A form of fixed dystonia (FD) without evidence of basal ganglia lesions or neurodegeneration has been recently characterized (Schrag et al., Brain 2004;127:2360-2372), which may overlap the clinical spectrum of either complex regional pain syndrome or psychogenic dystonia. Transcranial magnetic stimulation studies in typically mobile dystonia revealed abnormal motor cortical excitability and sensori-motor integration. We compared 12 patients with limb FD to 10 patients with primary adult-onset typically mobile dystonia and 11 age-matched healthy volunteers. Measurements at the first digital interosseus representation area on both hemispheres included: short intracortical inhibition (SICI), contralateral silent period (cSP), and short and long afferent inhibition (SAI and LAI). Repeated measure ANOVA and post-hoc t-tests were used for statistical analysis. SICI was significantly reduced in both hemispheres of patients with "typical" and FD, compared to healthy subjects. For both hemispheres, cSP duration was shorter in both fixed and "typical" dystonia patients. SAI and LAI did not significantly differ between the three groups. The abnormal cortical excitability observed in FD might represent an underlying trait predisposing to different clinical forms of dystonia.
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http://dx.doi.org/10.1002/mds.21801 | DOI Listing |
CNS Neurosci Ther
December 2024
Department of Functional Neurosurgery, The Affiliated Brain Hospital of Nanjing Medical University, Nanjing, China.
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View Article and Find Full Text PDFLin Chuang Er Bi Yan Hou Tou Jing Wai Ke Za Zhi
January 2025
This study aimed to compare the effects of cochlear implantation(CI) on vestibular function in patients with large vestibular aqueduct syndrome(LVAS) and in patients with extremely severe deafness with normal inner ear structure. A total of 28 LVAS patients and 28 patients with normal inner ear structure who suffered from extremely severe deafness were selected. The parameters of caloric tests, bone conduction evoked cervical vestibular-evoked myogenic potentials(cVEMP), bone conduction evoked ocular vestibular-evoked myogenic potentials(oVEMP) and video head impulse tests(v-HIT) were compared between the two groups before and after CI.
View Article and Find Full Text PDFLin Chuang Er Bi Yan Hou Tou Jing Wai Ke Za Zhi
January 2025
To retrospectively analyze the results of auditory examination,vestibular function examination and laboratory examination of 63 patients diagnosed as vestibular neuritis.Methods:A total of 63 patients diagnosed with vestibular neuritis hospitalized in the Department of Otolaryngology, Head and Neck Surgery of the Third Affiliated Hospital of Sun Yat-sen University, from October 2012 to December 2022 were recruited. All patients met the diagnostic criteria for the 2022 Bárány association vestibular neuritis.
View Article and Find Full Text PDFLin Chuang Er Bi Yan Hou Tou Jing Wai Ke Za Zhi
January 2025
To explore the trend of hearing changes in infants with gene p.V37I mutation at different months. The subjects were 54 children(108 ears) with p.
View Article and Find Full Text PDFNeurobiol Dis
December 2024
The Florey Institute of Neuroscience and Mental Health, Parkville, Victoria 3052, Australia. Electronic address:
Background: Developmental and epileptic encephalopathies (DEE) are rare but severe neurodevelopmental disorders characterised by early-onset seizures often combined with developmental delay, behavioural and cognitive deficits. Treatment for DEEs is currently limited to seizure control and provides no benefits to the patients' developmental and cognitive outcomes. Genetic variants are the most common cause of DEE with KCNQ2 being one of the most frequently identified disease-causing genes.
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