Motor Complications in Parkinson's Disease: Results from 3343 Patients Followed for up to 12 Years.

BACKGROUND: Motor complications are well recognized in Parkinson's disease (PD), but their reported prevalence varies and functional impact has not been well studied. OBJECTIVES: To quantify the presence, severity, impact and associated factors for motor complications in PD. METHODS: Analysis of three large prospective cohort studies of recent-onset PD patients followed for up to 12 years. The MDS-UPDRS part 4 assessed motor complications and multivariable logistic regression tested for associations. Genetic risk score (GRS) for Parkinson's was calculated from 79 single nucleotide polymorphisms. RESULTS: 3343 cases were included (64.7% male). Off periods affected 35.0% (95% CI 33.0, 37.0) at 4-6 years and 59.0% (55.6, 62.3) at 8-10 years. Dyskinesia affected 18.5% (95% CI 16.9, 20.2) at 4-6 years and 42.1% (38.7, 45.5) at 8-10 years. Dystonia affected 13.4% (12.1, 14.9) at 4-6 years and 22.8% (20.1, 25.9) at 8-10 years. Off periods consistently caused greater functional impact than dyskinesia. Motor complications were more common among those with higher drug doses, younger age at diagnosis, female gender, and greater dopaminergic responsiveness (in challenge tests), with associations emerging 2-4 years post-diagnosis. Higher Parkinson's GRS was associated with early dyskinesia (0.026 ≤ P ≤ 0.050 from 2 to 6 years). CONCLUSIONS: Off periods are more common and cause greater functional impairment than dyskinesia. We confirm previously reported associations between motor complications with several demographic and medication factors. Greater dopaminergic responsiveness and a higher genetic risk score are two novel and significant independent risk factors for the development of motor complications.

Does thyroid diseases contribute to the natural history of idiopathic adult-onset dystonia? Data from the Italian Dystonia Registry.

A few earlier observations and recent controlled studies pointed to the possible contribution of thyroid diseases in idiopathic adult-onset dystonia (IAOD). The aim of this study was to investigate the association between thyroid status and clinical characteristics of IAOD, focusing on dystonia localization, spread, and associated features such as tremors and sensory tricks. Patients were identified from those included in the Italian Dystonia Registry, a multicentre dataset of patients with adult-onset dystonia. The study population included 1518 IAOD patients. Patients with hypothyroidism and hyperthyroidism were compared with those without any thyroid disease. In the 1518 IAOD patients, 167 patients (11%; 95% CI 9.5-12.6%) were diagnosed with hypothyroidism and 42 (2.8%; 95% CI 1.99-3.74) with hyperthyroidism. The three groups were comparable in age at dystonia onset, but there were more women than men in the groups with thyroid disease. Analysing the anatomical distribution of dystonia, more patients with blepharospasm were present in the hyperthyroidism group, but the difference did not reach statistical significance after the Bonferroni correction. The remaining dystonia-affected body sites were similarly distributed in the three groups, as did dystonia-associated features and spread. Our findings provided novel information indicating that the high rate of thyroid diseases is not specific for any specific dystonia subpopulation and does not appear to influence the natural history of the disease.

Demographics and Clinical Characteristics Associated with the Spread of New-Onset Laryngeal Dystonia.

OBJECTIVES: Adult-onset idiopathic laryngeal dystonia (LD) can be associated with the risk of spread to muscles in the body. Subjects with extralaryngeal onset of dystonia have exhibited spread to the larynx. Previous studies analyze the spread of other dystonias but emphasis has not been placed on LD. The objective was to identify demographic and clinical factors contributing to the spread of dystonia to and from the larynx. METHODS: Data were obtained from the Dystonia Coalition (DC)-patients from 49 international clinical centers. Clinical and demographic data was taken from 143 out of 409 patients with diagnosed LD. Patient criteria included adult-onset LD diagnosed on exam with no co-morbid neurologic conditions and no dystonia in other locations. RESULTS: Among the 143 patients, 94 (65.7%) patients were diagnosed with focal laryngeal onset, with the remainder having extralaryngeal onset. Family history and age at study were statistically significant indicators of a patient developing laryngeal versus extralaryngeal onset of dystonia. Among the laryngeal onset group, 21 cases (22.3%) had an average time of 5.81 ± 5.79 years to spread from diagnosis, most commonly to neck (61.9%). Among extralaryngeal onset patients, mean time of larynx spread was 7.92 ± 7.737 years, most commonly to neck (22.7%). CONCLUSIONS: Our data indicates approximately a quarter of patients with laryngeal-onset dystonia will exhibit spread. There were no demographic or clinical factors that were statistically predictive of the likelihood of spread from larynx. Patients with dystonia elsewhere in the body should be counseled on the possibility of spread to larynx, and vice versa. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:2295-2299, 2024.

Incidence and risk factors for secondary extralaryngeal dystonia in patients with laryngeal dystonia.

BACKGROUND AND OBJECTIVES: Laryngeal dystonia (LD) is a focal dystonia affecting adductor and/or abductor muscles of the larynx. It can be isolated or may spread to extra laryngeal muscles. The aim of this study was to report the characteristics of LD over time in a large single-center study with a long follow-up. METHODS: Retrospective review of patients with LD referred to our institution between 1991 and 2021. Demographic data, time to diagnosis, type of LD, follow-up and spread of dystonia [SD] were recorded. Risk factors for spread of dystonia during follow-up were analyzed. RESULTS: Over the 30-year period, 516 patients (77.3 % female, median age 50 years, range 5-87 years) were analyzed. Three hundred and fifteen patients (61 %) had adduction laryngeal dystonia, 136 patients (26.4 %) had abduction laryngeal dystonia, 46 patients (8.9 %) had adductor respiratory laryngeal dystonia, 12 patients (2.3 %) had mixed laryngeal dystonia, and seven patients (1.4 %) had singer's laryngeal dystonia. A previous history of dystonia was found in 47 patients (9.1 %). A laryngeal tremor was found in 68 patients (13.2 %). Since the onset of symptoms, LD was diagnosed after a median of 3 years (IQR: 1.0, 7.0). SD occurred in 55 patients (10.7 %) after a median time of 4 year (IQR: 1.5, 13.0). Patients with mixed laryngeal dystonia had higher probability of SD (p = 0.018). DISCUSSION: This study reports a large European study of LD, with a long follow-up. SD occurred in 10.5 % of patients. Patients with mixed laryngeal dystonia had a higher probability of SD. A close follow-up may be recommended for patients with mixed laryngeal dystonia.

Dystonia and tremor: Do they have a shared biology?

Dystonia and tremor are the two most commonly encountered hyperkinetic movement disorders encountered in clinical practice. While there has been substantial progress in the research on these two disorders, there also exists a lot of gray areas. Entities such as dystonic tremor and tremor associated with dystonia occupy a major portion of the "gray zone". In addition, there is a marked clinical heterogeneity and overlap of several clinical and epidemiological features among dystonia and tremor. These facts raise the possibility that dystonia and tremor could be having shared biology. In this chapter, we revisit critical aspects of this possibility that may have important clinical and research implications in the future. We comprehensively review the points in favor and against the theory that dystonia and tremor have shared biology from clinical, epidemiological, genetic and neuroimaging studies.

Oromandibular Dystonia: Clinical and Demographic Data from Eight-Two Patients.

Objective: This study aimed to determine the demographic and clinical characteristics of patients with oromandibular dystonia (OMD). Background: Dystonia is a movement disorder characterized by sustained involuntary muscle contractions that often cause abnormal postures. OMD is a rare focal dystonia that affects the tongue, jaw, and mouth. OMD, which is a rare public health problem, is often recognized as psychogenic and there are delays in its diagnosis and treatment. Methods: Patients with OMD, both isolated and combined, followed at our Movement Disorders Outpatient Clinic between 2004 and 2021 were enrolled in this study. Age, sex, age at onset, and disease duration were recorded. The type of OMD, affected muscles, etiologies of accompanying neurological disorders, and treatment were noted. Results: A total of 82 patients (44 women, 38 men) were included in this study. Among these, 39 patients had isolated OMD, and 43 patients had either segmental or generalized dystonia. Seven patients reported a family history of dystonia. Only nine patients reported a sensory trick. The average disease duration was 6.01 ± 3.73 (range, 1-29) years, and the average age at onset was 43.34 ± 18.24 (range, 1-78) years. The disease etiology was unknown (idiopathic) in most patients. Fifteen patients reported task-specific dystonia. The most common type of dystonia was jaw-opening dystonia. Conclusion: OMD is focal dystonia that significantly affects the quality of life. This study adds more data to the literature by defining the clinical features of this rare disorder and draws attention to this neglected type of dystonia.

Sex and gender differences in movement disorders: Parkinson's disease, essential tremor, dystonia and chorea.

Sex and gender-based differences in epidemiology, clinical features and therapeutical responses are emerging in several movement disorders, even though they are still not widely recognized. In this chapter, we summarize the most relevant evidence concerning these differences in Parkinson's disease, essential tremor, dystonia and chorea. Indeed, both sex-related biological (hormonal levels fluctuations) and gender-related variables (socio-cultural and environmental factors) may differently impact symptoms manifestation and severity, phenotype and disease progression of movement disorders on men and women. Moreover, sex differences in treatment responses should be taken into account in any therapeutical planning. Physicians need to be aware of these major differences between men and women that will eventually have a major impact on better tailoring prevention, treatment, or even delaying progression of the most common movement disorders.

Fatigue is related to depression in idiopathic dystonia.

INTRODUCTION: Dystonia is a movement disorder presented with involuntary muscle contraction causing abnormal posture, movement, or both. Besides motor symptoms, patients may also report non-motor symptoms such as pain, anxiety, apathy, depression, sleep problems, fatigue, and cognitive impairment. The etiology of fatigue in patients with dystonia is not yet well understood. AIM: To evaluate the presence of fatigue, depression, anxiety, sleep disorders, and daily sleepiness in patients with focal and segmental dystonia and to determine which of these non-motor symptoms influence the occurrence and severity of fatigue. PATIENTS AND METHODS: Patients were surveyed for symptoms of fatigue, depression, anxiety, night-time sleep problems, and daily sleepiness using the Fatigue Assessment Scale, Beck Depression Inventory II, Beck Anxiety Inventory, Pittsburgh Sleep Questionnaire Index, and Epworth Sleepiness Scale. Demographic data (sex, age, and disease duration) were collected from patient medical records. On statistical analysis, we used SPSS for Windows 10. The level of significance was set at p<0.05. RESULTS: Sixty patients (43 female and 17 male) with focal or segmental dystonia were evaluated. Fatigue was reported by 67.2% of patients. Fatigue (general, physical, and mental fatigue) was found to correlate with depression, anxiety, and sleep problems. Daily sleepiness correlated only with mental fatigue. Disease duration, age, and gender did not influence the symptoms of fatigue. Multiple regression analysis showed that depression mostly predicted symptoms of general, physical, and mental fatigue. CONCLUSION: Depression mostly predicted symptoms of general, physical, and mental fatigue in patients with focal and segmental dystonia.

Clinical and demographic characteristics of patients with functional movement disorders: a consecutive cohort study from a specialized clinic.

There is a growing interest in functional movement disorders (FMD). However, epidemiological data from large cohorts of patients with FMD are scarce and come mainly from General Neurology and Movement Disorders Clinics. Recently, specialized FMD clinics have been developed and epidemiological data from such clinics may provide useful information. We aimed to describe the clinical and sociodemographic features of patients diagnosed with FMD at our specialized FMD clinic. A standardized form was used to extract data from electronic records from the first-100 consecutive patients who were evaluated and diagnosed with FMD at our clinic from 2017 to 2019. Mean age was 40.88 ± (14.02) years, 63% females. Most patients were within working-age range, but only 16% were working at the time of consultation. Mean disease duration was 3.74 ± 5.73 years and was longer among men. The most common FMD were gait disturbance (42%), tremor (22%) and dystonia (15%). A precipitating event (mainly physical) was reported by 74%. The onset was mostly acute (83%) and the clinical course fluctuating (62%). Pain (64%) and fatigue (44%) were common comorbidities. Potential joint-hypermobility was present in 21%, mostly women (90%) and related to the presence of dystonia. FMD affects men and women mostly in working-age. Gait disturbance was the most common diagnosis, possibly because it causes a higher level of disability that may lead to consultation in a specialized clinic. Non-motor symptoms (pain and fatigue) were frequent in this cohort. Further data from specialized units may contribute to both understanding and management of FMD.

Adult-onset idiopathic dystonia: A national data-linkage study to determine epidemiological, social deprivation, and mortality characteristics.

BACKGROUND AND PURPOSE: Accurate epidemiological information is essential for the improved understanding of dystonia syndromes, as well as better provisioning of clinical services and providing context for diagnostic decision-making. Here, we determine epidemiological, social deprivation, and mortality characteristics of adult-onset idiopathic dystonia in the Welsh population. METHODS: A retrospective population-based cohort study using anonymized electronic health care data in Wales was conducted to identify individuals with dystonia between 1 January 1994 and 31 December 2017. We developed a case-ascertainment algorithm to determine dystonia incidence and prevalence, as well as characterization of the dystonia cohort, based on social deprivation and mortality. RESULTS: The case-ascertainment algorithm (79% sensitivity) identified 54,966 cases; of these cases, 41,660 had adult-onset idiopathic dystonia (≥20 years). Amongst the adult-onset form, the median age at diagnosis was 41 years, with males significantly older at time of diagnosis compared to females. Prevalence rates ranged from 0.02% in 1994 to 1.2% in 2017. The average annual incidence was 87.7/100,000/year, increasing from 49.9/100,000/year (1994) to 96.21/100,000/year (2017). In 2017, people with dystonia had a similar life expectancy to the Welsh population. CONCLUSIONS: We have developed a case-ascertainment algorithm, supported by the introduction of a neurologist-reviewed validation cohort, providing a platform for future population-based dystonia studies. We have established robust population-level prevalence and incidence values for adult-onset idiopathic forms of dystonia, with this reflecting increasing clinical recognition and identification of causal genes. Underlying causes of death mirrored those of the general population, including circulatory disorders, respiratory disorders, cancers, and dementia.

High prevalence of self-reported non-motor symptoms and lack of correlation with motor severity in adult patients with idiopathic isolated dystonia.

BACKGROUND: Although abnormal movements and postures are the hallmark of dystonia, non-motor symptoms (NMS) are common and negatively affect quality of life. OBJECTIVES: The aim of this study was to screen dystonia patients for NMS and analyze their association with clinical parameters, including motor disability. METHODS: Adult patients with idiopathic isolated dystonia were interviewed and examined. Dystonia severity was evaluated with the Fahn-Marsden Dystonia Rating Scale and the presence of NMS was assessed using a list of 29 complaints. RESULTS: A hundred and two patients (63.7% female) were enrolled. Dystonia began after 20 years of age in 61.8% and was focal or segmental in 82.8% of patients. Only eight patients (7.8%) had no NMS and 59.8% reported more than five. The most prevalent NMS were pain (72.5%) and anxiety (63.7%), followed by difficulty recalling information (44.1%), sadness/anhedonia (41.2%), and difficulty falling asleep (38.2%). No correlation was found between the total number of NMS and dystonia severity (p = 0.18) or regular botulinum toxin use (p = 0.66). The majority of NMS domains correlated with each other. CONCLUSIONS: Our results confirm a high prevalence of NMS among dystonia patients, even in those with mild motor disability. The pathophysiology of NMS in dystonia remains to be completely understood.

TOR1A mutation-related isolated childhood-onset generalised dystonia in South Africa.

BACKGROUND: Childhood-onset generalised dystonia is commonly caused by TOR1A mutations and is known to respond well to pallidal deep-brain stimulation (DBS) surgery. The incidence and prevalence of monogenic dystonia in individuals from Africa and specifically of African ancestry are unknown, and no local cases of TOR1A mutation dystonia are found in the literature. OBJECTIVES: To describe our experience with the outcome of TOR1A mutation-positive patients with isolated generalised dystonia (IGD) of childhood onset who were treated with pallidal DBS. METHODS: All patients with TOR1A mutations from Steve Biko Academic Hospital and the Pretoria Neurology Institute in Pretoria, South Africa (SA), who underwent DBS for IGD of childhood onset were identified. We conducted a retrospective analysis of their demographics, clinical presentation and time to generalisation, genetic status and family history, and response to DBS treatment of the internal segment of the globus pallidus (GPi), utilising pre- and post-surgical scores of the United Dystonia Rating Scale (UDRS). RESULTS: Three patients, all of black African ancestry, were identified. The median age at onset was 12 years and the median time to surgery from dystonia generalisation was 3 years. Two children presented with cervical-onset dystonia. Two patients were related, representing the only two with a positive family history. All three patients had a positive outcome after surgery, with improvement of 67 - 90% on the UDRS recorded at last follow-up. CONCLUSIONS: TOR1A mutations are found in SA patients of black African ancestry, with age of onset and generalisation comparable to those described in international studies. However, onset with cervical dystonia was more common than previously reported. Response to GPi DBS was excellent in all patients.

Tremor in Primary Monogenic Dystonia.

PURPOSE OF REVIEW: Tremor is an important phenotypic feature of dystonia with wide variability in the reported prevalence ranging from 14 to 86.67%. This variability may be due to the types of dystonia patients reported in different studies. This article reviews research articles reporting tremor in primary monogenic dystonia. RECENT FINDINGS: We searched the MDS gene data and selected all research articles reporting tremor in primary monogenic dystonia. Tremor was reported in nine dystonia genes, namely DYT-HPCA, DYT-ANO3, DYT-KCTD17, DYT-THAP1, DYT-PRKRA, DYT-GNAL, DYT-TOR1A, DYT-KMT2B, and DYT-SGCE in the descending order of its frequency. HPCA gene mutation is rare, but all reported patients had tremor. Similarly, tremor was reported in eight genes associated with dystonia parkinsonism, namely DYT-SLC6A3, DYT-TH, DYT-SPR, DYT-PTS, DYT-GCH1, DYT-TAF1, DYT-QDPR, and DYT-SCL30A10 in the descending order of its prevalence. DYT-HPCA and DYT-ANO3 gene showed the highest prevalence of tremor in isolated dystonia, and DYT-SLC6A3 has the highest prevalence of tremor in combined dystonia.

Understanding Psychiatric Disorders in Idiopathic and Inherited (Monogenic) Forms of Isolated and Combined Dystonia: A Systematic Review.

OBJECTIVE: The relationship between idiopathic and inherited (monogenic) forms of isolated and combined dystonia and psychiatric disorders remains unclear. In the present review, the authors aimed to provide increased clarity on this association through a systematic review of all controlled quantitative studies using a structured or semi-structured psychiatric interview to diagnose psychiatric disorders in individuals with these conditions. METHODS: Three databases were searched to identify 20 eligible studies, with a total of 1,275 participants fulfilling inclusion criteria. Eligible articles were quality appraised and divided into four sections (idiopathic forms of dystonia [N=11], early-onset torsion dystonia [N=2], gene mutation positive myoclonus dystonia; DYT-SGCE [N=6], and rapid-onset dystonia-parkinsonism [N=1]). RESULTS: For each study, results were grouped into subcategories (overall psychiatric comorbidity, anxiety disorders, mood disorders, substance misuse, and other [personality disorder and cognitive impairment]). For idiopathic dystonia, higher rates of psychiatric comorbidity, including mood and anxiety disorders, were noted when cases were compared with both healthy control subjects and control groups with a medical comorbidity. However, for major depressive disorder and obsessive-compulsive disorder (OCD) specifically, no differences were seen between groups. Study subjects with DYT-SGCE appeared to be at higher risk of psychiatric comorbidity, major depressive disorder, OCD, and alcohol dependence than control populations. CONCLUSIONS: Overall, the prevalence of psychiatric comorbidity appears to be increased in individuals with idiopathic and inherited (monogenic) forms of isolated and combined dystonia compared with control subjects. This finding is not consistent for all comparisons, and further research is required to understand the nature of these associations and the underlying causative etiologies.

Predictive modeling of spread in adult-onset isolated dystonia: Key properties and effect of tremor inclusion.

BACKGROUND AND PURPOSE: Several clinical and demographic factors relate to anatomic spread of adult-onset isolated dystonia, but a predictive model is still lacking. The aims of this study were: (i) to develop and validate a predictive model of anatomic spread of adult-onset isolated dystonia; and (ii) to evaluate whether presence of tremor associated with dystonia influences model predictions of spread. METHODS: Adult-onset isolated dystonia participants with focal onset from the Dystonia Coalition Natural History Project database were included. We developed two prediction models, one with dystonia as sole disease manifestation ("dystonia-only") and one accepting dystonia OR tremor in any body part as disease manifestations ("dystonia OR tremor"). Demographic and clinical predictors were selected based on previous evidence, clinical plausibility of association with spread, or both. We used logistic regressions and evaluated model discrimination and calibration. Internal validation was carried out based on bootstrapping. RESULTS: Both predictive models showed an area under the curve of 0.65 (95% confidence intervals 0.62-0.70 and 0.62-0.69, respectively) and good calibration after internal validation. In both models, onset of dystonia in body regions other than the neck, older age, depression and history of neck trauma were predictors of spread. CONCLUSIONS: This predictive modeling of spread in adult-onset isolated dystonia based on accessible predictors (demographic and clinical) can be easily implemented to inform individuals' risk of spread. Because tremor did not influence prediction of spread, our results support the argument that tremor is a part of the dystonia syndrome, and not an independent or coincidental disorder.

Childhood dystonic reactions in the middle Black Sea region.

ABSTRACT: Acute dystonic reactions are a worrying reason for presentation to the pediatric emergency department and the pediatric neurology clinic in childhood. It must be diagnosed and treated quickly. The aim of this study was to examine the clinical presentations, etiological factors, and prognosis of patients presenting to our regional tertiary pediatric neurology clinic with a diagnosis of acute dystonic reactions in children.Nine pediatric patients who were treated for acute dystonic reactions between May, 2018 and May, 2020 and had adequate follow-up were included in the study. Medical record data were reviewed age, gender, etiology, features of family, treatment, and results.Three of the patients were female and 6 were male. Their average age was 11 years (4-17). All patients were evaluated as a drug-induced acute dystonic reaction. Of the 9 patients, 5 were due to metoclopramide, 3 were due to risperidone, and 1 was due to aripiprazole. It was learned that a similar situation against other drugs developed in the family history of 3 patients. As a treatment, all of them were intramuscularly applied biperiden suitable for their weight and 30 minutes dramatic improvement was observed. Additional dose had to be administered in only 1 case. All cases were discharged for 24 hours. No problem was observed in their follow-up.Drug-induced acute dystonic reaction can be diagnosed and has a clinical picture that completely resolves when effective treatment is applied. However, it should not be forgotten that it can reach life-threatening dimensions clinically.

Prevalence and incidence of oromandibular dystonia: an oral and maxillofacial surgery service-based study.

OBJECTIVES: Oromandibular dystonia is a focal dystonia characterized by sustained or intermittent contractions of the masticatory and/or tongue muscles. This epidemiological study aimed to estimate the prevalence and incidence of oromandibular dystonia in Kyoto (population: 1,465,701). MATERIALS AND METHODS: The population sample was citizens of Kyoto who visited our department between 2015 and 2019 and were differentially diagnosed by an oromandibular dystonia specialist having idiopathic (primary) and acquired (secondary) oromandibular dystonia. A total of 144 patients (100 women and 44 men; mean age, 57.5 years) were analyzed for clinical features, and the prevalence (prevalence date, January 1, 2020) and annual incidence were estimated. RESULTS: The male-to-female ratio was 1:2.3 (p<0.001). Age at onset was significantly (p<0.01) earlier in men (47.5 years) than that in women (56.9 years). The crude prevalence of oromandibular dystonia was estimated at 9.8 per 100,000 persons (95% confidence interval: 8.3-11.6) (idiopathic dystonia, 5.7 [4.6-7.1]; tardive dystonia, 3.4 [2.5-4.5]) and incidence at 2.0 (1.3-2.8) per 100,000 person-years (idiopathic dystonia, 1.2 [0.68-1.9], tardive dystonia, 0.68 [0.32-1.3]). The prevalence was 13.0 (10.5-15.8) in women and 6.3 (4.6-8.5) in men. All age groups showed female predominance. The highest prevalence was 23.6 (14.4-36.5) in women aged 60-69 years. CONCLUSIONS: As this is an oral and maxillofacial surgery service-based study, the actual prevalence of oromandibular dystonia may be even higher. CLINICAL RELEVANCE: It was suggested that oromandibular dystonia might be more common than cervical dystonia or blepharospasm.

Comparative study between idiopathic and non-idiopathic dystonia: a prospective observational study.

BACKGROUND: There are very few studies based on the updated dystonia classification. However, a comparison of the idiopathic and non-idiopathic dystonias based on the newer classification has not been done previously. OBJECTIVES: To study and compare the clinicoetiological profile of patients with idiopathic and non-idiopathic dystonia attending a movement disorder clinic of a tertiary care teaching institution. METHODS: All the consecutive dystonia patients from October 2017 to September 2019 fulfilling the inclusion criteria were subjected to a detailed clinical evaluation. Investigations were performed as per requirement. Patients were classified according to the consensus update on phenomenology and classification of dystonia. RESULTS: A total of 183 patients with dystonia were included, with 61.7% (113) males and 38.3% (70) females. The idiopathic group revealed a significantly earlier age of onset with cases slightly outnumbering (n = 96/183, 52.5%) the non-idiopathic group (n = 87/183, 47.5%). Focal dystonias were the commonest type in both the idiopathic (n = 58/96, 60.4%) and non-idiopathic groups (n = 30/87, 34.5%), while generalized dystonia accounted for 26.4% (n = 23/87) of the non-idiopathic cases and only 3.1% (n = 3/96) of the idiopathic cases. The majority of idiopathic cases were isolated dystonia (n = 93/96, 96.9%), while all hemidystonias were non-idiopathic. CONCLUSION: Focal dystonias were the commonest in both idiopathic and non-idiopathic groups, while generalized dystonia was significantly commoner in the non-idiopathic group. Acquired causes like drugs, perinatal insult were the commonest etiology in the non-idiopathic group. Hemidystonia was found exclusively in the non-idiopathic acquired group.