Treatment of Isolated Dystonia with Zolpidem.
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ABSTRACT:
SUBMITTER: Miyazaki Y
PROVIDER: S-EPMC6353392 | biostudies-literature | 2016 May-Jun
REPOSITORIES: biostudies-literature
Publications
Treatment of Isolated Dystonia with Zolpidem.
Miyazaki Yoshimichi Y Koizumi Hidetaka H Miyamoto Ryosuke R Kawarai Toshitaka T Kaji Ryuji R
Movement disorders clinical practice 20151218 3
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Project description:Although there are several reports of the significant efficacy of zolpidem for treating dystonia, zolpidem is still considered an anecdotal treatment. Here, we evaluated the efficacy and safety of zolpidem for treating residual dystonia in patients who previously received various neurosurgical treatments majorly including deep brain stimulation and radiofrequency ablation. We retrospectively reviewed medical records from January 2021 to September 2021 to identify patients with dystonia who had been prescribed zolpidem after undergoing neurosurgery. Twenty patients were enrolled in this study, including those with blepharospasm (two), tongue dystonia (four), mouth dystonia (one), spasmodic dysphonia (two), cervical dystonia (six), focal hand dystonia (three), hemidystonia (two), blepharospasm with cervical dystonia (one), and mouth dystonia with cervical dystonia (one). Single doses of zolpidem ranged between 2.5 and 10 mg, while daily dosages ranged from 10 to 30 mg. The zolpidem dose prescribed was 5-10 mg, with single and daily doses of 7 ± 2.9 and 14.5 ± 6.0 mg, respectively. With zolpidem administration, the participants' Burke-Fahn-Marsden Dystonia Rating Scale-Movement Scale score significantly improved from 8.1 ± 6.7 to 3.7 ± 2.5 (50.6% improvement, p < 0.0001). Improvements in arm dystonia, blepharospasm, and spasmodic dysphonia were observed using the Arm Dystonia Disability Scale, Jankovic Rating Scale, and Voice Handicap Index, respectively. No improvements were observed in cervical dystonia on the Toronto Western Spasmodic Torticollis Rating Scale. Drowsiness, including three cases each of mild and moderate drowsiness, was the most frequent adverse effect (30%), which persisted for 2-3 h. Transient amnesia and rapid eye movement sleep behavior disorder occurred in two patients and one patient, respectively. Although our findings suggest that zolpidem can be a valuable treatment option for patients with residual dystonia after neurosurgical treatments, the beneficial effects for cervical dystonia were limited.
| S-EPMC9111172 | biostudies-literature
Project description:Selecting the appropriate treatment for dystonia begins with proper classification of disease based on age, distribution, and underlying etiology. The therapies available for dystonia include oral medications, botulinum toxin, and surgical procedures. Oral medications are generally reserved for generalized and segmental dystonia. Botulinum toxin revolutionized the treatment of focal dystonia when it was introduced for therapeutic purposes in the 1980s. Surgical procedures are available for medication-refractory dystonia, markedly affecting an individual's quality of life.
| S-EPMC3899473 | biostudies-literature
Project description:The clinical evaluation of a patient with dystonia is a stepwise process, beginning with classification of the phenomenology of the movement disorder(s), then formulation of the dystonia syndrome, which, in turn, leads to a targeted etiological differential diagnosis. In recent years, there have been significant advances in our understanding of the etiological basis of dystonia, aided especially by discoveries in imaging and genetics. In this review, we provide an update on the assessment of a patient with dystonia, including the phenomenology of dystonia and highlighting how to integrate clinical, imaging, blood, and neurophysiological investigations in order to formulate a dystonia syndrome. Evolving or emerging dystonia syndromes are reviewed, and potential etiologies of these as well as established dystonia syndromes listed to guide diagnostic testing. © 2013 Movement Disorder Society.
| S-EPMC4216675 | biostudies-literature
Project description:Isolated inherited dystonia-formerly referred to as primary dystonia-is characterized by abnormal motor functioning of a grossly normal appearing brain. The disease manifests as abnormal involuntary twisting movements. The absence of overt neuropathological lesions, while intriguing, has made it particularly difficult to unravel the pathogenesis of isolated inherited dystonia. The explosion of genetic techology enabling the identification of the causative gene mutations is transforming our understanding of dystonia pathogenesis, as the molecular, cellular and circuit level consequences of these mutations are identified in experimental systems. Here, I review the clinical genetics and cell biology of three forms of inherited dystonia for which the causative mutation is known: DYT1 (TOR1A), DYT6 (THAP1), DYT25 (GNAL).
| S-EPMC4391387 | biostudies-literature
Project description:Neuroimaging studies using functional magnetic resonance imaging (fMRI), which measures brain activity by detecting the changes in blood oxygenation levels, are advancing our understanding of the pathophysiology of dystonia. Neurobiological disturbances in dystonia, however, may affect neurovascular coupling and impact the interpretability of fMRI studies. We evaluated here whether the hemodynamic response patterns during a behaviorally matched motor task are altered in isolated cervical dystonia (CD). Twenty-five CD patients and 25 healthy controls (HCs) underwent fMRI scanning during a paced finger tapping task (nondystonic task in patients). Imaging data were analyzed using a constrained principal component analysis-a statistical method that combines regression analysis and principal component analysis and enables the extraction of task-related functional networks and determination of the spatial and temporal hemodynamic response patterns associated with the task performance. Data from three patients and two controls were removed due to excessive movement. No significant differences in demographics or motor performance were observed. Three task-associated functional brain networks were identified. During task performance, reduced hemodynamic responses were seen in a sensorimotor network and in a network that included key nodes of the default mode, executive control and visual networks. During rest, reductions in hemodynamic responses were seen in the cognitive/visual network. Lower hemodynamic responses within the primary sensorimotor network in patients were correlated with the increased dystonia severity. Pathophysiological disturbances in isolated CD, such as alterations in inhibitory signaling and dopaminergic neurotransmission, may impact neurovascular coupling. Not accounting for hemodynamic response differences in fMRI studies of dystonia could lead to inaccurate results and interpretations.
| S-EPMC7015799 | biostudies-literature
Project description:BackgroundVPS16 pathogenic variants have been recently associated with inherited dystonia. Most patients affected by dominant VPS16-related disease display early-onset isolated dystonia with prominent oromandibular, bulbar, cervical, and upper limb involvement, followed by slowly progressive generalization.CasesWe describe six newly reported dystonic patients carrying VPS16 mutations displaying unusual phenotypic features in addition to dystonia, such as myoclonus, choreoathetosis, pharyngospasm and freezing of gait. Response to bilateral Globus Pallidus Internus Deep Brain Stimulation (GPi-DBS) is reported in three of them, associated with significant improvement of dystonia but only minor effect on other hyperkinetic movements. Moreover, five novel pathogenic/likely pathogenic variants are described.ConclusionsThis case collection expands the genetic and clinical spectrum of VPS16-related disease, prompting movement disorder specialists to suspect mutations of this gene not only in patients with isolated dystonia.
| S-EPMC10828607 | biostudies-literature
Project description:IntroductionPsychiatric symptoms are well recognised co-morbid traits in adult-onset idiopathic, isolated, focal cervical dystonia (AOIFCD), although few studies have sought to address their management. Internet-based cognitive behavioural therapy (iCBT) may provide an accessible solution. Here, we determine the feasibility of using iCBT in the management of non-motor symptoms for individuals with AOIFCD.MethodsParticipants were randomised to receive an 8-week iCBT programme (n = 10) or not (n = 10), both alongside routine clinical care. All participants underwent assessments at baseline, 3-, and 6- months for anxiety, depression, quality of life and motor symptoms, and engagement with iCBT was recorded. Group differences over time were determined using two-way mixed ANOVA, and simple statistics evaluated change on an individual participant level.ResultsOver half of participants receiving iCBT (6/10) showed high engagement, with feedback indicating most participants found iCBT useful (6/8), would continue to use it (7/8), and try it again if offered (7/8). Although no between-group significant differences were observed (e.g. Beck's Depression Inventory p = 0.067) anxiety and depression levels showed trends towards improvement at 3-months in those receiving iCBT. Individual level analysis also indicated higher percentage level improvements in these symptoms, with this sustained in 86% participants.ConclusioniCBT represents a feasible therapeutic option in the management of co-morbid anxiety and depression in AOIFCD. Further work is needed to replicate these findings in a larger cohort, identify those most likely to benefit from this form of therapy and overcome barriers hindering those less likely to engage with this form of treatment.
| S-EPMC8649077 | biostudies-literature
Project description:Treatment of dystonia and tics continues to evolve. In dystonia, while oral agents such as benzodiazepines, baclofen and anticholinergics remain in use, botulinum toxin (BoNT) continues to be regarded as the treatment of choice for focal and segmental dystonia, but new preparations are being studied. While deep brain stimulation (DBS) has typically focused on targeting the globus pallidus internus (GPi) when treating dystonia, more recent research has expanded the targets to include subthalamic nucleus (STN) and other targets. In addition to DBS, thalamotomies continue to show therapeutic benefit in focal hand dystonias. Treatment of tics includes a growing armamentarium of options besides the three FDA-approved drugs, all dopamine receptor blockers (haloperidol, pimozide and aripiprazole). Because of lower risk of adverse effects, dopamine depleters (e.g. tetrabebazine, deutetrabenazine, and valbenazine), along with novel D1 receptor antagonists, are currently studied as treatment alternatives in patients with tics. Practice guidelines for the treatment of tics and Tourette syndrome have been recently updated. Data regarding the use of DBS in treatment of tics remains relatively sparse, but international registries have expanded our understanding of the effect of stimulation at several targets.
| S-EPMC8302199 | biostudies-literature
Project description:Mutations in TUBB4A have been identified to cause a wide phenotypic spectrum ranging from hereditary generalized dystonia with whispering dysphonia (DYT4) to the leukodystrophy hypomyelination syndrome with atrophy of the basal ganglia and cerebellum (H-ABC). To test for the contribution of TUBB4A mutations in different ethnicities (Spanish, Italian, Korean, Japanese), we screened 492 isolated dystonia cases for mutations in this gene and for the first time determined TUBB4A copy number variations in 336 dystonia patients. A potentially pathogenic rare 3bp-in-frame deletion was found in a patient with cervical dystonia but no copy number variations were detected in this study, suggesting that TUBB4A mutations exceedingly rarely contribute to the etiology of isolated dystonia.
| S-EPMC5769152 | biostudies-other
Project description:There is increasing evidence that supports the role of the cerebellum in the pathophysiology of dystonia. We used transcranial magnetic stimulation to test the hypothesis that patients with cervical dystonia may have a disrupted cerebellar cortical connectivity at rest, and that cerebellar plasticity is altered too. We enrolled 12 patients with isolated cervical dystonia and 13 controls. A paired-pulse transcranial magnetic stimulation protocol was applied over the right cerebellum and the left primary motor area. Changes in the amplitude of motor evoked potentials were analysed. Continuous and intermittent Theta Burst Stimulation over the cerebellum was also applied. The effects of these repetitive protocols on cortical excitability, on intra-cortical circuits and on cerebellar cortical inhibition were analysed. In healthy subjects, but not in dystonic patients, a conditioning stimulus over the cerebellum was able to inhibit the amplitude of the motor evoked potentials from primary motor cortex. In healthy subjects continuous and intermittent cerebellar Theta Burst Stimulation were able to decrease and increase respectively motor cortex excitability. Continuous Theta Burst Stimulation was able to abolish the cerebellar cortical inhibition observed in basal condition. These effects were not observed in patients with cervical dystonia. Cerebellar cortical connectivity and cerebellar plasticity is altered at rest in patients with cervical dystonia.
| S-EPMC6347195 | biostudies-literature