Phenotypic Variability in Acquired and Idiopathic Dystonia

Author:

Defazio Giovanni1,Gigante Angelo Fabio2ORCID,Erro Roberto3ORCID,Belvisi Daniele45,Esposito Marcello6,Trinchillo Assunta7ORCID,De Joanna Gabriella6,Ceravolo Roberto89,Mazzucchi Sonia89,Unti Elisa89,Barone Paolo3,Scannapieco Sara3,Cotelli Maria Sofia10,Turla Marinella10,Bianchi Marta10,Bertolasi Laura11,Pisani Antonio1213ORCID,Valentino Francesca13,Altavista Maria Concetta14,Moschella Vincenzo14,Girlanda Paolo15,Terranova Carmen15,Bono Francesco16,Spano Giorgio16,Fabbrini Giovanni45,Ferrazzano Gina5,Albanese Alberto17ORCID,Castagna Anna18ORCID,Cassano Daniela19,Coletti Moja Mario20ORCID,Pellicciari Roberta21,Bentivoglio Anna Rita2223,Eleopra Roberto24,Cossu Giovanni25,Ercoli Tommaso1,Mascia Marcello Mario1ORCID,Di Biasio Francesca26ORCID,Misceo Salvatore2,Magistrelli Luca27,Romano Marcello28,Scaglione Cesa Lorella Maria29,Tinazzi Michele30ORCID,Maderna Luca31,Zibetti Maurizio3233ORCID,Berardelli Alfredo45ORCID

Affiliation:

1. Department of Medical Sciences and Public Health University of Cagliari Cagliari Italy

2. Section of Neurology San Paolo Hospital Bari Italy

3. Department of Medicine, Surgery and Dentistry "Scuola Medica Salernitana" University of Salerno Fisciano Italy

4. IRCCS Neuromed Pozzilli Italy

5. Department of Human Neurosciences Sapienza University of Rome Rome Italy

6. Clinical Neurophysiology Unit Cardarelli Hospital Naples Italy

7. Department of Neurosciences, Reproductive Sciences and Odontostomatology “Federico II” University Naples Italy

8. Parkinson and Movement Disorders Centre Neurology Unit Azienda Ospedaliero‐Universitaria Pisana Pisa Italy

9. Clinical and Experimental Medicine University of Pisa Pisa Italy

10. Neurology Unit ASST Valcamonica Esine Italy

11. Neurology Unit University Hospital Verona Italy

12. Department of Brain and Behavioral Sciences University of Pavia Pavia Italy

13. IRCCS Mondino Foundation Pavia Italy

14. Neurology Unit San Filippo Neri Hospital Rome Italy

15. Department of Clinical and Experimental Medicine University of Messina Messina Italy

16. Center for Botulinum Toxin Therapy Neurology Unit, A.O.U. Mater domini Catanzaro Italy

17. Department of Neurology IRCCS Humanitas Research Hospital Milan Italy

18. IRCCS Fondazione Don Carlo Gnocchi Onlus Milan Italy

19. Unit of Neurology Ospedale Maria Vittoria Turin Italy

20. Departmentt of Neurology Ospedale degli Infermi Ponderano Italy

21. Department of Basic Medical Sciences, Neurosciences and Sense Organs University of Bari “Aldo Moro” Bari Italy

22. Agostino Gemelli IRCCS University Hospital Foundation Rome Italy

23. Institute of Neurology Università Cattolica del Sacro Cuore Rome Italy

24. Neurology Unit 1, Fondazione IRCSS Istituto Neurologico “Carlo Besta” Milan Italy

25. Neurology Service and Stroke Unit, Department of Neuroscience AO Brotzu Cagliari Italy

26. Ospedale Policlinico San Martino IRCCS Genoa Italy

27. Movement Disorders Centre, Neurology Unit, Department of Translational Medicine University of Piemonte Orientale Novara Italy

28. Neurology Unit Villa Sofia Hospital Palermo Italy

29. IRCCS Institute of Neurological Sciences Ospedale Bellaria Bologna Italy

30. Neurology Unit, Department of Neuroscience, Biomedicine and Movement University of Verona Verona Italy

31. Department of Neuroscience, U.O. of Neurophysiology IRCCS Istituto Auxologico Italiano Milan Italy

32. Department of Neuroscience “Rita Levi Montalcini” University of Torino Torino Italy

33. Neurology 2 Unit A.O.U. Città Della Salute e Della Scienza di Torino Torino Italy

Abstract

AbstractBackgroundTo date, a few studies have systematically investigated differences in the clinical spectrum between acquired and idiopathic dystonias.ObjectivesTo compare demographic data and clinical features in patients with adult‐onset acquired and idiopathic dystonias.MethodsPatients were identified from among those included in the Italian Dystonia Registry, a multicenter Italian dataset of patients with adult‐onset dystonia. Study population included 116 patients with adult‐onset acquired dystonia and 651 patients with isolated adult‐onset idiopathic dystonia.ResultsComparison of acquired and idiopathic dystonia revealed differences in the body distribution of dystonia, with oromandibular dystonia, limb and trunk dystonia being more frequent in patients with acquired dystonia. The acquired dystonia group was also characterized by lower age at dystonia onset, greater tendency to spread, lower frequency of head tremor, sensory trick and eye symptoms, and similar frequency of neck pain associated with CD and family history of dystonia/tremor.ConclusionsThe clinical phenomenology of dystonia may differ between acquired and idiopathic dystonia, particularly with regard to the body localization of dystonia and the tendency to spread. This dissimilarity raises the possibility of pathophysiological differences between etiologic categories.

Publisher

Wiley

Subject

Neurology (clinical),Neurology

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