Changes in pallidal neural activity following long-term symptom improvement from botulinum toxin treatment in DYT6 dystonia: a case report

Giorni, Andrea1,2; Coyne, Terry1,2; Silburn, Peter A.1,2; Mellick, George D.3; Sah, Pankaj1,4,5; Windels, Francois1

2022-01-09

10.1186/s13256-021-03215-4

JOURNAL OF MEDICAL CASE REPORTS   影响因子和分区

1752-1947

Background The globus pallidus internus is the main target for the treatment of dystonia by deep brain stimulation. Unfortunately, for some genetic etiologies, the therapeutic outcome of dystonia is less predictable. In particular, therapeutic outcomes for deep brain stimulation in craniocervical and orolaryngeal dystonia in DYT6-positive patients are poor. Little is known about the neurophysiology of the globus pallidus internus in DYT6-positive dystonia, and how symptomatic treatment affects the neural activity of this region. Case presentation We present here the case of a 55-year-old Caucasian female DYT6-dystonic patient with blepharospasm, spasmodic dysphonia, and oromandibular dystonia where single-unit and local field potential activity was recorded from the globus pallidus internus during two deep brain stimulation revision surgeries 4 years apart with no symptomatic improvement. Botulinum toxin injections consistently improved dysphonia, while some of the other symptoms were only inconsistently or marginally improved. Neural activity in the globus pallidus internus during both revision surgeries were compared with previously published results from an idiopathic dystonic cohort. Single-cell firing characteristics and local field potential from the first revision surgery showed no differences with our control group. However, during the second revision surgery, the mean firing rate of single units and local field potential power in the gamma range were lower than those present during the first revision surgery or the control group. Conclusions Symptoms related to facial movements were greatly improved by botulinum toxin treatment between revision surgeries, which coincided with lower discharge rate and changes in gamma local field oscillations.

Dystonia Deep brain stimulation Globus pallidus Botulinum toxin Case report

ESCI

英语

其他

General & Internal Medicine

Medicine, General & Internal

WOS:000740364700001

BMC

2-s2.0-85122730992

Web of Science

1.Univ Queensland, Queensland Brain Inst, Synapt Plast Lab, St Lucia, Qld 4072, Australia
2.St Andrews War Mem Hosp, Asia Pacific Ctr Neuromodulat, Brisbane, Qld, Australia
3.Griffith Univ, Griffith Inst Drug Discovery GRIDD, Brisbane, Qld, Australia
4.Southern Univ Sci & Technol, Brain Res Ctr, Shenzhen, Guangdong, Peoples R China
5.Southern Univ Sci & Technol, Dept Biol, Shenzhen, Guangdong, Peoples R China

Giorni, Andrea,Coyne, Terry,Silburn, Peter A.,et al. Changes in pallidal neural activity following long-term symptom improvement from botulinum toxin treatment in DYT6 dystonia: a case report[J]. JOURNAL OF MEDICAL CASE REPORTS,2022,16(1).

Giorni, Andrea,Coyne, Terry,Silburn, Peter A.,Mellick, George D.,Sah, Pankaj,&Windels, Francois.(2022).Changes in pallidal neural activity following long-term symptom improvement from botulinum toxin treatment in DYT6 dystonia: a case report.JOURNAL OF MEDICAL CASE REPORTS,16(1).

Giorni, Andrea,et al."Changes in pallidal neural activity following long-term symptom improvement from botulinum toxin treatment in DYT6 dystonia: a case report".JOURNAL OF MEDICAL CASE REPORTS 16.1(2022).