Premium
Spread of dystonia in patients with idiopathic adult‐onset laryngeal dystonia
Author(s) -
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.
Publication year - 2018
Publication title -
european journal of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.881
H-Index - 124
eISSN - 1468-1331
pISSN - 1351-5101
DOI - 10.1111/ene.13731
Subject(s) - dystonia , blepharospasm , medicine , focal dystonia , larynx , age of onset , botulinum toxin , pediatrics , surgery , disease , psychiatry
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.