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Myoclonus-dystonia
- Source :
- Parkinsonism & Related Disorders, 69, 85-90. Elsevier
- Publication Year :
- 2019
-
Abstract
- Background: myoclonus-dystonia (M-D) due to a pathogenic variant of SGCE is an autosomal dominant inherited movement disorder. Apart from motor symptoms, psychiatric disorders are highly prevalent in patients with MD. Previous studies suggest, but never tested directly, that the type of psychiatric disorder differs between dystonia syndromes, probably related to disease specific pathology. Little is known about other non-motor symptoms (NMS) in M.D. Here, we systematically study NMS in M-D in direct comparison to other types of dystonia and healthy controls.Methods: Standardized questionnaires were used to assess type and severity of psychiatric co-morbidity, sleep problems, fatigue and quality of life. Results of M-D patients with a pathogenic variant of SGCE were compared to results of idiopathic cervical dystonia (CD) patients, dopa-responsive dystonia (DRD) patients with a pathogenic variant of GCH1 and controls.Results: We included 164 participants: 41 M-D, 51 CD, 19 DRD patients, 53 controls. Dystonia patients (M-D, CD and DRD) had an increased prevalence of psychiatric disorders compared to controls (56-74% vs. 29%). In M-D we found a significantly increased prevalence of obsessive-compulsive disorder (OCD) and psychosis compared to CD and DRD. All dystonia patients had more sleep problems (49-68% vs. 36%) and fatigue (42-73% vs. 15%) than controls. Compared to other dystonia subtypes, M-D patients reported less excessive daytime sleepiness and fatigue.Conclusion: Psychiatric comorbidity is frequent in all dystonia types, but OCD and psychosis are more common in M-D patients. Further research is necessary to elucidate underlying pathways.
- Subjects :
- 0301 basic medicine
Male
SYMPTOMS
SGCE
Excessive daytime sleepiness
Comorbidity
FATIGUE
CERVICAL DYSTONIA
0302 clinical medicine
Quality of life
QUALITY-OF-LIFE
Surveys and Questionnaires
Prevalence
Medicine
ANXIETY
Cervical dystonia
SCALE
Dystonia
Aged, 80 and over
Myoclonus-dystonia
Mental Disorders
PSYCHIATRIC-DISORDERS
Middle Aged
Phenotype
Neurology
Dystonic Disorders
Anxiety
Female
medicine.symptom
Adult
Sleep Wake Disorders
Psychosis
medicine.medical_specialty
Non-motor symptoms
03 medical and health sciences
Young Adult
Internal medicine
Sarcoglycans
Humans
Aged
business.industry
medicine.disease
Dopa-responsive dystonia
nervous system diseases
030104 developmental biology
Mutation
Neurology (clinical)
Geriatrics and Gerontology
business
Myoclonus
030217 neurology & neurosurgery
Subjects
Details
- Language :
- English
- ISSN :
- 13538020
- Volume :
- 69
- Database :
- OpenAIRE
- Journal :
- Parkinsonism & Related Disorders
- Accession number :
- edsair.doi.dedup.....7d94f1b9da7990b1de94847c84ee62ce