Response of motor complications in Cockayne syndrome to carbidopa-levodopa

Edward G. Neilan, Mauricio R. Delgado, Melissa A. Donovan, Sara Y. Kim, Rita L. Jou, Bai Lin Wu, Peter B. Kang

Research output: Contribution to journalArticlepeer-review

15 Scopus citations


Background: Gait difficulties, tremors, and coordination difficulties are common features of Cockayne syndrome that are consequences of leukodystrophy, cerebellar atrophy, and demyelinating neuropathy, but no pharmacotherapy for these disabling symptoms is available. Objective: To determine whether carbidopa-levodopa relieves tremors and other motor complications of Cockayne syndrome. Design: Mutation analysis and case report study. Setting: Hospital clinic and genetics research laboratory. Patients: We studied 3 patients with Cockayne syndrome, a rare autosomal recessive neurodegenerative disorder for which no known treatments are available. Intervention: Carbidopa-levodopa therapy. Main Outcome Measures: Status of tremors, ability to perform daily tasks, serial physical examinations, and results of handwriting samples. Results: All 3 patients had a clear reduction in tremors and improvements in handwriting and manipulation of utensils and cups. Conclusions: Patients with Cockayne syndrome should be evaluated carefully for movement disorders. A clinical trial should be considered to evaluate this therapy further.

Original languageEnglish (US)
Pages (from-to)1117-1121
Number of pages5
JournalArchives of neurology
Issue number8
StatePublished - Aug 2008

ASJC Scopus subject areas

  • Arts and Humanities (miscellaneous)
  • Clinical Neurology


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