AIMTo provide insight into outcome and long‐term safety and efficacy of deep brain stimulation (DBS), from the perspective of individuals with Lesch–Nyhan disease (LND) and their families.METHODWe used patient‐centered outcome measures to assess long‐term outcomes of DBS for 14 individuals (mean [SD] age 10y 10mo [5y 6mo], range 5–23y, all males) with LND, after an average duration of 5y 6mo (range 11mo–10y 5mo) after surgery. We compared these results with a comprehensive review of previously published cases.RESULTSPatients and their families reported that DBS of the globus pallidus can be effective both for motor and behavioral disturbances in LND. However, outcome measures were often not significantly changed owing to substantial variability among individuals, and were overall less positive than in previous reports based on clinician assessments. In addition, there was an unexpectedly high rate of adverse events, tempering overall enthusiasm for the procedure.INTERPRETATIONAlthough DBS might be an effective treatment for LND, more research is needed to understand the reasons for response variability and the unusually high rates of adverse events before DBS can be recommended for these patients. What this paper adds Individuals with Lesch–Nyhan disease and their families report variable efficacy of deep brain stimulation. Long‐term outcomes are associated with a high adverse event rate.

A consensus has yet to emerge whether deep brain stimulation (DBS) for treatment-refractory obsessive-compulsive disorder (OCD) can be considered an established therapy. In 2014, the World Society for Stereotactic and Functional Neurosurgery (WSSFN) published consensus guidelines stating that a therapy becomes established when “at least two blinded randomized controlled clinical trials from two different groups of researchers are published, both reporting an acceptable risk-benefit ratio, at least comparable with other existing therapies. The clinical trials should be on the same brain area for the same psychiatric indication.” The authors have now compiled the available evidence to make a clear statement on whether DBS for OCD is established therapy. Two blinded randomized controlled trials have been published, one with level I evidence (Yale-Brown Obsessive Compulsive Scale (Y-BOCS) score improved 37% during stimulation on), the other with level II evidence (25% improvement). A clinical cohort study (N = 70) showed 40% Y-BOCS score improvement during DBS, and a prospective international multi-center study 42% improvement (N = 30). The WSSFN states that electrical stimulation for otherwise treatment refractory OCD using a multipolar electrode implanted in the ventral anterior capsule region (including bed nucleus of stria terminalis and nucleus accumbens) remains investigational. It represents an emerging, but not yet established therapy. A multidisciplinary team involving psychiatrists and neurosurgeons is a prerequisite for such therapy, and the future of surgical treatment of psychiatric patients remains in the realm of the psychiatrist.

Deep brain stimulation (DBS) has been proposed for severe, chronic, treatment-refractory obsessive-compulsive disorder (OCD) patients. Although serious adverse events can occur, only a few studies report on the safety profile of DBS for psychiatric disorders. In a prospective, open-label, interventional multi-center study, we examined the safety and efficacy of electrical stimulation in 30 patients with DBS electrodes bilaterally implanted in the anterior limb of the internal capsule. Safety, efficacy, and functionality assessments were performed at 3, 6, and 12 months post implant. An independent Clinical Events Committee classified and coded all adverse events (AEs) according to EN ISO14155:2011. All patients experienced AEs (195 in total), with the majority of these being mild (52% of all AEs) or moderate (37%). Median time to resolution was 22 days for all AEs and the etiology with the highest AE incidence was ‘programming/stimulation’ (in 26 patients), followed by ‘New illness, injury, condition’ (13 patients) and ‘pre-existing condition, worsening or exacerbation’ (11 patients). Sixteen patients reported a total of 36 serious AEs (eight of them in one single patient), mainly transient anxiety and affective symptoms worsening (20 SAEs). Regarding efficacy measures, Y-BOCS reduction was 42% at 12 months and the responder rate was 60%. Improvements in GAF, CGI, and EuroQol-5D index scores were also observed. In sum, although some severe AEs occurred, most AEs were mild or moderate, transient and related to programming/stimulation and tended to resolve by adjustment of stimulation. In a severely treatment-resistant population, this open-label study supports that the potential benefits outweigh the potential risks of DBS.

Ataxia telangiectasia (A-T) is a multisystemic early-onset disorder caused by biallelic mutations in the ATM gene [ [1] Gatti R. Perlman S. Ataxia-telangiectasia. in: Adam M.P. Ardinger H.H. Pagon R.A. GeneReviews®. University of Washington, Seattle, Seattle (WA)1999 Google Scholar ]. The A-T phenotype consists of ataxia, hyperkinesias, oculomotor apraxia, polyneuropathy, intellectual disability, immunodeficiency, pulmonary symptoms and marked risk for malignancy. Classic A-T is progressive, leading to reduced life expectancy with loss of ambulation by age 10 [ [1] Gatti R. Perlman S. Ataxia-telangiectasia. in: Adam M.P. Ardinger H.H. Pagon R.A. GeneReviews®. University of Washington, Seattle, Seattle (WA)1999 Google Scholar ]. A milder phenotype with slower rate of progression is called variant ataxia telangiectasia (vA-T). Sometimes vA-T mimics primary generalized dystonia with ataxia being absent and with normal neuroimaging and immunoglobulin (Ig) levels. However, elevated α-fetoprotein (AFP) occurs in both classical A-T and vA-T [ [2] Verhagen M.M. Abdo W.F. Willemsen M.A. Hogervorst F.B. Smeets D.F. Hiel J.A. et al. Clinical spectrum of ataxia-telangiectasia in adulthood. Neurology. 2009 Aug 11; 73: 430-437 Google Scholar , [3] Saunders-Pullman R. Raymond D. Stoessl A.J. Hobson D. Nakamura K. Pullman S. et al. Variant ataxia-telangiectasia presenting as primary-appearing dystonia in Canadian Mennonites. Neurology. 2012 Feb 28; 78: 649-657 Crossref PubMed Scopus (70) Google Scholar ]. We report a case of vA-T with two novel observations [ [1] Gatti R. Perlman S. Ataxia-telangiectasia. in: Adam M.P. Ardinger H.H. Pagon R.A. GeneReviews®. University of Washington, Seattle, Seattle (WA)1999 Google Scholar ]: a severe generalized dystonia presentation, and [ [2] Verhagen M.M. Abdo W.F. Willemsen M.A. Hogervorst F.B. Smeets D.F. Hiel J.A. et al. Clinical spectrum of ataxia-telangiectasia in adulthood. Neurology. 2009 Aug 11; 73: 430-437 Google Scholar ] a novel sensory trick ("duck walk") to alleviate walking-associated camptocormia.

Background: In neuromodulation therapies, hardware-related infections are a major challenge often leading to hardware removal. Objective: To investigate the role of adjuvant hyperbaric oxygen therapy (HBOT) in hardware-related infections. Methods: Fourteen hardware-related infection events in 12 consecutive patients between 2002 and 2015 were treated with antibiotics and adjuvant HBOT at the Karolinska University Hospital (Stockholm, Sweden). Two time-independent infection events related to hardware replacements occurred in 2 patients. Infection resolution and the need for hardware removal were assessed. Results: Twelve out of 14 events of hardware-related infection were successfully treated without hardware removal (86%). The 2 patients treated twice with HBOT on 2 time-independent occasions could retain their hardware in both cases. Hardware was removed following HBOT failure in 2 infection events, with long-term infection control achieved in all patients. Further, an intrathecal pump malfunction caused by HBOT at 2.8 bars was observed, leading to a change in the manufacturer’s guidelines. Conclusions: This study indicates a potential benefit of adjuvant HBOT in the treatment of hardware-related infections in neuromodulation, diminishing the need for hardware removal and treatment interruption. Prospective studies are warranted to establish the role of adjuvant HBOT in the treatment of hardware-related infections in neuromodulation.

There is a need to improve outcome in patients with brain abscesses and hyperbaric oxygen therapy (HBOT) is a promising treatment modality. The objective of this study was to evaluate HBOT in the treatment of intracranial abscesses.

The mortality rate of patients with brain oedema after malignant middle cerebral artery (MCA) infarction approaches 80 % without surgical intervention. Surgical treatment with ipsilateral decompressive hemicraniectomy (DHC) has been shown to dramatically improve survival rates. DHC currently lacks established inclusion criteria and additional research is needed to assess the impact of prognostic factors on functional outcome. The aim of this study was to assess the impact of prognostic factors on functional outcome.

La estimulación espinal crónica (EEC) es un tratamiento eficaz para el dolor neuropático, sin embargo, aproximadamente un 40% de los pacientes no obtienen un adecuado alivio del dolor y además, muchos pacientes sienten que el efecto de la EEC tiende a disminuir con el paso del tiempo. En este artículo se describe una nueva estrategia terapéutica combinando la EEC con dosis bajas de drogas administradas por vía intratecal (i.t.) con el objetivo de potenciar el efecto de la EEC en pacientes, en los cuales la EEC no ofrece un efecto terapéutico adecuado. Para tener una mejor comprensión de este abordaje terapéutico, se desarrollan en forma resumida las bases neuroquímicas de los mecanismos de acción de la EEC, en particular los mecanismos colinérgicos y GABAérgicos estudiados en modelos experimentales de dolor neuropático.Para determinar la eficacia terapéutica de la combinación de la EEC con drogas i.t. que actúan sobre el sistema colinérgico o GA BAérgicos, clonidina y baclofen, se realizó un estudio randomizado, doble ciego, placebo/control en 10 pacientes refractarios a la EEC con dolor neuropático puro o asociado a dolor nociceptivo1. Siete de los diez pacientes reportaron reducción significativa del dolor al combinar la EEC con clonidina o con baclofen. En cinco de estos pacientes, previamente tratados solamente con EEC, fue implantada una bomba para la administración crónica de clonidina (n=3) o el baclofen (n=2). La clonidina como adyuvante a la EEC mostró ser más efectiva en los pacientes que presentaron dolor mixto (neuropático y nociceptivo) mientras que baclofen en combinación con EEC demostró ser una mejor opción en pacientes con dolor neuropático puro.El abordaje terapéutico de potenciar el efecto de la EEC por medio de la combinación con drogas por vía i.t. en pacientes refractarios a la EEC constituye una nueva estrategia en el campo de la neuromodulación para tratar el dolor neuropático severo. El estudio de los mecanismos de acción de la EEC proporcionará en el futuro mejores alternativas terapéuticas para mejorar el efecto de la EEC.

BACKGROUND:For patients with psychiatric illnesses remaining refractory to 'standard' therapies, neurosurgical procedures may be considered. Guidelines for safe and ethical conduct of such procedures have previously and independently been proposed by various local and regional expert groups. METHODS:To expand on these earlier documents, representative members of continental and international psychiatric and neurosurgical societies, joined efforts to further elaborate and adopt a pragmatic worldwide set of guidelines. These are intended to address a broad range of neuropsychiatric disorders, brain targets and neurosurgical techniques, taking into account cultural and social heterogeneities of healthcare environments. FINDINGS:The proposed consensus document highlights that, while stereotactic ablative procedures such as cingulotomy and capsulotomy for depression and obsessive-compulsive disorder are considered 'established' in some countries, they still lack level I evidence. Further, it is noted that deep brain stimulation in any brain target hitherto tried, and for any psychiatric or behavioural disorder, still remains at an investigational stage. Researchers are encouraged to design randomised controlled trials, based on scientific and data-driven rationales for disease and brain target selection. Experienced multidisciplinary teams are a mandatory requirement for the safe and ethical conduct of any psychiatric neurosurgery, ensuring documented refractoriness of patients, proper consent procedures that respect patient's capacity and autonomy, multifaceted preoperative as well as postoperative long-term follow-up evaluation, and reporting of effects and side effects for all patients. INTERPRETATION:This consensus document on ethical and scientific conduct of psychiatric surgery worldwide is designed to enhance patient safety.

Background: Most studies in the field of neurosurgical treatment for movement disorders have been published by a small number of leading centers in developed countries. This study aimed to investigate the clinical practice of stereotactic neurosurgery for Parkinson's disease (PD) worldwide. Methods: Neurosurgeons were contacted via e-mail to participate in a worldwide survey. The results obtained are presented in order of the countries' economic development according to the World Bank, as well as by the source of financial support. Results: A total of 353 neurosurgeons from 51 countries who had operated on 13,200 patients in 2009 were surveyed. Surgical procedures performed in high-income countries were more commonly financed by a public health care system. In contrast, in lower-middle-income and upper-middle-income countries, patients frequently financed surgeries themselves, and ablative surgeries were most commonly performed. Unexpectedly, ablative surgery is still used by about 65% of neurosurgeons, regardless of their country's economic status. Conclusions: This study provides a previously unavailable picture of the surgical aspects of PD across the globe in relation to health economics and sociodemographic factors. Global educational and training programs are warranted to raise awareness of economically viable surgical options for PD that could be adopted by public health care systems in lower-income countries.

Parkinson's disease (PD) is a neurodegenerative condition that can be pharmacologically treated with levodopa. However, important motor and nonmotor symptoms appear with its long-term use. The subthalamic nucleus (STN) is known to be involved in the pathophysiology of PD and to contribute to levodopa-induced complications. Surgery is considered in patients who have advanced PD that is refractory to pharmacotherapy and who display disabling dyskinesia. Deep brain stimulation of the STN is currently the main surgical procedure for PD, but lesioning is still performed. This review covers the clinical aspects and complications of subthalamotomy as one of the lesion-based options for PD patients with levodopa-induced dyskinesias. Moreover, the authors discuss the possible effects of subthalamic lesioning.

OBJECTIVE:Spinal cord stimulation (SCS) is a well-established treatment for neuropathic pain; nevertheless, 40% of patients fail to obtain satisfactory pain relief and in many patients, the effect tends to diminish with time. Based on animal experiments, intrathecal baclofen was previously introduced clinically to enhance suboptimal SCS effects. Later animal experiments demonstrated similar data for clonidine. The aim of this study was to elucidate whether intrathecal clonidine or baclofen enhances the effect of SCS in neuropathic pain patients in whom the pain relieving-effect of SCS is inadequate.METHODS:A randomized, double-blind, placebo-controlled clinical trial was conducted with 10 patients experiencing neuropathic pain with insufficient pain relief with SCS alone. Clonidine, baclofen, and saline (control) were intrathecally administered by bolus injections in combination with SCS.RESULTS:Seven of 10 patients reported significant pain reduction when SCS was combined with active drugs. The mean visual analog scale ratings were reduced by more than 50% with either drug combined with SCS. Four patients previously treated with SCS alone later underwent implantation of a pump for long-term administration of clonidine or baclofen. In the 2 patients with clonidine pumps with a mean follow-up of 15 months, the combined therapy produced pain reduction of 55% and 45%, respectively. The corresponding effect with baclofen was 32% and 82%, respectively, at 7 months follow-up.CONCLUSION:A trial with clonidine and baclofen combined with SCS may be warranted in patients who do not obtain satisfactory pain relief with SCS alone or experienced a decreasing therapeutic effect.

OBJECT:The aim of this study was to compare percutaneous balloon compression (PBC) and percutaneous retrogasserian glycerol rhizotomy (PRGR) in terms of effectiveness, complications, and technical aspects.METHODS:Sixty-six consecutive PBC procedures were performed in 45 patients between January 2004 and December 2008, and 120 PRGR attempts were performed in 101 patients between January 2006 and December 2008. The PRGR procedures were not completed due to technical reasons in 19 cases. Five patients in the Balloon Compression Group and 9 patients in the Glycerol Group were lost to follow-up and were excluded from the study. The medical records and the intraoperative fluoroscopic images from the remaining cases were retrospectively examined, and the follow-up was completed with telephone contact, when necessary. The 2 groups were compared in terms of initial effect, duration of effect, and rates of complications as well as severity and type of complications.RESULTS:The rates for immediate pain relief were 87% for patients treated with glycerol injection and 85% for patients treated with balloon compression. The Kaplan-Meier plots for the 2 treatment modalities were similar. The 50% recurrence time was 21 months for the balloon procedure and 16 months for the glycerol procedure. When the groups were broken down by the "previous operations" criterion, the 50% recurrence time was 24 months for the Glycerol First Procedure Group, 6 months for the Balloon First Procedure Group, 8 months for the Glycerol Previous Procedures Group, and 21 months for the Balloon Previous Procedures Group. The rates of complications (excluding numbness) were 11% for PRGR and 23% for PBC, and this difference was statistically significant (chi-square test, p = 0.04).CONCLUSIONS:Both PRGR and PBC are effective techniques for the treatment of trigeminal neuralgia, with PRGR presenting some advantages in terms of milder and fewer complications and allowing lighter anesthesia without compromise of analgesia. For these reasons the authors consider PRGR as the first option for the treatment of trigeminal neuralgia in patients who are not suitable candidates or are not willing to undergo microvascular decompression, while PBC is reserved for patients in whom the effect of PRGR has proven to be short or difficult to repeat due to cisternal fibrosis.