Successful Deep Brain Stimulation Surgery With Intraoperative Magnetic Resonance Imaging on a Difficult Neuroacanthocytosis Case

Lim, Thien Thien; Fernández, Hubert H.; Cooper, Scott E.; Wilson, Kathryn Mary K.; Machado, André G.

doi:10.1227/01.neu.0000429852.45073.73

Cited by 14 publications

(5 citation statements)

References 25 publications

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“…These findings, although observed in few patients, suggest that epilepsy may not be a contraindication or a serious concern for GPi-DBS in ChAc, but further studies are needed to address this point. Indeed, a recent case reported possible intraoperative seizures and also post-operative intracranial hematoma in a 32-years-old patient with non-molecularly proven ChAc [40]. Two years later, the patient underwent a second DBS operation leading to a marked improvement of his dystonia, chorea and overall quality of life 2 and 8 months postoperatively [40].…”

Section: Discussionmentioning

confidence: 99%

Short and Long Term Outcome of Bilateral Pallidal Stimulation in Chorea-Acanthocytosis

et al. 2013

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BackgroundChorea-acanthocytosis (ChAc) is a neuroacanthocytosis syndrome presenting with severe movement disorders poorly responsive to drug therapy. Case reports suggest that bilateral deep brain stimulation (DBS) of the ventro-postero-lateral internal globus pallidus (GPi) may benefit these patients. To explore this issue, the present multicentre (n=12) retrospective study collected the short and long term outcome of 15 patients who underwent DBS.MethodsData were collected in a standardized way 2-6 months preoperatively, 1-5 months (early) and 6 months or more (late) after surgery at the last follow-up visit (mean follow-up: 29.5 months).ResultsMotor severity, assessed by the Unified Huntington’s Disease Rating Scale-Motor Score, UHDRS-MS), was significantly reduced at both early and late post-surgery time points (mean improvement 54.3% and 44.1%, respectively). Functional capacity (UHDRS-Functional Capacity Score) was also significantly improved at both post-surgery time points (mean 75.5% and 73.3%, respectively), whereas incapacity (UHDRS-Independence Score) improvement reached significance at early post-surgery only (mean 37.3%). Long term significant improvement of motor symptom severity (≥20 % from baseline) was observed in 61.5 % of the patients. Chorea and dystonia improved, whereas effects on dysarthria and swallowing were variable. Parkinsonism did not improve. Linear regression analysis showed that preoperative motor severity predicted motor improvement at both post-surgery time points. The most serious adverse event was device infection and cerebral abscess, and one patient died suddenly of unclear cause, 4 years after surgery.ConclusionThis study shows that bilateral DBS of the GPi effectively reduces the severity of drug-resistant hyperkinetic movement disorders such as present in ChAc.

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Section: Discussionmentioning

confidence: 99%

Short and Long Term Outcome of Bilateral Pallidal Stimulation in Chorea-Acanthocytosis

et al. 2013

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“…In the context of these overall positive findings, some limitations must be highlighted in the published case reports. First, the data come from case reports or case series studies and articles involving operations performed by different surgeons and evaluations performed by different doctors (Table 1 4,[10][11][12][13][20][21][22][23][24][25][26][27][28][29][30][31][32][33][34] ), which may affect surgical efficacy evaluation. The majority of the reported cases did not use blind methods to evaluate symptoms, which could bias results.…”

Section: Discussionmentioning

confidence: 99%

Pallidus Stimulation for Chorea-Acanthocytosis: A Systematic Review and Meta-Analysis of Individual Data

Dong

et al. 2022

JMD

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A significant proportion of patients with chorea-acanthocytosis (ChAc) fail to respond to standard therapies. Recent evidence suggests that globus pallidus internus (GPi) deep brain stimulation (DBS) is a promising treatment option; however, reports are few and limited by sample sizes. We conducted a systematic literature review to evaluate the clinical outcome of GPi-DBS for ChAc. PubMed, Embase, and Cochrane Library databases were searched for relevant articles published before August 2021. The improvement of multiple motor and nonmotor symptoms was qualitatively presented. Improvements in the Unified Huntington's Disease Rating Scale motor score (UHDRS-MS) were also analyzed during different follow-up periods. A multivariate linear regression analysis was conducted to identify potential predictors of clinical outcomes. Twenty articles, including 27 patients, were eligible. Ninety-six percent of patients with oromandibular dystonia reported significant improvement. GPi-DBS significantly improved the UHDRS-motor score at < 6 months (p < 0.001) and ≥ 6 months (p < 0.001). The UHDRS-motor score improvement rate was over 25% in 75% (15/20 cases) of patients at long-term follow-up (≥ 6 months). The multiple linear regression analysis showed that sex, age at onset, course of disease, and preoperative movement score had no linear relationship with motor improvement at long-term follow-up (p > 0.05). GPi-DBS is an effective and safe treatment in most patients with ChAc, but no reliable predictor of efficacy has been found. Oromandibular dystonia-dominant patients might be the best candidates for GPi-DBS.

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“…As the clinical response to GPi‐DBS are symptom‐specific, discussions may be best placed in this context. For cardinal features of ChAc, it seems that chorea and orolingual dyskinesia have had rapid and satisfactory responses to both HFS and LFS . Interestingly, Guehl reported that chorea was improved only under LFS and exacerbated with HFS.…”

Section: Discussionmentioning

confidence: 99%

“…Interestingly, Guehl reported that chorea was improved only under LFS and exacerbated with HFS. For dystonia, varied improvements have been found with HFS and LFS, which appears to be delayed , less satisfactory and less frequency‐sensitive than chorea. Our findings on the symptoms of dysarthria are in accordance with previous reports that have noted dysarthria is generally insensitive to GPi‐DBS or even exacerbated after DBS regardless of stimulation frequency.…”

Section: Discussionmentioning

confidence: 99%

“…Taken together, these factors may collectively contribute to heterogeneous outcomes of GPi‐DBS. Thus, it remains important for the application of techniques for optimizing target accuracy, such as intraoperative microelectrode recordings and intraoperative MRI . An additional method for optimizing the area of stimulation is to change the contacts during programming.…”

Section: Discussionmentioning

confidence: 99%

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Pallidal Deep Brain Stimulation in Patients With Chorea-Acanthocytosis

Liu

Wan

et al. 2018

Neuromodulation: Technology at the Neural Interface

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Successful Deep Brain Stimulation Surgery With Intraoperative Magnetic Resonance Imaging on a Difficult Neuroacanthocytosis Case

Cited by 14 publications

References 25 publications

Short and Long Term Outcome of Bilateral Pallidal Stimulation in Chorea-Acanthocytosis

Short and Long Term Outcome of Bilateral Pallidal Stimulation in Chorea-Acanthocytosis

Pallidus Stimulation for Chorea-Acanthocytosis: A Systematic Review and Meta-Analysis of Individual Data

Pallidal Deep Brain Stimulation in Patients With Chorea-Acanthocytosis

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