Generalized Dystonia Due to a Pathogenic Variant Showing Sustained Response to Globus Pallidus Deep Brain Stimulation.

Tremor and other hyperkinetic movements (New York, N.Y.) 2023 Vol.13() p. 23

Bhattacharjee S, Silverdale MA, Bonello M, Evans J, Kobylecki C

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Abstract

A 21-year-old woman of south Asian origin presented with cervical dystonia which had progressed over the previous three years. Her symptoms started as writer's cramp since the age of seven years. She did not respond to medications and needed botulinum toxin injection for generalised dystonia. Subsequent whole genome sequencing revealed a likely pathogenic c.98G>A p.(Cys33Tyr) heterozygous variant in the gene. She underwent bilateral posteroventral globus pallidus interna (GPi) deep brain stimulation (Medtronic Activa PC) implantation at the age of thirty-one years. She responded well to the deep brain stimulation even after more than 8 years post-surgery though she needs botulinum toxin injection for her cervical dystonia.

추출된 의학 개체 (NER)

유형영어 표현한국어 / 풀이UMLS CUI출처등장
시술 botulinum toxin 보툴리눔독소 주사 dict 2

MeSH Terms

Female; Humans; Child; Adult; Young Adult; Torticollis; Deep Brain Stimulation; Globus Pallidus; Dystonic Disorders; Botulinum Toxins; DNA-Binding Proteins; Apoptosis Regulatory Proteins

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