Acetyl-DL-leucine in two individuals with REM sleep behavior disorder improves symptoms, reverses loss of striatal dopamine-transporter binding and stabilizes pathological metabolic brain pattern-case reports.

Autor: Oertel WH; Department of Neurology, Philipps University of Marburg, Marburg, Germany. oertelw@med.uni-marburg.de.; Institute of Neurogenomics, Helmholtz Center for Medicine and Environment, Munich, Germany. oertelw@med.uni-marburg.de., Janzen A; Department of Neurology, Philipps University of Marburg, Marburg, Germany., Henrich MT; Department of Neurology, Philipps University of Marburg, Marburg, Germany.; Department of Psychiatry and Psychotherapy, Philipps University of Marburg, Marburg, Germany.; Department of Neuroscience, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA., Geibl FF; Department of Neurology, Philipps University of Marburg, Marburg, Germany.; Department of Psychiatry and Psychotherapy, Philipps University of Marburg, Marburg, Germany.; Department of Neuroscience, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA., Sittig E; Department of Neurology, Philipps University of Marburg, Marburg, Germany., Meles SK; Department of Neurology, University Medical Center Groningen, Groningen, The Netherlands., Carli G; Department of Nuclear Medicine and Molecular Imaging, University Medical Center Groningen, Groningen, The Netherlands., Leenders K; Department of Nuclear Medicine and Molecular Imaging, University Medical Center Groningen, Groningen, The Netherlands., Booij J; Department of Radiology and Nuclear Medicine, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, The Netherlands., Surmeier DJ; Department of Neuroscience, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA., Timmermann L; Department of Neurology, Philipps University of Marburg, Marburg, Germany., Strupp M; Department of Neurology, LMU University Hospital, LMU, Munich, Germany. Michael.Strupp@med.uni-muenchen.de.
Jazyk: angličtina
Zdroj: Nature communications [Nat Commun] 2024 Sep 02; Vol. 15 (1), pp. 7619. Date of Electronic Publication: 2024 Sep 02.
DOI: 10.1038/s41467-024-51502-7
Abstrakt: Isolated REM Sleep Behavior Disorder (iRBD) is considered a prodrome of Parkinson's disease (PD). We investigate whether the potentially disease-modifying compound acetyl-DL-leucine (ADLL; 5 g/d) has an effect on prodromal PD progression in 2 iRBD-patients. Outcome parameters are RBD-severity sum-score (RBD-SS-3), dopamine-transporter single-photon emission computerized tomography (DAT-SPECT) and metabolic "Parkinson-Disease-related-Pattern (PDRP)"-z-score in 18 F-fluorodeoxyglucose positron emission tomography (FDG-PET). After 3 weeks ADLL-treatment, the RBD-SS-3 drops markedly in both patients and remains reduced for >18 months of ADLL-treatment. In patient 1 (female), the DAT-SPECT putaminal binding ratio (PBR) decreases in the 5 years pretreatment from normal (1.88) to pathological (1.22) and the patient's FDG-PET-PDRP-z-score rises from 1.72 to 3.28 (pathological). After 22 months of ADLL-treatment, the DAT-SPECT-PBR increases to 1.67 and the FDG-PET-PDRP-z-score stabilizes at 3.18. Similar results are seen in patient 2 (male): his DAT-SPECT-PBR rises from a pretreatment value of 1.42 to 1.72 (close to normal) and the FDG-PET-PDRP-z-score decreases from 1.02 to 0.30 after 18 months of ADLL-treatment. These results support exploration of whether ADLL may have disease-modifying properties in prodromal PD.
(© 2024. The Author(s).)
Databáze: MEDLINE
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