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This article is a tribute to Stanley Fahn, a prominent figure in the field of movement disorders. The authors discuss the origin and definition of dystonic tremor, specifically addressing the misconception that Fahn considered jerkiness a qualifier for dystonic tremor. They review Fahn's publications and clarify that he described dystonic tremor as oscillations seen as part of dystonia movements, emphasizing rhythmicity and oscillations as key features. The authors argue for a proper historical reconstruction of the original definition of dystonic tremor as a tribute to Fahn's contributions to the field. [Extracted from the article]

Background: The differentiation of functional dystonia from idiopathic dystonia may be clinically challenging. Objective: To identify clinical features suggestive of functional dystonia to guide physicians to distinguish functional dystonia from idiopathic dystonia. Methods: Patient data were extracted from the Italian Registry of Functional Motor Disorders and the Italian Registry of Adult Dystonia. Patients with functional and idiopathic dystonia were followed up at the same clinical sites, and they were similar in age and sex. Results: We identified 113 patients with functional dystonia and 125 with idiopathic dystonia. Sudden onset of dystonia, evidence of fixed dystonia, and acute peripheral trauma before dystonia onset were more frequent in the functional dystonia group. No study variable alone achieved satisfactory sensitivity and specificity, whereas a combination of variables yielded 85% sensitivity and 98% specificity. A diagnostic algorithm was developed to reduce the risk of misclassifying functional dystonia. Conclusion: Our findings extend the current diagnostic approach to functional dystonia by showing that clinical information about symptom onset, fixed dystonia, and history of peripheral trauma may provide key clues in the diagnosis of functional dystonia. [ABSTRACT FROM AUTHOR]

Keywords: dystonia, deep brain stimulation EN dystonia, deep brain stimulation 6 8 3 01/06/21 20210101 NES 210101 Dystonia syndromes have many different causes (inherited, acquired, or idiopathic) and variable phenomenology, as dystonia can be isolated, combined with another movement disorder or with additional neurological features.1 Symptomatic treatment is based on oral medications, focal injections of botulinum neurotoxins (BoNT), or deep brain stimulation (DBS).2 In this issue of I Movement Disorders Clinical Practice i , 2 patients with inherited combined syndromes are independently described to benefit from DBS targeted to the globus pallidus internus (GPi). The possibility of using GPi DBS was first shown in patients with early-onset generalized inherited dystonia, specifically I TOR1A i (DYT1) gene mutation carriers,5 who are now known to have long-lasting benefit.6 GPi DBS is currently accepted as a first-line symptomatic indication for DYT1 dystonia. In these cases, assessment of outcome is not based on dystonia rating scales but, rather, on the number and duration of dyskinetic spells at baseline and after implant.4 These data are therefore difficult to compare with those collected in patients with other dystonia syndromes. [Extracted from the article]

Background: The benefits of neurosurgery in Tourette Syndrome (TS) are still incompletely understood. Prefrontal cortical electrical stimulation offers a less invasive alternative to deep brain stimulation. Objective: To perform a pilot assessment on safety and efficacy of prefrontal cortical bilateral electrical stimulation in TS using clinical and brain metabolic assessments. Methods: Four adult TS patients underwent tic assessment using the Yale Global Tic Severity Scale and the Rush Video Rating Scale at baseline and 1, 3, 6, and 12‐months after implant; whereas FDG‐PET scans were acquired at baseline and after 6 and 12 months. Results: Tic clinical scores were improved at 6 months after implant, meanwhile they showed a tendency to re‐emerge at the 12‐month follow‐up. There was a correlation between FDG‐PET and tics, mainly consisting in a reduction of baseline brain hypermetabolism, which paralleled tic score reduction. Conclusion: Epidural stimulation in TS is safe and yields a modulation of tics, paralleled by FDG‐PET metabolic modulation. [ABSTRACT FROM AUTHOR]

Background: Functional motor disorders (FMD) are a frequent neurological condition affecting patients with movement disorders. Commonly described in younger adults, their manifestation can be also associated to an elderly onset., Objective: To assess the prevalence and describe the clinical manifestations of FMD with elderly and younger onset and their relationship with demographical and clinical variables., Methods: We recruited patients with a "clinically definite" diagnosis of FMD from the Italian Registry of FMD. Patients underwent extensive clinical assessments. For elderly onset, we set a chronological cut-off at 65 years or older according to WHO definition. Multivariate regression models were implemented to estimate adjusted odds ratio of elderly FMD onset related to clinical characteristics., Results: Among the 410 patients, 34 (8.2%) experienced elderly-onset FMD, with a mean age at onset of 70.9 years. The most common phenotype was tremor (47.1%), followed by gait disorders, weakness, and dystonia (29.4%, 23.5%, 14.7%, respectively). Eleven elderly patients had a combined phenomenology: 9 exhibited two phenotypes, 2 had three phenotypes. Weakness was isolated in 3/8 patients and combined with another phenotype in 5/8, manifesting as paraplegia (n = 4); upper limb diplegia (n = 2), hemiparesis/hemiplegia (n = 1), and tetraparesis/tetraplegia (n= 1). Non-motor and other functional neurological disorders occurred more frequently in the younger group (89.1%) than the elderly (73.5%). Neurological and non-neurological comorbidities were more prevalent in the elderly group (82.4%) as opposed to the younger (32.7%). In a multivariate regression analysis, elderly-onset FMD was significantly associated with neurological comorbidities, including parkinsonism (OR 6.73) and cerebrovascular diseases (OR 5.48)., Conclusions: These results highlight the importance of achieving an accurate diagnosis of FMD in the elderly, as it is crucial for effectively managing FMD symptoms and addressing neurological comorbidities., (© 2023 The Authors. Movement Disorders Clinical Practice published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.)

Background: Multiple system atrophy (MSA) is a devastating disease characterized by a variable combination of motor and autonomic symptoms. Previous studies identified numerous clinical factors to be associated with shorter survival., Objective: To enable personalized patient counseling, we aimed at developing a risk model of survival based on baseline clinical symptoms., Methods: MSA patients referred to the Movement Disorders Unit in Innsbruck, Austria, between 1999 and 2016 were retrospectively analyzed. Kaplan-Meier curves and multivariate Cox regression analysis with least absolute shrinkage and selection operator penalty for variable selection were performed to identify prognostic factors. A nomogram was developed to estimate the 7 years overall survival probability. The performance of the predictive model was validated and calibrated internally using bootstrap resampling and externally using data from the prospective European MSA Study Group Natural History Study., Results: A total of 210 MSA patients were included in this analysis, of which 124 patients died. The median survival was 7 years. The following clinical variables were found to significantly affect overall survival and were included in the nomogram: age at symptom onset, falls within 3 years of onset, early autonomic failure including orthostatic hypotension and urogenital failure, and lacking levodopa response. The time-dependent area under curve for internal and external validation was >0.7 within the first 7 years of the disease course. The model was well calibrated showing good overlap between predicted and actual survival probability at 7 years., Conclusion: The nomogram is a simple tool to predict survival on an individual basis and may help to improve counseling and treatment of MSA patients., (© 2023 The Authors. Movement Disorders Clinical Practice published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.)