Stem cells have proved to be the “wonder treatment” for various genetic diseases and holds great potential for the treatment of numerous, but presently incurable maladies. However, stem cells may not be the answer for all such diseases. With the rampant growth of clinics offering stem cell therapy for almost every incurable disease, it is prudent that the indications, ethical considerations, and potential side effects of this treatment are known to the physicians and patients. In this article, we have summarized the available evidence on stem cell therapy in spinocerebellar ataxias.

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BACKGROUND AND AIM: Clinical services were severely affected globally during the COVID-19 pandemic. This study aimed to characterize the clinical experience of using botulinum toxin (BTX) injections during the COVID-19 pandemic. METHODS: This is a retrospective chart review of patients who received BTX injections from April 2019 to January 2022. RESULTS: A total of 105 patients received an BTX injections, out of which 76 (72.4%) were men. The mean age of the patients was 47.9 ± 15.1 years. The most common indication for receiving BTX injections was dystonia (n = 79; 75.2%), followed by hemifacial spasm (n = 22; 21%) and miscellaneous movement disorders (n = 4; 3.8%). Focal dystonia (n = 45; 57%) was the most frequent form of dystonia, followed by segmental dystonia (n = 24; 30%). The percentage of generalized dystonia and hemidystonia was 12% and 1%, respectively. Cervical dystonia (44.4%), blepharospasm (17.8%), and writer’s cramp (15.6%) were the most frequent forms of focal dystonia. The miscellaneous group included four patients (3.8%) with trigeminal neuralgia, Holmes tremor, dystonic tics, and hemimasticatory spasm. The mean ages of patients in the dystonia, hemifacial spasm, and the miscellaneous groups were 47.7 ± 14.9 years, 49.2 ± 14.0 years, and 44.2 ± 26.0 years, respectively. The mean BTX dose was 131.6 ± 104.1 U. The mean BTX doses for the dystonia group, hemifacial spasm, and the miscellaneous group were 158.7 ± 105.3 U, 40.1 ± 11.3 U, and 100.0 ± 70.7 U, respectively. CONCLUSION: Most patients in our cohort had dystonia, followed by hemifacial spasm. Among the patients with dystonia, most had focal dystonia, with cervical dystonia being the most common movement disorder. The data obtained in our study is important to increase awareness of the effectiveness of BTX injections in patients with chronic disorders.

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OBJECTIVE: Diabetic striatopathy (DS) is characterized by a hyperglycemic state associated with chorea/ballism, and/or striatal hyperdensity on computed tomography, or hyperintensity on T1-weighted magnetic resonance imaging. To date, there have been only a few case series reported in the literature on this topic. In the present study, we report four cases of DS associated with movement disorders. METHODS: The patients were recruited based on the presence of hyperglycemia associated with chorea/ballism or striatal hyperintensity on T1-weighted magnetic resonance imaging. RESULTS: Four patients with DS (two men and two women), with a mean age of 61 years, were included in our study. Three out of the four patients had a previous diagnosis of type 2 diabetes mellitus. The mean blood glucose level on admission and glycated hemoglobin were 390.25 mg/dl and 12.45%, respectively. Hemiballism was present in two patients: one patient had dystonia and the other had choreiform movement at presentation. The putamen was affected in all patients, with involvement of the globus pallidus and caudate nucleus in one patient. All patients had resolution of their abnormal movements after glucose-lowering therapy, with additional use of anti-chorea medication in three patients. CONCLUSION: DS should be considered in elderly patients who present with chorea/ballism/dystonia and should be accordingly managed with resolution of abnormal movements. In addition, dystonia can be a presenting symptom in DS.

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Propriospinal myoclonus (PSM) is characterized by predominant axial jerk-like movements. To date, most of the PSM cases reported in the literature are considered to be functional (psychogenic) in origin. The “huffing and puffing sign” is a clinical clue with high diagnostic specificity for a functional etiology in gait disorders. However, this sign has not yet been described in patients with PSM. Here, we report a case of PSM of functional etiology, with coexisting gait disorder and the “huffing and puffing sign.” Our case adds to the list of clinical diagnostic clues that suggest a functional etiology of PSM.

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Objective: To develop an automated algorithm to detect, quantify, and differentiate between tremor using pen-on-paper spirals. Methods: Patients with essential tremor (n = 25), dystonic tremor (n = 25), Parkinson’s disease (n = 25), and healthy volunteers (HV, n = 25) drew free-hand spirals. The algorithm derived the mean deviation (MD) and tremor variability from scanned images. MD and tremor variability were compared with 1) the Bain and Findley scale, 2) the Fahn–Tolosa–Marin tremor rating scale (FTM–TRS), and 3) the peak power and total power of the accelerometer spectra. Inter and intra loop widths were computed to differentiate between the tremor. Results: MD was higher in the tremor group (48.9 ± 26.3) than in HV (26.4 ± 5.3; p < 0.001). The cut-off value of 30.3 had 80.9% sensitivity and 76.0% specificity for the detection of the tremor [area under the curve: 0.83; 95% confidence index (CI): 0.75, 0.91, p < 0.001]. MD correlated with the Bain and Findley ratings (rho = 0.491, p = 0 < 0.001), FTM–TRS part B (rho = 0.260, p = 0.032) and accelerometric measures of postural tremor (total power, rho = 0.366, p < 0.001; peak power, rho = 0.402, p < 0.001). Minimum Detectable Change was 19.9%. Inter loop width distinguished Parkinson’s disease spirals from dystonic tremor (p < 0.001, 95% CI: 54.6, 211.1), essential tremor (p = 0.003, 95% CI: 28.5, 184.9), or HV (p = 0.036, 95% CI: -160.4, -3.9). Conclusion: The automated analysis of pen-on-paper spirals generated robust variables to quantify the tremor and putative variables to distinguish them from each other. Significance: This technique maybe useful for epidemiological surveys and follow-up studies on tremor.

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Sensory tricks, also known as “geste antagoniste” or “alleviating maneuvers,” refer to specific maneuvers that temporarily improve dystonic postures; this is often considered to be a hallmark of primary dystonia. Although classically described to be simple activities such as a gentle touch, they can be complex and multisensory, including tactile, proprioceptive, visual, auditory, and thermal stimuli or even imaginary tricks. To date, there is no concrete concept to explain the mechanisms by which geste antagoniste alleviate dystonia. The suggested mechanisms imply an increase in intracortical facilitation in dystonia, and balance between facilitation and inhibition is restored by the geste. This narrative review aims to provide a brief overview of geste antagoniste, covering the historical aspects, types of geste, known mechanisms, and implications.

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