L-Dopa response, choreic dyskinesia, and dystonia in Perry syndrome

Jarosław Dulski, Catalina Cerquera-Cleves, Lukasz Milanowski, Jolanta Kwiatek-Majkusiak, Dariusz Koziorowski, Owen A. Ross, Jolanta Pentela-Nowicka, Jarosław Sławek, Zbigniew K. Wszolek

Research output: Contribution to journalArticlepeer-review


Introduction: A marked response to L-Dopa and L-Dopa-induced dyskinesia (LID) make the diagnosis of Parkinson's disease (PD) highly likely. This paper evaluates response to L-Dopa in Perry syndrome (PS), parkinsonism with distinct molecular and neuropathologic characteristics. Methods: Six patients with PS with a mean follow-up of 5 years (0.5–12) were assessed by movement disorder specialists and video recorded in states off and on. Additionally, DATSCAN-SPECT was performed in 3 subjects. Results: Four patients displayed a marked and sustained response to L-Dopa and LID. Additionally, we observed a distinct pattern of off-state predominant craniocervical dystonia responsive to L-Dopa in 4 patients, truncal dystonia in one, and dystonic head tremor in another. DATSCAN-SPECT was abnormal in 3 patients. Conclusions: Patients with PS may present PD-like parkinsonism with a marked and sustained response to L-Dopa and LID. The characteristic pattern of craniocervical dystonia may be a helpful clue to the diagnosis of PS.

Original languageEnglish (US)
Pages (from-to)19-23
Number of pages5
JournalParkinsonism and Related Disorders
StatePublished - Jul 2022


  • Atypical parkinsonism
  • DCTN1 gene
  • Neurodegenerative disease
  • TDP-43

ASJC Scopus subject areas

  • Neurology
  • Geriatrics and Gerontology
  • Clinical Neurology


Dive into the research topics of 'L-Dopa response, choreic dyskinesia, and dystonia in Perry syndrome'. Together they form a unique fingerprint.

Cite this