Journal
PARKINSONISM & RELATED DISORDERS
Volume 100, Issue -, Pages 19-23Publisher
ELSEVIER SCI LTD
DOI: 10.1016/j.parkreldis.2022.05.023
Keywords
Neurodegenerative disease; DCTN1 gene; TDP-43; Atypical parkinsonism
Categories
Funding
- NIH/NIA [1U19AG063911, FAIN: U19AG063911]
- NIH/NINDS [1U19AG063911, FAIN: U19AG063911]
- Mayo Clinic Center for Regenerative Medicine
- Haworth Family Professorship in Neurodegenerative Diseases fund
- Albertson Parkinson's Reuter Foundation
- Biohaven Pharmaceuticals, Inc. [BHV4157-206, BHV3241-301]
- Neuraly, Inc. [NLY01-PD-1]
- Vigil Neuroscience, Inc. [VGL101-01.001]
- Mayo Clinic in Florida Focused Research Team Program
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Patients with Perry syndrome (PS) show a marked and sustained response to L-Dopa and L-Dopa-induced dyskinesia (LID). The characteristic pattern of craniocervical dystonia may be a helpful clue to the diagnosis of PS.
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.
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