of our recollection this is the first case in Iranian medical literature of a 13 year old boy, affected by this interesting, rare and treatable disease which can be easily diagnosed if clinicians have a high index of suspicion. We also have a brief discussion on the pathophysiology of DRD.
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dopa responsive dystonia: a case report
dopa responsive dystonia (drd) is a dystonic syndrome of childhood, usually affecting gait and subsequently developing into parkinsonism with a dramatic therapeutic response to levodopa. at the best of our recollection this is the first case in iranian medical literature of a 13 year old boy, affected by this interesting, rare and treatable disease which can be easily diagnosed if clinicians ha...
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full textDopa responsive dystonia.
There may be insufficient awareness of dopa responsive dystonia (DRD), which has a characteristic diurnal variation of symptoms. Two children are reported in whom the diagnosis of DRD was missed. The first was thought to have hysteria and the second hereditary spastic paraparesis. A full history is vital for the diagnosis of this important treatable syndrome.
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Treatment of dopa-responsive dystonia is one of the more satisfying experiences in clinical neurology. The response to treatment with levodopa is usually dramatic and complete with no long-term complications. Carbidopa/levodopa is the mainstay in treating dopa-responsive dystonia. There is some experience using anticholinergic agents, but they are more likely to cause side effects and do not tr...
full textDopa-responsive dystonia: a syndrome of selective nigrostriatal dopaminergic deficiency.
Dopa-responsive dystonia (DRD) is no longer a rare oddity. For the clinician, DRD poses a diagnostic challenge as its clinical presentation can be quite diverse. Marked and sustained response to L-dopa is the most crucial and absolute hallmark in confirming a diagnosis. Absence of degenerative nigral cell loss underlies the remarkable L-dopa response. The broadening spectrum of the clinical pre...
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Journal title
volume 13 issue 3
pages 235- 236
publication date 1999-11
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