Session Information
Date: Monday, September 23, 2019
Session Title: Other
Session Time: 1:45pm-3:15pm
Location: Agora 2 West, Level 2
Objective: To describe improvement in muscle spasms of the jaw and upper limb associated with Satoyoshi syndrome using botulinum toxin injections.
Background: Satoyoshi syndrome is a rare disorder of presumed autoimmune origin that typically presents with muscle spasms, alopecia, endocrinopathy, and diarrhea. Muscle spasms can be severe and painful. Improvement in muscle spasms and other symptoms has been previously reported with immunomodulatory therapies, including steroids, IVIG, and plasmapheresis.[1, 2] There is one prior report of botulinum toxin injections to target muscle spasms in oromandibular muscles in a patient with Satoyoshi syndrome,[3] but there are no reports of its use to target muscle spasms in the limbs in this setting.
Method: A 25 year-old woman with a diagnosis of Satoyoshi syndrome was seen for painful muscle spasms affecting her jaw, legs, and arms. She had symptom onset at age 11 with painful muscle spasms in her limbs. Muscle spasms of shoulder girdle muscles had been severe enough to cause dislocation of the right shoulder on multiple occasions. She received a clinical diagnosis of Satoyoshi syndrome after developing alopecia, diarrhea, and secondary amenorrhea, and after exhaustive clinical investigation to rule out other causes, including muscle biopsy, electrodiagnostic testing, and whole exome sequencing. She had no clinical response to multiple immunomodulatory therapies including steroids, IVIG, plasmapheresis, methotrexate, and tacrolimus. On exam she had normal tone and full power of all muscle groups to confrontational testing. Upon opening her jaw, she developed muscle spasms resulting in involuntary jaw clenching and side to side movements. When abducting either shoulder she developed intermittent spontaneous activation of multiple shoulder girdle muscles with a rippling and flickering appearance, at times forceful enough to move the limb and cause irregular jerking movements.
Results: Botulinum toxin injections using onabotulinumtoxinA were administered to bilateral masseters, temporalis, and deltoid muscles, and to the right pectoralis. After two years of injections every 3 months, she has experienced significant and sustained improvement in unwanted jaw and limb movements without notable adverse effects.
Conclusion: Botulinum toxin injections may be a useful adjunctive therapy to consider in patients with Satoyoshi syndrome with muscle spasms that are refractory to immunomodulatory therapies.
References: [1] Heger S, Kuester RM, Volk R, Stephani U, Sippell WG. Satoyoshi syndrome: a rare multisystemic disorder requiring systemic and symptomatic treatment. Brain Dev 2006;28(5):300-304. [2] Montanaro VV, Hora TF, Couto CM, Ribas FD. Adult-onset Satoyoshi syndrome in a young male. Neuromuscul Disord 2017;27(4):382-384. [3] Merello M, Garcia H, Nogues M, Leiguarda R. Masticatory muscle spasm in a non-Japanese patient with Satoyoshi syndrome successfully treated with botulinum toxin. Mov Disord 1994;9(1):104-105.
To cite this abstract in AMA style:
I. Bledsoe. Botulinum toxin treatment of muscle spasms in a case of Satoyoshi syndrome [abstract]. Mov Disord. 2019; 34 (suppl 2). https://www.mdsabstracts.org/abstract/botulinum-toxin-treatment-of-muscle-spasms-in-a-case-of-satoyoshi-syndrome/. Accessed September 7, 2024.« Back to 2019 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/botulinum-toxin-treatment-of-muscle-spasms-in-a-case-of-satoyoshi-syndrome/