RESUMO
Given the good results of deep brain stimulation (DBS) in the treatment of movement disorders, DBS was initially tried to treat Lesch-Nyhan syndrome (LNS) with the aim to alleviate LNS-related dystonia. Some cases have reported clinical results of DBS in LNS thus far. This systematic review was conducted to comprehensively summarize cases of LNS treated with DBS and evaluate the efficacy and safety of DBS in LNS. Eight publications covering 12 LNS patients were included in this review. DBS improved dystonia of the LNS to varying degrees. All the included cases achieved partial or complete control of self-injurious behavior (SIB). Overall, DBS is a promising treatment for both motor and behavior disorders of LNS patients, but the results reported thus far have varied widely, especially for motor outcomes. The ultimate clinical benefits in LNS patients were still unpredictable. DBS-related complications were rather common, which raised questions about the safety of the procedure in LNS. More research is needed to further clarify the safety and effectiveness of this treatment.
Assuntos
Estimulação Encefálica Profunda , Distonia , Distúrbios Distônicos , Síndrome de Lesch-Nyhan , Humanos , Estimulação Encefálica Profunda/métodos , Distonia/terapia , Distúrbios Distônicos/terapia , Globo Pálido , Síndrome de Lesch-Nyhan/terapia , Síndrome de Lesch-Nyhan/complicações , Resultado do TratamentoRESUMO
The application of spinal cord stimulation (SCS) and deep brain stimulation (DBS) for disorders of consciousness (DoC) has been increasingly reported. However, there is no sufficient evidence to determine how effective and safe SCS and DBS are for DoC owing to various methodological limitations. We conducted a systematic review to elucidate the safety and efficacy of SCS and DBS for DoC by systematically reviewing related literature by searching PubMed, EMBASE, Medline, and Cochrane Library. Twenty eligible studies with 608 patients were included in this study. Ten studies with 508 patients reported the efficacy of SCS for DoC, and the estimated overall effectiveness rate was 37%. Five studies with 343 patients reported the efficacy of SCS for VS, and the estimated effectiveness rate was 30%. Three studies with 53 patients reported the efficacy of SCS for MCS, and the estimated effectiveness rate was 63%. Five studies with 92 patients reported the efficacy of DBS for DoC, and the estimated overall effectiveness rate was 40%. Four studies with 63 patients reported the efficacy of DBS for VS, and the estimated effectiveness rate was 26%. Three studies with 19 patients reported the efficacy of DBS for MCS, and the estimated effectiveness rate was 74%. The adverse event rate of DoC was 8.1% and 18.2% after SCS and DBS, respectively. These results suggest that SCS and DBS can be considered reasonable treatments for DoC with considerable efficacy and safety.
Assuntos
Estimulação Encefálica Profunda , Estimulação da Medula Espinal , Humanos , Estimulação da Medula Espinal/métodos , Estimulação Encefálica Profunda/métodos , Transtornos da Consciência/terapiaRESUMO
Deep brain stimulation (DBS) is a reversible treatment for chorea-acanthocytosis (ChAc). Its safety and efficacy remain elusive due to the low prevalence of ChAc. We aimed to investigate the safety and efficacy of DBS for ChAc by systematically reviewing literature through PubMed and EMBASE. Inclusion criteria were reports on the efficacy or safety of DBS for ChAc and English language articles, and exclusion criteria were other movement disorders, non-human subjects, and studies without original data. Most studies were published as case reports, and we therefore pooled these cases in one cohort. Twenty studies with 34 patients were included. The mean age of symptom onset was 29.3 years (range, 17-48). The median follow-up was 12 months (range, 2-84). Twenty-nine patients underwent GPi-DBS, two received STN-DBS, and one underwent Vop-DBS. Electrodes were implanted into the ventralis oralis complex of the thalamus and the pallidal in two patients. Symptoms seemed to be easier relieved in chorea (88.5%) and dystonia (76.9%) but dysarthria of most patients (85.7%) was no response after DBS. The Unified Huntington's Disease Rating Scale-Motor Score was used to assess the efficacy of DBS in 25 patients; the mean score decreased from 43.2 to 22.3 and the median improvement rate was 46.7%. Of 24 patients with data on adverse events, complications occurred in 9 patients (37.5%; mostly transient and mild events). DBS is a promising treatment for ChAc with satisfactory efficacy and safety based on the review. Pallidal and thalamic DBS have been applied in ChAc; GPi-DBS seems to be more widely used.