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Frontal circuits play a critical role in motor, cognitive and affective processing, and their dysfunction may result in a variety of brain disorders. However, exactly which frontal domains mediate which (dys)functions remains largely elusive. We studied 534 deep brain stimulation electrodes implanted to treat four different brain disorders. By analyzing which connections were modulated for optimal therapeutic response across these disorders, we segregated the frontal cortex into circuits that had become dysfunctional in each of them. Dysfunctional circuits were topographically arranged from occipital to frontal, ranging from interconnections with sensorimotor cortices in dystonia, the primary motor cortex in Tourette's syndrome, the supplementary motor area in Parkinson's disease, to ventromedial prefrontal and anterior cingulate cortices in obsessive-compulsive disorder. Our findings highlight the integration of deep brain stimulation with brain connectomics as a powerful tool to explore couplings between brain structure and functional impairments in the human brain.

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OBJECTIVE: The objective of this study was to analyze the trends in publications on intrathecal baclofen (ITB) therapy. METHODS: We searched Elsevier's Scopus database in February 2022 to find articles focused on ITB therapy. Data extracted included citation count, publication year, author's country and income category, journal and its 5-year impact factor, research type, disease requiring ITB, and target population. RESULTS: The analysis covered 615 articles from 1985 to 2022. The average citation count per article was 27.47 (95% confidence interval 23.75-31.18) and the mean impact factor was 4.54 (95% confidence interval 3.84-5.24). The majority (76.42%) were primary research, with 8.1% being interventional and 91.9% observational. Even so, one half of the top ten most cited were interventional. Secondary research and case reports made up 12.68% and 10.73% respectively, with narrative reviews making up most of the secondary research (79.48%). Only 1 study conducted a meta-analysis. The United States was the most prolific country. High-income countries published 96.42% of articles. CONCLUSIONS: The rising number of ITB articles and citations indicates growing interest and expanding knowledge in this field. However, there's a notable scarcity of research from low- and middle-income countries, particularly those with high prevalence of ITB-treatable diseases. The need for more evidence to overcome potential barriers to ITB implementation is emphasized. Despite an increasing number of publications, a large proportion presented low levels of evidence, such as case reports and narrative reviews, highlighting the need for more rigorous research methods to solidify the evidence base for ITB therapy.

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BACKGROUND: Cervical Dystonia ("CD") is a movement disorder characterised by sustained muscle contractions in the neck, causing involuntary posturing. Deep brain stimulation ("DBS") of the globus pallidal internus (GPi) is advanced treatment for pharmaco-refractory patients. As CD is a rare disease, cohort studies are often limited to patients of heterogenous disease profile, small sample size or short follow-up. This study firstly aimed to measure the efficacy of GPi-DBS on motor and non-motor symptoms of CD. A secondary aim was to evaluate if clinical factors - such as age, disease duration and baseline disease severity - influence variability of motor outcomes. METHODS: 37 idiopathic CD patients were recruited from movement disorders clinics at The Walton NHS Foundation Trust, Liverpool, UK. Patients were assessed pre-operatively, and 1 year, 3 years and 5 years post-operatively with the following clinical scales: Toronto Western Spasmodic Torticollis Rating Scale ("TWSTRS"), Hospital Anxiety and Depression Scale and EuroQuol-5D. RESULTS: GPI-DBS significantly improved overall TWSTRS scores by 57% from baseline to 5Y FU (p < 0.001). It also significantly improved TWSTRS severity, disability, and pain sub-scores by 72%, 59% and 46% respectively. We did not find a significant improvement in mood or quality of life scores at 5 years. Similarly, clinical factors at baseline did not correlate with variability in motor outcome. CONCLUSION: We concluded that GPi-DBS is an effective treatment for motor symptoms and pain in CD. There was limited effect on mood and QoL, and no clinical predictive factors of outcome were identified.

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Limited therapies are available for severe cerebral palsy children (CP) with complex movement disorders, especially when both dystonia and spasticity are present. In this publication, we present the improvement of a child with severe CP after intracerebroventricular baclofen therapy. The treatment can impact not just the movement disorders but also on the quality of life of the child and caregivers. Global functional improvements can be observed on the 6-month follow-up.

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Intramedullary tumors represent the major cause of spinal cord injuries, and its symptoms include pain and weakness. Progressive weakness may concomitantly occur in the upper and lower limbs, along with lack of balance, spine tenderness, sensory loss, trophic changes of extremity, hyperreflexia, and clonus. The study protocol was in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines. A systematic search of the MEDLINE electronic database was performed to identify the studies reporting the clinical features of children and adults who presented with an intramedullary lymphoma. Twenty-one studies were included, reporting 25 cases. Manuscripts were excluded if the full-text article was not available, original data were not reported (e.g., review articles), or if the main disease was not intramedullary lymphoma. A structured data extraction form was employed to standardize the identification and retrieval of data from manuscripts. To enlighten the discussion, a case is also presented. An 82-year-old woman with Fitzpatrick skin type II, diagnosed and treated for non-Hodgkin's lymphoma 7 years ago, was admitted with mental confusion and memory loss for the past 2 months-evolving with recurring falls from her own height. One day before admission, she displayed Brown-Séquard syndrome. An expansive lesion from C2 to C4 in the cervical spinal cord was found and a hypersignal spinal cord adjacent was described at the bulb medullary transition to the C6-C7 level. A primary spinal cord tumor was considered, as well as a melanoma metastasis, due to the lesion's flame pattern. The patient presented a partial recovery of symptoms and a reduction of the spinal cord edema after being empirically treated with corticosteroids, but the lesion maintained its extent. Subsequently, a large diffuse B-cell lymphoma with nongerminal center was found in open body biopsy, infiltrating neural tissue. The main objective of the present study is to report a surgical case treated for a large diffuse B-cell lymphoma, in addition to presenting the results of a systematic review of primary intramedullary spinal cord lymphoma.

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Frontal circuits play a critical role in motor, cognitive, and affective processing - and their dysfunction may result in a variety of brain disorders. However, exactly which frontal domains mediate which (dys)function remains largely elusive. Here, we study 534 deep brain stimulation electrodes implanted to treat four different brain disorders. By analyzing which connections were modulated for optimal therapeutic response across these disorders, we segregate the frontal cortex into circuits that became dysfunctional in each of them. Dysfunctional circuits were topographically arranged from occipital to rostral, ranging from interconnections with sensorimotor cortices in dystonia, with the primary motor cortex in Tourette's syndrome, the supplementary motor area in Parkinson's disease, to ventromedial prefrontal and anterior cingulate cortices in obsessive-compulsive disorder. Our findings highlight the integration of deep brain stimulation with brain connectomics as a powerful tool to explore couplings between brain structure and functional impairment in the human brain.

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Primary central nervous system lymphoma (PCNSL) is an uncommon lymphoproliferative disease associated with immunosuppression. Here, we report the case of a patient with multiple sclerosis, under treatment with fingolimod (FTY720, Gilenya) for 4 years, who developed this condition. Although the causal relationship cannot be established, there are cases in the literature that describe the appearance of lymphoma after the use of this medication. Considering the high mortality of PCNSL, epidemiological studies are necessary to establish a relationship between its arising and the use of immunosuppressants.

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BACKGROUND: Spinal cord stimulation (SCS) is traditionally performed by implanting surgical leads along the midline of the spinal cord, over the dorsal columns. Here, we present a patient who successfully underwent lateral cervical SCS to treat chronic refractory neuropathic pain. METHODS: A 46-year-old female, with a schwannoma involving the right axillary nerve, presented with a chronic refractory right upper extremity pain syndrome. The tumor was located between the fibers of the teres minor and the posterior deltoid, and measured 2.2 cm in diameter. After 8 months of analgesics, opioids, physiotherapy, and acupuncture, the patient underwent surgery; however, the tumor was unresectable (i.e., due to significant adjacent vascular/neural structures). Three months later, she had a midline C6-C7 laminectomy for placement of a right-sided epidural SCS lead (i.e., containing 16 electrode contacts). RESULTS: Within 4 days following this SCS procedure, the patient's pain completely resolved; at 10 postoperative months, she still remains pain free. CONCLUSION: Lateral SCS at the C6-C7 level provided a safe and effective option for the relief of chronic neuropathic pain attributed to an unresectable schwannoma of the right axillary nerve in a 46-year-old female.

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Introduction Complex regional pain syndrome (CRPS) is a disease that causes intense pain mainly in the upper and lower limbs of the patients, impairing the quality of life of those affected by the syndrome. Its pathophysiology has not yet been fully discovered and described. Also, treatments need to advance in the search for pain relief in those affected by the disease. The present article aims to describe the pathophysiology of CRPS and, mainly, to quantitatively analyze the efficiency of new treatments against pain caused by the disease. Methods Several articles on clinical trials described in a table were included in the present study, and a systematic review of the effectiveness of current treatments was performed. Results A total of 29 articles from clinical trials were selected using the preselection criteria. Surgical treatments against CRPS had a 56.9% efficiency in reducing painful sensation, and conservative treatments against CRPS had a 40.82% efficiency in reducing pain sensation. Conclusion Complex regional pain syndrome is a disease that causes pain in patients and worsens the quality of life of those affected by it. The treatments are diverse, and their efficiencies vary from bad to excellent.

Introdução A síndrome dolorosa regional complexa (SDRC) é uma doença que causa dor intensa principalmente nos membros superiores e inferiores dos pacientes, prejudicando a qualidade de vida dos afetados pela síndrome. Sua fisiopatologia ainda não foi completamente descoberta e descrita. Ademais, tratamentos precisam avançar na busca do alívio da dor naqueles afetados pela doença. O objetivo do presente artigo é descrever a fisiopatologia da SDRC e, principalmente, analisar quantitativamente a eficiência dos novos tratamentos contra a dor causada pela doença. Métodos Foram incluídos no presente estudo diversos artigos sobre ensaios clínicos descritos em uma tabela e foi feita uma revisão sistemática sobre a eficiência dos tratamentos atuais. Resultados Foram selecionados 29 artigos de ensaios clínicos por meio do critério de pré-seleção. Tratamentos cirúrgicos contra a SDRC tiveram uma eficiência de 56,9% na redução da sensação dolorosa e os tratamentos conservadores contra a SDRC tiveram uma eficiência de 40,82% na redução da sensação dolorosa. Conclusão A SDRC é uma doença que causa dor nos pacientes e piora da qualidade de vida dos afetados por ela. Os tratamentos são diversos e suas eficiências variam de ruim a excelente.

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Surgical resection of the insula (insulectomy) is a procedure used for brain lesions and for refractory epilepsy. It has a difficult surgical access and the need of a wide anatomical knowledge and preoperative planning. There are two types of surgical approaches aiming the exposure of the insular cortex: transsylvian and transcortical. The importance of insulectomies is the efficacy in providing a remarkable decrease in seizures. The objective of the present article is to document the results of a series of 10 patients submitted to insulectomies for refractory epilepsies and compare them with the results of other studies reported in the literature, as well as to describe the main nuances of the surgical approaches and their associated risks. In the new case series, all patients corresponded to preoperative Engel classification IV for; after a mean 2-year follow-up period, they corresponded to Engel classification II. A subtotal resection was performed in six patients, and the remaining four underwent a partial resection, most of them leading to temporary complications. The literature review endorsed the good outcomes of the casuistry. A critical analysis of the presented data reiterates the opinion of several authors that insulectomies are beneficial and safe for the patients. A broad anatomical knowledge of the insular region, preoperative planning (limits of resections), and the use of modern microsurgical techniques must be considered as basic principles by neurosurgeons for the prevention of perioperative morbidities. Insulectomies are safe and effective, although they result in temporary postoperative complications, and provide highly satisfactory results in terms of seizure control.

A ressecção cirúrgica da ínsula (insulectomia) é um procedimento utilizado para lesões cerebrais e epilepsia refratária. A ínsula possui um acesso cirúrgico difícil com necessidade de um amplo conhecimento anatômico com planejamento pré-operatório. Existem dois tipos de abordagens cirúrgicas que visam a exposição do córtex insular: transsilvianas e transcorticais. A importância das insulectomias é a eficácia em proporcionar uma diminuição das convulsões. O objetivo do presente artigo é documentar os resultados de uma série de 10 pacientes submetidos a insulectomias para epilepsia refratária e compará-los com os resultados de outros estudos relatados na literatura, além de descrever as principais nuances das abordagens cirúrgicas e os seus riscos associados. Na série de casos, todos os pacientes se enquadravam na classificação pré-operatória de Engel IV e, após um período médio de seguimento de 2 anos, eles se enquadravam na classificação de Engel II. Seis pacientes foram submetidos a uma ressecção subtotal e os quatro restantes a uma ressecção parcial, implicando, majoritariamente, em complicações temporárias. A revisão da literatura endossou os bons resultados da casuística. A análise crítica dos dados apresentados reitera a opinião de vários autores de que as insulectomias são benéficas e seguras para os pacientes. O amplo conhecimento anatômico da região insular, o planejamento pré-operatório (limites das ressecções) e a utilização de técnicas microcirúrgicas modernas devem ser considerados princípios básicos para a prevenção de morbidades perioperatórias. As insulectomias são seguras e eficazes conquanto resultem em complicações pós-operatórias temporárias e proporcionem resultados altamente satisfatórios no que diz respeito ao controle das convulsões.

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BACKGROUND: Skin erosion is a common complication after deep brain stimulator procedures. Despite being a relatively common event, there is no standard surgical technique or a widely accepted guideline for managing this kind of complication. METHODS: We describe a case of cutaneous erosion in the connector's site of deep brain stimulation case, surgically managed with anterior displacement of the connectors and overlapping and wrapping the connections within the temporal muscle. RESULTS: Postoperatively, the patient did well and achieved complete resolution of the skin erosion, with no signs of infection or new skin lesions. CONCLUSION: This technique demonstrated to be effective in this case in the long-term follow-up.

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BACKGROUND: A spinal intramedullary abscess is a rare clinical entity in which patients classically present with a subacute myelopathy and progressive paraplegia, sensory deficits, and/or bowel and bladder dysfunction. We report the second case of spinal intramedullary abscess caused by Candida albicans to ever be published and the first case of its kind to be surgically managed. CASE DESCRIPTION: A 44-year-old female presented with severe lumbar pain associated with paraparesis, incontinence, and paraplegia. She reported multiple hospital admissions and had a history of seizures, having already undergone treatment for neurotuberculosis and fungal infection of the central nervous system unsuccessfully. Nevertheless, no laboratory evidence of immunosuppression was identified on further investigation. Magnetic resonance imaging showed a D10-D11, well-circumscribed, intramedullary mass within the conus, which was hypointense on T1-weighted imaging and hyperintense on T2/STIR weighted. The patient underwent surgery for removal and biopsy of the lesion, which provided the diagnosis of an intramedullary abscess caused by C. albicans, a very rare condition with only one case reported in literature so far. CONCLUSION: C. albicans intramedullary abscess is a very rare clinical entity, especially in immunocompetent patients. We highlight C. albicans as an important etiology that must be considered in differential diagnosis. Critical evaluation of every case, early diagnosis, timely referral and surgical management of the abscess is essential to improve neurological outcome.

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BACKGROUND: Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytic proliferative disorder classically as a massive cervical lymphadenopathy. However, over the years, extranodal locations were confirmed with the central nervous system involvement in less than 5% of cases, which is marked as a significant differential diagnosis of meningiomas, with which they are widely confused due to the similarity of their radiological images. CASE DESCRIPTION: We report a 37-year-old man and 45-year-old man who were diagnosed with intracranial RDD but whose radiological images mimic meningiomas, requiring anatomopathological and tumor's immunohistochemistry for definitive diagnosis. Moreover, a review of 184 publications with 285 cases of intracranial involvement of this disease was also performed, comparing these findings with those brought in the previous studies. CONCLUSION: Intracranial Rosai-Dorfman tumors should always be remembered as differential diagnosis of meningiomas since they are similar radiologically and macroscopically. Once remembered and diagnosed, the lesion must be treated following the same pattern of resection done in meningiomas and, treatment's differences will not occur in the surgical excision technique, but in complementary chemotherapy implementation, radiotherapy, and even with radiosurgery aid, depending on the case. Thus, it is possible to obtain better results than with just the isolated surgical procedure.

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BACKGROUND: Lead migration is a complication associated with occipital nerve stimulation (ONS). We present a rare case in which fibrosis in the stress relief loop caused lead migration in the treatment of occipital neuralgia. CASE DESCRIPTION: A 30-year-old woman with a 5-year history of refractory occipital neuralgia, who had been under ONS therapy for 2 months, presented with a sudden onset of typical occipital neuralgia pain associated with cervical muscles spasms and myoclonus. A skull radiography showed lead migration. The patient underwent surgery for lead repositioning. During surgery, we identified extensive fibrosis throughout the stress relief loop that produced several constriction points. The fibrosis in the stress relief loop increased tension on the lead during head-and-neck movement, causing progressive migration of the lead. CONCLUSION: Although lead migration is a common complication of ONS, its association with fibrosis in the stress relief loop has not, to the best of our knowledge, been reported before. Lead migration can directly affect treatment outcome and it is, therefore, important to fully understand the possible mechanisms that can cause it and how to promptly manage them.

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BACKGROUND: The treatment of neuropathic pain (NP) continues to be controversial as well as an economic health issue and a challenge to health care. Neurosurgery can offer different methods of neuromodulation that may improve patients' condition, including deep brain stimulation (DBS), motor cortex stimulation (MCS), spinal cord stimulation (SCS), and posterior insula stimulation (PIS). There is no consensus of opinion as to the final effects of these procedures, which stimulation parameters to select, the correct timing, or how to select the patients who will best benefit from these procedures. OBJECTIVE: To review the evidence available regarding these 4 procedures and the management of NP. METHODS: We conducted a PubMed, Embase, and Cochrane Library database search from 1990 to 2020. The strategy of the search concentrated on the following keywords: "neuropathic pain," "chronic pain," "deep brain stimulation," "motor cortex stimulation," "spinal cord stimulation," "insula stimulation," and "neuromodulation." Studies that provided data regarding the immediate and long-term effectiveness of the procedure, anatomic stimulation target, percentage of pain control, and cause of the NP were included. RESULTS: The most frequent causes of NP were phantom limb pain and central poststroke pain in the MCS group; central poststroke pain, phantom limb pain, and spinal cord injury (SCI) in the DBS group; and complex regional pain syndrome and failed back surgery syndrome in the SCS group. Pain improvement varied between 35% and 80% in the MCS group and 50% and 60% in the DBS group. In the SCS group, successful rates varied between 38% and 89%. CONCLUSIONS: This systematic review highlights the literature supporting SCS, DBS, MCS, and PIS methods for the treatment of NP. We found consistent evidence supporting MCS, DBS, and SCS as possible treatments for NP; however, we were not able to define which procedure should be indicated for each cause. Furthermore, we did not find enough evidence to justify the routine use of PIS. We conclude that unanswered points need to be discussed in this controversial field and emphasize that new research must be developed to treat patients with NP, to improve their quality of life.

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Study Design Case report and review of the literature. Objective To present an unusual case of conus medullaris ependymal cyst. Methods This is a case of a 41-year-old woman with a diagnosis of ependymal cyst who presented with a history of shock-like pain to the lower limbs. Results The patient underwent a successful cyst decompression. Conclusion A few cases have been reported in the literature. Including this case, there are a total of eight cases of ependymal cyst in the conocaudal segment reported.

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OBJECTIVE: The occurrence of psychiatric comorbidity among individuals with crack or inhalant dependence is frequently observed. The objective of this study was to investigate anxiety symptoms among crack cocaine and inhalant users in southern Brazil. METHODS: The study investigated two groups of volunteers of equal size (n = 50): one group consisted of crack cocaine users, and the other group consisted of inhalant users. Research volunteers completed the Portuguese versions of the State-Trait Anxiety Inventory (STAI), Hamilton Anxiety Rating Scale (HAM-A), and Self-Report Questionnaire (SRQ). RESULTS: Both crack and inhalant users experience significant symptoms of anxiety. Inhalant users presented significantly more anxiety symptoms than crack users according to the HAM-A questionnaire only. In contrast to the results of the HAM-A, the STAI failed to demonstrate a significant difference between the two groups of substance users. SRQ scores revealed that crack and inhalants users had significant degrees of morbidity. CONCLUSION: A significant difference regarding anxiety symptomatology, especially state anxiety, was observed among inhalant and crack users. Anxiety and overall mental psychopathology were significantly correlated in this sample. The results indicate that screening initiatives to detect anxiety and additional psychiatric comorbidities among crack and inhalant users are feasible and relevant.

OBJETIVO: Investigar sintomas de ansiedade em usuários de crack e inalantes no Sul do Brasil. MÉTODOS: Oestudo investigou dois grupos de voluntárioscomomesmonúmero de indivíduos (n = 50): um grupo consistiu de usuários de crack e o outro grupo, de usuários de inalantes. Os voluntários da pesquisa completaram as versões em português das escalas de ansiedade State-Trais Anxiety Inventory (STAI) e Hamilton Anxiety Rating Scale (HAM-A), e o Questionário de Autoavaliação (SRQ). RESULTADOS: Ambos os grupos apresentaram sintomas significativos de ansiedade. Os usuários de inalantes apresentaram escores quanto a HAM-A significativamente superiores aos usuários de crack. Ansiedade e psicopatologia geral se apresentaram significativamente correlacionados. CONCLUSÃO: Sintomas de ansiedade são frequentes entre usuários de crack e inalantes. Um processo de triagem de sintomas de ansiedade entre usuários de crack e inalantes deve facilitar ainda mais as intervenções terapêuticas nesta área.

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OBJECTIVE: The occurrence of psychiatric comorbidity among individuals with crack or inhalant dependence is frequently observed. The objective of this study was to investigate anxiety symptoms among crack cocaine and inhalant users in southern Brazil. METHODS: The study investigated two groups of volunteers of equal size (n=50): one group consisted of crack cocaine users, and the other group consisted of inhalant users. Research volunteers completed the Portuguese versions of the State-Trait Anxiety Inventory (STAI), Hamilton Anxiety Rating Scale (HAM-A), and Self-Report Questionnaire (SRQ). RESULTS: Both crack and inhalant users experience significant symptoms of anxiety. Inhalant users presented significantly more anxiety symptoms than crack users according to the HAM-A questionnaire only. In contrast to the results of the HAM-A, the STAI failed to demonstrate a significant difference between the two groups of substance users. SRQ scores revealed that crack and inhalants users had significant degrees of morbidity. CONCLUSION: A significant difference regarding anxiety symptomatology, especially state anxiety, was observed among inhalant and crack users. Anxiety and overall mental psychopathology were significantly correlated in this sample. The results indicate that screening initiatives to detect anxiety and additional psychiatric comorbidities among crack and inhalant users are feasible and relevant.

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OBJECTIVE: The aim of this study was to demonstrate the validity and reliability of the Portuguese version of the Quality of Life Enjoyment and Satisfaction Questionnaire (Q-LES-Q) among substance users in southern Brazil. METHODS: The original English version of the Q-LES-Q was translated into Portuguese taking into account semantic and conceptual equivalence. The Portuguese versions of the Q-LES-Q and the World Health Organization Quality of Life Instrument (WHOQOL-BREF) were used to assess quality of life among crack cocaine and inhalant users. Factorial and reliability analyses as well as correlation studies were conducted to establish the psychometric properties of the Portuguese version of the Q-LES-Q. RESULTS: All areas of the Q-LES-Q achieved significant Cronbach's coefficients. Significant Pearson correlation coefficients were obtained between various subscales of the Q-LES-Q and the four dimensions of the WHOQOL-BREF. Correlations were significant when crack and inhalant users were analyzed separately or conjointly. Inhalant users presented significantly higher scores than crack users in the social (p=0.035) and general (p=0.005) subscales of the Q-LES-Q. CONCLUSION: The Portuguese version of the Q-LES-Q possesses sound internal validity. The English version of the Q-LES-Q was adequately adapted to Portuguese. Inhalant users may present lower levels of satisfaction with social life and overall quality of life than crack users. The Portuguese version of the Q-LES-Q is a reliable questionnaire for future research and clinical use with substance users in Brazil.

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