000			04150nam a22005177a 4500
008			201229s20202020 xxu||||| |||| 00| 0 eng d
022			_a0270-6474
024			_a10.1523/JNEUROSCI.1181-20.2020 [doi]
024			_aJNEUROSCI.1181-20.2020 [pii]
040			_aOvid MEDLINE(R)
099			_a33097635
245			_aThe Pathophysiology of Dystonic Tremors and Comparison With Essential Tremor.
251			_aJournal of Neuroscience. 40(48):9317-9326, 2020 11 25.
252			_aJ Neurosci. 40(48):9317-9326, 2020 11 25.
252			_zJ Neurosci. 2020 Oct 22
253			_aThe Journal of neuroscience : the official journal of the Society for Neuroscience
260			_c2020
260			_fFY2021
265			_saheadofprint
265			_sppublish
266			_d2020-12-29
268			_aJournal of Neuroscience. 2020 Oct 22
501			_aAvailable online from MWHC library: 1981 - present (after 18 months)
520			_aThere are two types of dystonic tremor syndromes (DTS), dystonic tremor (DT) and tremor associated with dystonia (TAWD), and neither is understood. DTS likely share some mechanisms with non-tremulous dystonia, and there may also be overlaps with essential tremor. We studied 21 ET (8 females, 13 males) and 22 DTS human patients (10 females, 12 males), including 13 human patients with DT (DT; writer's cramp with writing tremor) and 9 human patients with tremor associated with dystonia (TAWD; cervical dystonia with hand tremor). Tremors were analyzed using accelerometry and surface EMG of the antagonist pairs of arm muscles during posture, simple kinetic movement, and writing. Cerebellar inhibition (CBI) was performed to assess cerebello-thalamo-cortical (CTC) involvement. DT exhibited higher variability of peak frequency and greater instability of tremor burst intervals over time (higher tremor stability index; TSI) than ET or TAWD regardless of tasks. Intermuscular coherence magnitude between the antagonist pairs increased during the writing task in DT, but not ET or TAWD. ET and TAWD exhibited different phase relationships of the temporal fluctuations of voluntary movement and tremor in the kinetic condition. A linear discriminant classifier based on these tremor parameters was able to distinguish the three groups with a classification accuracy of 95.1%. CBI was significantly reduced in DT, but not in TAWD, compared to ET and healthy controls. Our study shows that the two DTS are distinct entities with DT closer to non-tremorous dystonia and TAWD closer to ET. SIGNIFICANCE STATEMENTThis study provides novel findings about characteristics and pathophysiology of the two different types of dystonic tremor syndromes (DTS), in comparison with essential tremor. Patients with DT are classified into DT (DT) who have dystonia and tremor in the same area, and tremor associated with dystonia (TAWD) who have dystonia and tremor elsewhere. Our results showed that DT exhibits increased tremor variability, instability, and intermuscular coherence, and decreased cerebello-thalamo-cortical inhibition compared to TAWD. Our study shows that DT and TAWD are distinct phenotypes, and that the physiological characteristics of DT are more similar to non-tremorous dystonia, and TAWD is closer to ET. Copyright (c) 2020 the authors.
546			_aEnglish
650			_a*Dystonia/pp [Physiopathology]
650			_a*Essential Tremor/pp [Physiopathology]
650			_a*Tremor/pp [Physiopathology]
650			_aAccelerometry
650			_aAged
650			_aCerebellum/pp [Physiopathology]
650			_aDystonic Disorders/pp [Physiopathology]
650			_aElectromyography
650			_aFemale
650			_aHumans
650			_aMale
650			_aMiddle Aged
650			_aMovement/ph [Physiology]
650			_aTranscranial Magnetic Stimulation
651			_aMedStar National Rehabilitation Network
657			_aJournal Article
700			_aLee, Sang Wook
790			_aCho HJ, Hallett M, Lee SW, Panyakaew P, Wu T
856			_uhttps://dx.doi.org/10.1523/JNEUROSCI.1181-20.2020 _zhttps://dx.doi.org/10.1523/JNEUROSCI.1181-20.2020
942			_cART _dArticle
999			_c5866 _d5866