Levodopa response in Parkinsonism with multiple mitochondrial DNA deletions

Robert A. Wilcox, Andrew Churchyard, Henrik H. Dahl, Wendy M. Hutchison, Denise M. Kirby, Dominic Thyagarajan

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8 Citations (Scopus)


We report a patient with an autosomal dominant chronic progressive external ophthalmoplegia phenotype associated with multiple mtDNA deletions in muscle from a family in which linkage analysis excluded mutations in DNA polymerase γ (POLG), adenine nucleotide translocase (ANT-1) or C10orf2 (Twinkle). She presented with prominent Parkinsonism characterized by prolonged benefit from levodopa (L-dopa) and the later development of L-dopa induced dyskinesias and motor fluctuations. Thus L-dopa responsiveness, L-dopa induced dyskinesias and motor fluctuations may also occur in atypical Parkinsonism of mitochondrial disease, just as they may in multiple system atrophy.

Original languageEnglish
Pages (from-to)1020-1023
Number of pages4
JournalMovement Disorders
Issue number7
Publication statusPublished - 15 May 2007
Externally publishedYes


  • Chronic progressive external ophthalmoplegia
  • Mitochondrial disease
  • Neuropathy
  • Parkinson's disease
  • Parkinsonism

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