Jaw clonus is a rare neurologic finding associated with supranuclear lesions of the trigeminal nerve. It is rare in amyotrophic lateral sclerosis (ALS).1 We describe a 63-year-old patient with ALS who presented with a disabling and sustained jaw clonus, reduced by mouth resting and elicited by mouth opening (Video 1). Association of baclofen (25 mg/3id), tizanidine (2 mg/id), and benzodiazepine treatments were ineffective. Onabotulinum toxin A injections were administered into the temporal (13 units) and masseter (25 units) muscles bilaterally. She experienced a complete relief of her symptom up to 4–5 months, with no side effects (Video 1). She confirmed improvement in quality of life.
Video 1
A disabling and sustained jaw clonus before and 4 months after onabotulinum toxin A treatment.Download Supplementary Video 1 via http://dx.doi.org/10.1212/201114_Video_1
Study Funding
No targeted funding reported.
Disclosure
The authors report no relevant disclosures. Go to Neurology.org/N for full disclosures.
Appendix Authors
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
↵* These authors contributed equally to this work.
Teaching slides links.lww.com/WNL/C274
Submitted and externally peer reviewed. The handling editor was Roy Strowd III, MD, MEd, MS.
- Received January 20, 2022.
- Accepted in final form June 30, 2022.
- © 2022 American Academy of Neurology
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