Objective: To identify the clinical profile and treatment response patterns in patients with truncal dystonia.

Background: Truncal Dystonia is characterized by involuntary contractions and postures of the paraspinal, abdominal and chest muscles. Presence of truncal dystonia usually points to a secondary cause for dystonia like exposure to dopamine receptor blockers or neurodegenerative illness. Rarely, it can occur as an idiopathic focal or segmental dystonia.

Method: Retrospective review of medical records and videos of patients of truncal dystonia in our registry from May 2016 to February 2019.

Results: A total of 14 patients (out of more than 500 patients of dystonia treated with botulinum toxin) with predominant truncal dystonia were found. There were nine males and five females with mean age of 52 years (range 33-70). Extensor truncal dystonia was most common (10/14) followed by camptocormia (4/14).The various etiologies included Idiopathic Parkinson’s disease (4/14), Tardive (3/14), Neurodegeneration with brain iron accumulation (genetically confirmed) (2/14) or idiopathic (5/14) in nature. All patients except one were refractory to a combination of oral medications tried over a minimum period of six months. Suboptimal relief was the most common indication for Botulinum toxin injection. 13 patients received electromyographic guided botulinum toxin in the involved muscles (lumbar paraspinals, rectus abdominis, pectoralis as indicated) and showed modest response over medications. Mean dose of abobotulinum toxin used was 500 units.

Conclusion: Truncal dystonia is a relatively rare presentation. It can be involved as an isolated dystonia or with involvement of other body regions. It is usually refractory to multiple oral medications but may show modest benefit with botulinum toxin injections.

References: 1. Comella, C. L., Shannon, K. M. and Jaglin, J. (1998), Extensor truncal dystonia: Successful treatment with botulinum toxin injections. Mov. Disord., 13: 552-555. 2. Ehrlich DJ, Frucht SJ. The phenomenology and treatment of idiopathic adult-onset truncal dystonia: A retrospective review. J Clin Mov Disord 2016;3:15.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/clinical-profile-and-treatment-response-patterns-in-patients-of-truncal-dystonia-a-retrospective-case-series-from-a-tertiary-care-hospital-in-india/