Familial Adolescent-Onset Scoliosis and Segmental Dystonia

REVIEW ARTICLE

David Bradley, Sean O’Riordan and Michael Hutchinson

Affiliation : Department of Neurology, St. Vincent’s University Hospital, Dublin, Ireland

ABSTRACT

Idiopathic scoliosis is a common disorder characterized by abnormal curvature of the spine with onset typically in adolescence. Adult-onset primary torsion dystonia (AOPTD) is a focal dystonia with onset in early-middle age consisting of abnormal twisting or directional movements and postures; common phenotypes include cervical dystonia, blepharospasm, and focal hand dystonia. Numerous case reports and case–control studies indicate an association between childhood and adolescent-onset scoliosis and the subsequent development of adult-onset focal dystonia, most commonly cervical or segmental dystonia. In this review we examine the literature linking these two disorders and consider the pathophysiology of their association.
 

Keywords: adult-onset primary torsion dystonia (AOPTD), segmental dystonia, cervical dystonia, idiopathic scoliosis

Correspondence: David Bradley (or Prof Michael Hutchinson), Department of Neurology, St. Vincent’s University Hospital, Elm Park, Dublin 4, Ireland. Tel: +35312774179; Fax: +35312773506; e-mail: david.bradley@ucd.ie (or mhutchin@iol.ie)