Spread of dystonia in patients with idiopathic adult-onset laryngeal dystonia
| Autor: | Giovanni Fabbrini, Ugo Cesari, Marcello Esposito, Anna Rita Bentivoglio, Giovanni Defazio, Alfredo Berardelli, Cesa Scaglione, Maurizio Zibetti, Marinella Turla, Marcello Romano, Laura Fadda, Roberto Erro, Tommaso Schirinzi, Stefano Peluso, Giovanni Cossu, Laura Avanzino, M. Coletti Moja, Roberto Eleopra, Paolo Barone, Gina Ferrazzano, Francesca Morgante, Brigida Minafra, Martina Petracca, Angelo Fabio Gigante |
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| Přispěvatelé: | Esposito, M., Fabbrini, G., Ferrazzano, G., Berardelli, A., Peluso, S., Cesari, U., Gigante, A. F., Bentivoglio, A. R., Petracca, M., Erro, R., Barone, P., Schirinzi, T., Eleopra, R., Avanzino, L., Romano, M., Scaglione, C. L., Cossu, G., Morgante, F., Minafra, B., Zibetti, M., Coletti Moja, M., Turla, M., Fadda, L., Defazio, G. |
| Rok vydání: | 2018 |
| Předmět: |
Male
Registrie 0301 basic medicine Larynx Pediatrics adult focal dystonia laryngeal dystonia spread Age Factors Age of Onset Aged Aged 80 and over Disease Progression Dystonia Dystonic Disorders Female Humans Italy Laryngeal Diseases Middle Aged Registries Sex Factors 0302 clinical medicine 80 and over Age Factor Laryngeal dystonia Dystonic Disorder Italian population Settore MED/26 - NEUROLOGIA medicine.anatomical_structure Laryngeal Disease Neurology Body region medicine.symptom Human medicine.medical_specialty Blepharospasm 03 medical and health sciences otorhinolaryngologic diseases medicine In patient Neurology (clinical) business.industry medicine.disease 030104 developmental biology business 030217 neurology & neurosurgery Clinical progression |
| Zdroj: | European Journal of Neurology. 25:1341-1344 |
| ISSN: | 1351-5101 1468-1331 |
| Popis: | BACKGROUND AND PURPOSE: Adult-onset laryngeal dystonia (LD) can be isolated or can be associated with dystonia in other body parts. Combined forms can be segmental at the onset or can result from dystonia spread to or from the larynx. The aim of this study was to identify the main clinical and demographic features of adult-onset idiopathic LD in an Italian population with special focus on dystonia spread. METHODS: Data were obtained from the Italian Dystonia Registry (IDR) produced by 37 Italian institutions. Clinical and demographic data of 71 patients with idiopathic adult-onset LD were extracted from a pool of 1131 subjects included in the IDR. RESULTS: Fifty of 71 patients presented a laryngeal focal onset; the remaining subjects had onset in other body regions and later laryngeal spread. The two groups did not show significant differences of demographic features. 32% of patients with laryngeal onset reported spread to contiguous body regions afterwards and in most cases (12 of 16 subjects) dystonia started to spread within 1 year from the onset. LD patients who remained focal and those who had dystonia spread did not show other differences. CONCLUSIONS: Data from IDR show that dystonic patients with focal laryngeal onset will present spread in almost one-third of cases. Spread from the larynx occurs early and is directed to contiguous body regions showing similarities with clinical progression of blepharospasm. This study gives a new accurate description of LD phenomenology that may contribute to improving the comprehension of dystonia pathophysiology. |
| Databáze: | OpenAIRE |
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