Objective: The aim of the study is to fully characterize the spread of segmental/multifocal dystonia to a third body site in idiopathic adult-onset patients.
Background: With time adult-onset focal dystonia can spread to involve one, or less frequently, two additional regions of the body (segmental/multifocal dystonia). Several retrospective data and a recent prospective study indicated that the site of dystonia onset may affect the risk of initial spread [1]. While prior studies have advanced our understanding of spread of focal dystonia to a second body site, no study has fully characterized spread of segmental/multifocal dystonia to a third body site.
Method: We retrospectively analyzed data from the Italian Dystonia Registry [2], including patients with a diagnosis of segmental/multifocal dystonia involving at least two parts of the body or more.
Results: We identified 340 patients with segmental/multifocal dystonia involving at least two parts of the body. Spread of dystonia to a third body site occurred in 10/99 (10.1%) patients with segmental/multifocal dystonia and in 42/241 (17.4%) patients with focal onset. Patients with focal dystonia onset had a greater tendency to spread, but gender, years of schooling, comorbidity, family history of dystonia/tremor, age at dystonia onset, and disease duration did not affect the risk of spread to a third body site.
Conclusion: Spread to a third body site seems to occur in a relative low percentage of adult patients who develop idiopathic dystonia in two parts of the body. The tendency to spread to a third body site was greater in patients who presented with focal onset. However, neither the site of dystonia onset nor any other relevant demographic/clinical variable affected spread to a third body site in patients with focal onset.
References: [1] B.D. Berman, C.L. Groth, S.H. Sillau, S. Pirio Richardson, S.A. Norris, J. Junker, N. Brüggemann, P. Agarwal, R.L. Barbano, A.J. Espay, J.A. Vizcarra, C. Klein, T. Baümer, S. Loens, S.G. Reich, M. Vidailhet, C. Bonnet, E. Rose, H.A. Jinnah, J.S. Perlmutter, Risk of spread in adult-onset isolated focal dystonia: A prospective international cohort study, J. Neu-rol. Neurosurg. Psychiatry. (2019) 1–7. [2] G. Defazio, M. Esposito, G. Abbruzzese, C.L. Scaglione, G. Fabbrini, G. Ferrazzano, S. Pelu-so, R. Pellicciari, A.F. Gigante, G. Cossu, R. Arca, L. Avanzino, F. Bono, M.R. Mazza, L. Bertolasi, R. Bacchin, R. Eleopra, C. Lettieri, F. Morgante, M.C. Altavista, L. Polidori, R. Li-guori, S. Misceo, G. Squintani, M. Tinazzi, R. Ceravolo, E. Unti, L. Magistrelli, M. Coletti Moja, N. Modugno, M. Petracca, N. Tambasco, M.S. Cotelli, M. Aguggia, A. Pisani, M. Ro-mano, M. Zibetti, A.R. Bentivoglio, A. Albanese, P. Girlanda, A. Berardelli, The Italian Dy-stonia Registry: rationale, design and preliminary findings, Neurol. Sci. 38 (2017) 819–825.
To cite this abstract in AMA style:
T. Ercoli, R. Erro, G. Fabbrini, R. Pellicciari, P. Girlanda, C. Terranova, L. Avanzino, F. Di Biasio, P. Barone, M. Esposito, G. de Joanna, R. Eleopra, F. Bono, L. Manzo, AR. Bentivoglio, M. Petracca, MM. Mascia, A. Albanese, A. Castagna, R. Ceravolo, MC. Altavista, C. Scaglione, L. Magistrelli, M. Zibetti, L. Bertolasi, M. Coletti Moja, MS. Cotelli, G. Cossu, B. Minafra, A. Pisani, S. Misceo, N. Modugno, M. Romano, D. Cassano, A. Berardelli, G. Defazio. Spread to a third body site in patients with segmental/multifocal idiopathic adult-onset dystonia [abstract]. Mov Disord. 2021; 36 (suppl 1). https://www.mdsabstracts.org/abstract/spread-to-a-third-body-site-in-patients-with-segmental-multifocal-idiopathic-adult-onset-dystonia/. Accessed June 15, 2025.« Back to MDS Virtual Congress 2021
MDS Abstracts - https://www.mdsabstracts.org/abstract/spread-to-a-third-body-site-in-patients-with-segmental-multifocal-idiopathic-adult-onset-dystonia/