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

Parkinsonism Relat Disord. 2022 Jul:100:19-23. doi: 10.1016/j.parkreldis.2022.05.023. Epub 2022 Jun 4.

Abstract

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.

Keywords: Atypical parkinsonism; DCTN1 gene; Neurodegenerative disease; TDP-43.

MeSH terms

  • Antiparkinson Agents / adverse effects
  • Depression
  • Dyskinesia, Drug-Induced*
  • Dystonia* / drug therapy
  • Dystonia* / etiology
  • Dystonic Disorders* / diagnostic imaging
  • Dystonic Disorders* / drug therapy
  • Humans
  • Hypoventilation
  • Levodopa / adverse effects
  • Parkinson Disease* / complications
  • Parkinson Disease* / drug therapy
  • Parkinson Disease* / pathology
  • Parkinsonian Disorders* / complications
  • Parkinsonian Disorders* / diagnostic imaging
  • Parkinsonian Disorders* / drug therapy

Substances

  • Antiparkinson Agents
  • Levodopa

Supplementary concepts

  • Perry Syndrome