The Impact of the Course of Disease before Botulinum Toxin Therapy on the Course of Treatment and Long-Term Outcome in Cervical Dystonia.

Harald Hefter,Sara Samadzadeh,Dietmar Rosenthal,Max Schomaecker,Isabelle Schomaecker

doi:10.3390/toxins13070493

Abstract

This study analyses the influence of the course of the disease of idiopathic cervical dystonia (CD) before botulinum toxin (BoNT) therapy on long-term outcomes. 74 CD-patients who were treated on a regular basis in the botulinum toxin outpatient department of the University of Düsseldorf and had received at least 3 injections were consecutively recruited after written informed consent. Patients were asked to rate the amount of change of CD in relation to the severity of CD at begin of BoNT therapy (IMPQ). Then they had to draw the course of disease of CD from onset of symptoms until initiation of BoNT therapy (CoDB-graph) on a sheet of paper into a square of 10 × 10 cm2 size. Remaining severity of CD was estimated by the treating physician using the TSUI-score. Demographical and treatment related data were extracted from the charts of the patients. Depending on the curvature four different types of CoDB-graphs could be distinguished. Time to BoNT therapy, increase of dose and improvement during BoNT treatment were significantly (p < 0.05) different when patients were split up according to CoDB-graph types. The lower the age at onset of symptoms, the shorter was the time to therapy (p < 0.02). Initial dose (p < 0.04) and actual dose (p < 0.009) were negatively correlated with the age of the patients at recruitment. The course of disease of CD before BoNT therapy has influence on long-term outcome. This has implications on patient management and information on the efficacy of BoNT treatment.

Highlights

Cervical dystonia (CD) is the most frequent form of focal dystonia [1,2,3,4], has a prevalence around 3 to 28/100,000 people in Europe and the U.S [3,5,6,7], and goes along with abnormal head and neck posture and abnormal voluntary and involuntary head movements [8,9]
74 CD-patients who were treated on a regular basis in the botulinum toxin outpatient department of the University of Düsseldorf and had received at least 3 injections were consecutively recruited after written informed consent
They had to draw the course of disease of CD from onset of symptoms until initiation of botulinum toxin (BoNT) therapy (CoDB-graph) on a sheet of paper into a square of 10 × 10 cm2 size

Summary

Introduction

Cervical dystonia (CD) is the most frequent form of focal dystonia [1,2,3,4], has a prevalence around 3 to 28/100,000 people in Europe and the U.S [3,5,6,7], and goes along with abnormal head and neck posture and abnormal voluntary and involuntary head movements [8,9]. As in children with cerebral palsy (CP) [22] primary non-response [17,23], disease progression [24,25,26,27], cost of therapy, inconvenience, side effects and incorrect diagnoses [21,22,28] contribute to discontinuation of BoNT therapy in CD. In children with CP secondary non-response was associated with neutralizing antibody (NAB) formation in up to 75% [22]. This is consistent with recent crosssectional studies in adult CD-patients showing a non-linear increase of NAB-formation with duration of therapy [31,32,33]. The current opinion seems to be that “NAB formation occurs only in a small percentage” of BoNT/A treated patients and does not explain secondary non-response and discontinuation of BoNT therapy in the majority of CD-patients. [21,27,34,35]

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