Late onset of cervical dystonia in a 39-year-old patient following cerebellar hemorrhage
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Cervical dystonia
Neuroradiology
Cervical dystonia
Pathway Analysis
Metabolic pathway
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The relation between age at dystonia onset and sex was investigated in 264 patients with cranial-cervical dystonia and 56 patients with upper limb dystonia. In cranial-cervical dystonia, women had a significantly greater age at the onset of dystonia than men. The association was independent of duration of disease and distance of referral, but it was no longer detectable after adjustment for educational level. In upper limb dystonia, men and women did not differ for age at dystonia onset, duration of disease, education level, or distance of referral. A significant inverse association between age at the onset of dystonia and education was observed in both cranial-cervical dystonia and upper limb dystonia series.
Cervical dystonia
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Physical examination findings of dystonia are often underrecognized, especially in the setting of other movement disorders such as essential tremor (ET).A patient with ET exhibited numerous textbook features of cervical dystonia, which were misattributed to ET by a primary care physician and two neurologists.To provide a clear and unmistakable visual example of the clinically significant characteristics of cervical dystonia in the setting of concomitant ET.
Cervical dystonia
Concomitant
Movement Disorders
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Focal dystonia
Cervical dystonia
Blepharospasm
Movement Disorders
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We reviewed the database of the Dystonia Clinic at the University of Kansas Medical Center for patients with dystonia and tremor. Of 296 patients with idiopathic dystonia, 24 had dystonic tremor, 20 with cervical dystonia had an isolated head-nodding tremor, two patients with writer9s cramp had ipsilateral hand tremor, and two patients with generalized dystonia had arm tremor. Eight patients, all with cervical dystonia, had essential tremor that preceded the onset of their dystonia.
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Abstract The link between dystonia and tremor has been known for decades, but the question of whether they are two separate illnesses or just different manifestations of one disease with the same pathophysiological background remains unanswered. We distinguish two types of tremor in dystonia: dystonic tremor (DT), which appears on the body part affected by dystonia, and tremor associated with dystonia (TAWD), which appears in locations where the dystonia does not occur. In this study, the frequency of occurrence of different forms of tremor was determined by clinical examination in a group of adult-onset isolated cervical dystonia (CD) patients treated with regular local injections of botulinum toxin A in our department. In total, 120 patients were included in the study, of which 70 (58.3%) had DT of the head. TAWD was, in all 14 cases (11.7%), observed on the upper limbs, in the form of static or intentional tremor. The aim of this study was to point out the presence of TAWD as one of the clinical signs of CD. DT occurred in more than half of the patients and appears to be a relatively common part of the clinical picture in patients with CD.
Cervical dystonia
Focal dystonia
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Background: Family studies of dystonia may be limited in part by small family size and incomplete ascertainment of dystonia in geographically dispersed families. Further, prevalence estimates of dystonia are believed to be underestimates, as most studies are clinic-based and many individuals do not present to a physician or are misdiagnosed. As a low-cost highly sensitive screening tool is needed to improve case detection for genetic and epidemiologic studies, the authors developed the Beth Israel Dystonia Screen (BIDS), a computer-assisted telephone interview. Objective: To evaluate the validity and utility of a computer-assisted telephone interview in screening for cervical dystonia. Methods: The BIDS was administered and videotaped neurologic examinations performed on 193 individuals from 16 families with cervical and cranial dystonia. With use of a final rating of definite dystonia, as determined by video review of a systematic neurologic evaluation, as the gold standard, the predictive value of a subset of questions from the BIDS was assessed. Results: A positive response to at least two of five screening questions had a sensitivity for cervical dystonia of 100% and a specificity of 92%. With use of a positive response to three or more questions, definite dystonia was determined with 81% sensitivity and 97% specificity. Conclusions: The Beth Israel Dystonia Screen (BIDS) identifies cervical dystonia with excellent sensitivity and specificity in a family-based sample. The authors recommend the BIDS for family studies, but cross-validation in a population sample is advisable before applying this method to epidemiologic studies.
Cervical dystonia
Gold standard (test)
Telephone interview
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Neuroradiology
Clinical neurology
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Cervical dystonia
Sanger sequencing
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Abstract In Iceland, the crude prevalence for all types of primary dystonia was 37.1/10 5 (confidence interval, 30.4–44.9). Focal dystonia had the highest prevalence (31.2/10 5 ), followed by segmental (3.1/10 5 ), multifocal (2.4/10 5 ) and generalized dystonia (0.3/10 5 ). Cervical dystonia was the most common focal dystonia (11.5/10 5 ), followed by limb dystonia (8.0/10 5 ), laryngeal dystonia (5.9/10 5 ), blepharospasm (3.1/10 5 ), and oromandibular dystonia (2.8/10 5 ). The male:female ratio for all patients was 1:1.9 ( P = 0.0007), and females outnumbered males in all subtypes except oromandibular dystonia. Mean age of onset for all patients was 42.7 years (range, 3–82 years). This prevalence of primary dystonia is higher than in most reported studies, possibly because of more complete ascertainment but the relative frequencies of dystonia subtypes is similar. © 2005 Movement Disorder Society
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