Session Information
Date: Sunday, October 7, 2018
Session Title: Other
Session Time: 1:45pm-3:15pm
Location: Hall 3FG
Objective: To report increased risks of Botulinum Toxin Injection for cervical dystonia and migraine in patients with Hypermobility Ehlers Danlos Syndrome.
Background: Ehlers-Danlos syndrome (EDS) is a group of inherited disorders of connective tissues. The prevalence of hypermobility form of EDS is unknown, although it is at least 1 in 5000. Myalgia (muscle pain) and arthralgia (joint pain) are common and may be severe in EDS[1]. Headaches are reported in about one-third of patients (including migraines, tension-type headaches, headache from cerebrospinal fluid leakage, headache from Chiari malformation, and cervicogenic headache from cervical spine hypermobility[2].
Methods: Data was retrospectively collected over a 5-year period from 2013 to 2018 in a university clinic. All patients with hypermobility EDS who were treated with Botulinum toxin injection and were evaluated by one movement disorder specialist in this clinic were included.
Results: All seven patients in this study were female with a mean age of 45, ranging from 25 to 64 years. Their diagnoses were intractable migraine headache, cervicogenic headache and/or cervical dystonia. Among them, four patients were initially treated by four other practitioners. OnabotulinumtoxinA was utilized with injection dose ranging from 100 units to 200 units. Following injection, all subjects had prolonged severe neck pain and headache, and neck “weakness”. Subsequently, five patients received repeated injections with a modified protocol including avoidance or reduction of cervical muscle injection. Four out of five of these patients reported significant improvement in headache control without neck pain, and one patient reported partial improvement with both headache and neck pain.
Conclusions: Patients with EDS have ligamentous laxity. Significant cervical muscle hypertrophy and increased muscular tone are often seen in these patients, likely representing muscular compensation to ligamentous laxity to help with joint stability. Botulinum toxin may break such compensation, leading to head and neck instability, worsening of pain and even serious injuries. Extra caution should be exercised for Botulinum toxin injection in patients with hypermobility EDS. Muscles involving head and neck stabilization should be avoided. A large prospective study is warranted to shed light on safety and efficacy of Botulinum toxin injection in hypermobility EDS patients.
References: 1. Gedalia A, Press J, Klein M, Buskila D (1993). Joint hypermobility and fibromyalgia in schoolchildren. Annals of the Rheumatic Diseases. 52 (7): 494–6. PMID 8346976. 2. Castori M, Morlino S, Ghibellini G, Celletti C, Camerota F, Grammatico P (2015). Connective tissue, Ehlers-Danlos syndrome(s), and head and cervical pain. Am J Med Genet C Semin Med Genet. 169C(1):84-96. PMID: 25655119.
To cite this abstract in AMA style:
C. Wang, E. Wang, H. Lei. Increased Risks of Botulinum Toxin Injection in Patients with Hypermobility Ehlers Danlos Syndrome: A Case Series [abstract]. Mov Disord. 2018; 33 (suppl 2). https://www.mdsabstracts.org/abstract/increased-risks-of-botulinum-toxin-injection-in-patients-with-hypermobility-ehlers-danlos-syndrome-a-case-series/. Accessed December 1, 2024.« Back to 2018 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/increased-risks-of-botulinum-toxin-injection-in-patients-with-hypermobility-ehlers-danlos-syndrome-a-case-series/