Dravet syndrome and Dravet syndrome-like phenotype: a systematic review of the SCN1A and PCDH19 variants.

Dravet syndrome (DS) is a rare and severe epileptic syndrome of childhood with prevalence between 1/22,000 and 1/49,900 of live births. Approximately 80% of patients with this syndrome present SCN1A pathogenic variants, which encodes an alpha subunit of a neural voltage-dependent sodium channel. There is a correlation between PCDH19 pathogenic variants, encodes the protocadherin 19, and a similar disease to DS known as DS-like phenotype. The present review aims to clarify the differences between DS and DS-like phenotype according to the SCN1A and PCDH19 variants. A systematic review was conducted in PubMed and Virtual Health Library (VHL) databases, using "Dravet Syndrome" and "Severe Myoclonic Epilepsy in Infancy (SMEI)" search words, selecting cohort of studies published in journal with impact factor of two or greater. The systematic review was according to the Preferred Reporting Items for Systematic Review and Meta-Analysis recommendations. Nineteen studies were included in the present review, and a significant proportion of patients with DS-carrying SCN1A was greater than patients with DS-like phenotype-harboring PCDH19 variants (76.6% versus 23.4%). When clinical and genetic data were correlated, autism was predominantly observed in patients with DS-like-carrying PCDH19 variants compared to SCN1A variant carriers (62.5% versus 37.5%, respectively, P-value = 0.044, P-value corrected = 0.198). In addition, it was noticed a significant predisposition to hyperthermia during epilepsy crisis in individuals carrying PCDH19 variants (P-value = 0.003; P-value corrected = 0.027). The present review is the first to point out differences between the DS and DS-like phenotype according to the SCN1A and PCDH19 variants.

Puerperal Extracranial Vertebral Artery Dissection and Nonaneurysmal Subarachnoid Hemorrhage.

Previously reported only a few times before, we present a case of extracranial vertebral dissection and spontaneous frontoparietal subarachnoid hemorrhage (SAH) in the puerperium, discussing possible mechanisms and difficulties in management. A 35-year-old woman presented 10 days postcaesarean section with neck pain and vertigo with normal initial investigations. Following recurrent vertigo, headache, and ataxia, imaging revealed a frontoparietal SAH and vertebral artery dissection. The patient was consequently treated with aspirin, and then following a return of symptoms 3 weeks later, warfarin therapy was continued for 6 months. The possible underlying mechanisms for this case are discussed, including reversible cervical vasoconstriction syndrome and posterior reversible encephalopathy syndrome, although neither was identified. The small SAH alongside recurrent posterior circulation symptoms resulted in the initiation of antithrombotic therapy. This report supports studies demonstrating higher incidence of cervicocephalic arterial dissection in the puerperium. Moreover, the heterogeneous presentation and manifestations of such cases require individualized treatment, and warrant studies into underlying mechanisms behind extracranial dissection and nonaneurysmal SAH.

Cerebrospinal fluid aquaporin-4 antibody levels in neuromyelitis optica attacks.

To elucidate immunopathogenetic roles of aquaporin-4 antibodies in the cerebrospinal fluid (CSF) of neuromyelitis optica spectrum disorders (NMOSD), we analyzed aquaporin-4 antibody titers, cellular and inflammatory markers in the CSF collected from 11 aquaporin-4 antibody seropositive patients. The CSF aquaporin-4 antibody levels during attacks (but not in sera) closely correlated with pleocytosis, inflammatory cytokines including interleukin-6 that can regulate antibody-producing plasmablasts, and glial fibrillary acidic protein levels in the CSF. The amount of aquaporin-4 antibodies present in the central nervous system may have therapeutic implications, as it is associated with astrocyte injury and inflammatory responses during NMOSD attacks.

Prospective factors of temporary arterial occlusion during anterior communicating artery aneurysm repair.

INTRODUCTION: This study was undertaken to determine variables that could predict, in the perioperative period of anterior communicating artery (ACom) aneurysms surgeries, the likelihood of postoperative sequelae and complications, after temporary arterial occlusion (TAO). PATIENTS AND METHODS: In a universe of 32 patients submitted to ACom aneurysm repair in the last 7 years, 21 needed TAO intraoperatively, and had their data examined retrospectively. RESULTS: Aneurysms larger than 7 mm were more likely to be treated with longer TAO time than small aneurysms, (p < 0.0001). There was no statistical correlation between time of occlusion and outcome. Age, Glasgow Coma Scale at initial evaluation, and Fisher scale at first CT scanning were independent factors of unfavorable outcome (p < 0.001). Meanwhile gender, tobacco addiction, obesity, arterial hypertension, dyslipidemia, location of TAO (A1 or A2), intraoperative rupture (IR) and the aneurysm size were not identified as independent prognostic factors.During follow-up period, two thirds of the patients had a favorable outcome, accomplishing normal daily life activities without major complications. Most patients developed clinical vasospasm (66.6 %), with 19 % of the patients harboring a severe disease. Delayed ischemic neurological deficit was observed in 28.5 %, without any statistical correlation to time of TAO or IR. CONCLUSION: TAO during ACom aneurysm repair does not seem to add more morbidities to the procedure, and is not an independent prognostic factor.

Transcranial Doppler as a routine in the treatment of vasospasm following subarachanoid hemorrhage (SAH).

BACKGROUND: Vasospasm is an important complication observed after subarachnoid hemorrhage (SAH) and is a frequent cause of mortality and morbidity. We present our routine management of vasospasm after SAH and emphasize the importance of transcranial Doppler (TCD) ultrasonography in this management. METHOD: Historical records and images were sampled from June 2005 to September 2011 for 110 patients with SAH due to ruptured aneurysm in the anterior circulation. All surviving patients were followed after discharge. Vasospasm was defined as mild (Lindegaard index 3-4), moderate (Lindegaard index 4-5), and severe (Lindegaard index greater than 5). We excluded patients treated after 72 h of symptom onset. TCD was performed twice per day. FINDINGS: Ninety-nine patients had surgical clipping of the aneurysm, and 11 had endovascular treatment. Seventy patients treated by clipping and six treated by endovascular procedure had vasospasm. Of the 70 clipped patients with vasospasm, 40 had mild vasospasm, 13 had moderate vasospasm, and 17 had severe vasospasm. All six patients treated by coils had moderate vasospasm. The average duration of vasospasm was 9 days (from 7 to 32 days). CONCLUSIONS: TCD was crucial for monitoring patients with SAH, and to identify which patients will have a higher risk of developing vasospasm.

Removal of clots in subarachnoid space could reduce the vasospasm after subarachnoid hemorrhage.

BACKGROUND: Cerebral vasospasm after subarachnoid hemorrhage (SAH) is a major cause of morbidity and mortality. We studied the effects of clot removal on multiple outcome variables following the clipping of ruptured anterior communicating aneurysms. METHODS: From 2007 to 2011, 30 patients with Fisher grade III aneurysmal SAH underwent clipping of an anterior communicating artery aneurysm before SAH day 3. There were 20 women and 10 men, mean age 53.4, range 28-80 years. Seventeen underwent fenestration of lamina terminalis and cisternal removal of clots (group A), and 13 did not (Group B). We compared clinical grades, presence of hydrocephalus at admission, treatment modality, occurrence of clinical vasospasm, the need for interventional vasospasm therapy, and need for ventriculoperitoneal shunting. FINDINGS: Vasospasm affected 5 of 17 (29%) in group A and 8 of 13 (61.5%) in group B (p < 0.05). Endovascular treatment for vasospasm was required in one patient in group A (5.8% of 17, 20% of 5) and in five from group B (38.4% of 13, 62.5% of 8) (p < 0.05). Mortality was observed in one case in group A (5.8% of 17, 20% of 5) and in two cases in group B (15.3% of 13, 25% of 8) and was related to vasospasm after SAH. Ventriculoperitonal shunt (VPS) was required in one case in group A (5.8%) and in five cases in group B (38.4%). CONCLUSIONS: Fenestration of the lamina terminalis and removal of cisternal clots significantly decreased the incidence of post-SAH hydrocephalus and was associated with better outcomes in our series.

Neuromodulation for Medically Refractory Neuropathic Pain: Spinal Cord Stimulation, Deep Brain Stimulation, Motor Cortex Stimulation, and Posterior Insula Stimulation.

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.

Long-term efficacy and safety of alemtuzumab in patients with RRMS: 12-year follow-up of CAMMS223.

BACKGROUND: In the phase 2 CAMMS223 trial (NCT00050778), alemtuzumab significantly improved clinical and MRI outcomes versus subcutaneous interferon beta-1a over 3 years in treatment-naive patients with relapsing-remitting MS. Here, we assess efficacy and safety of alemtuzumab over 12 years in CAMMS223 patients who enrolled in the CAMMS03409 extension (NCT00930553), with available follow-up through the subsequent TOPAZ extension (NCT02255656). METHODS: In CAMMS223, patients received 2 alemtuzumab courses (12 mg/day; baseline: 5 days; 12 months later: 3 days); 22% received a third course. In the open-label, nonrandomized extensions, patients could receive as-needed additional alemtuzumab or other disease-modifying therapies. RESULTS: Of 108 alemtuzumab-treated patients in CAMMS223, 60 entered the CAMMS03409 extension; 33% received a total of 2 alemtuzumab courses, and 73% received no more than 3 courses through Year 12. Over 12 years, annualized relapse rate was 0.09, 71% of patients had stable or improved Expanded Disability Status Scale scores, and 69% were free of 6-month confirmed disability worsening. In Year 12, 73% of patients were free of MRI disease activity. Cumulatively throughout the extensions (Years 7-12), 34% of patients had no evidence of disease activity. Adverse event (AE) incidence declined through Year 12. Infusion-associated reactions peaked at first course and declined thereafter. Cumulative thyroid AE incidence was 50%; one immune thrombocytopenia event occurred, and there were no autoimmune nephropathy cases. CONCLUSIONS: Alemtuzumab efficacy was maintained over 12 years in CAMMS223 patients, with 73% receiving no more than three courses. The safety profile in this cohort was consistent with other alemtuzumab clinical trials.

Pregnancy outcomes and postpartum relapse rates in women with RRMS treated with alemtuzumab in the phase 2 and 3 clinical development program over 16 years.

BACKGROUND: Relapsing-remitting multiple sclerosis (RRMS) is frequently diagnosed in women of reproductive age. Because the use of disease-modifying therapies (DMTs) early in the disease course is increasing, it is important to evaluate the safety of DMTs in pregnant women and their developing fetuses. Alemtuzumab, approved for the treatment of relapsing forms of MS, is administered as 2 courses of 12 mg/day on 5 consecutive days at baseline and on 3 consecutive days 12 months later. Alemtuzumab is eliminated from the body within approximately 30 days after administration; it is recommended that women of childbearing potential use effective contraception during and for 4 months after treatment. Here, we report pregnancy outcomes in alemtuzumab-treated women from the phase 2 and 3 clinical development program over 16 years. METHODS: We followed 972 women who had alemtuzumab in phase 2 (CAMMS223 [NCT00050778]) and phase 3 (CARE-MS I [NCT00530348], CARE-MS II [NCT00548405]) studies, and/or in 2 consecutive extension studies (NCT00930553; NCT02255656 [TOPAZ]). In the extension studies, patients could receive additional alemtuzumab (12 mg/day on 3 days; ≥12 months apart) as needed for disease activity. All women who received alemtuzumab in the clinical development program were included. Pregnant or lactating patients were followed up for safety. RESULTS: As of November 26, 2018, 264 pregnancies occurred in 160 alemtuzumab-treated women, with a mean age at conception of 32.6 years, and mean time from last alemtuzumab dose to conception of 35.9 months. Of the 264 pregnancies, 233 (88%) were completed, 11 (4%) were ongoing, and 20 (8%) had unknown outcomes; 16 (6%) conceptions occurred within 4 months, and 5 conceptions within 1 month of the last alemtuzumab dose. Of the 233 completed pregnancies with known outcomes, there were 155 (67%) live births with no congenital abnormalities or birth defects, 52 (22%) spontaneous abortions, 25 (11%) elective abortions, and 1 (0.4%) stillbirth. Maternal age was associated with an increased risk of spontaneous abortion in alemtuzumab-treated patients (<35 years: 15%; ≥35 years: 37%; relative risk [RR], 2.46 [95% CI: 1.53-3.95], p=0.0002). Risk of spontaneous abortion was not increased in patients becoming pregnant ≤4 months versus >4 months since alemtuzumab exposure (19% vs 23%; RR, 1.08 [95% CI: 0.41-2.85], p=0.88). Autoimmune thyroid adverse events did not increase risk for spontaneous abortion (patients with vs without thyroid adverse events, 23.7% vs 21.3%; RR, 1.11 [95% CI: 0.69-1.80], p=0.75). Annualized relapse rate was 0.10 and 0.12 in the 2 years prior to pregnancy (post alemtuzumab), and was 0.22, 0.12, and 0.12 in each of the first 3 years postpartum, respectively. CONCLUSION: Normal live births were the most common outcome in women exposed to alemtuzumab 12 mg or 24 mg in clinical studies. Spontaneous abortion rate in alemtuzumab-treated patients was comparable with rates in the general population and treatment-naive MS patients, and was not increased in women with pregnancy onset within 4 months of alemtuzumab exposure. There was a minimal increase in postpartum relapses.

Olfactory groove meningiomas: approaches and complications.

Olfactory groove meningiomas (OGM) account for 4.5% of all intracranial meningiomas. We report 21 patients with OGMs. Tumors were operated on using three surgical approaches: bifrontal (7 patients), fronto-pterional (11 patients) and fronto-orbital (3 patients). Total tumor removal (Simpson Grade 1) was achieved in 13 patients and Simpson II in 8 patients. Perioperative mortality was 4.76%. The average size of the OGM was 4.3+/-1.1cm. The overall recurrence rate was 19%. We preferred to use the pterional approach, which provides quick access to the tumor with less brain exposure. It also allows complete drainage of cisternal cerebrospinal fluid, providing a good level of brain relaxation during surgery. However, for long, thin tumors, hemostasis can be difficult using this approach.

Spinal Cord Stimulation in Pregnant Patients: Current Perspectives of Indications, Complications, and Results in Pain Control: A Systematic Review.

Spinal cord stimulation (SCS) has been described as a valuable neuromodulator procedure in the management of chronic medically untreated neuropathic pain. Although the use of this technique has been published in many papers, a question still remains regarding its applicability in pregnant patients. The goal of this paper is to discuss the risks, complications, and results as well as the prognosis of SCS in pregnant patients. We performed a systematic review from 1967 to 2018 using the databases MEDLINE, LILACS, SciELO, PubMed, and BIREME, utilizing language as selection criteria. Eighteen studies that met our criteria were found and tabulated. SCS is a reversible and adjustable surgical procedure, which results in patients that demonstrated a significant effect in the reduction of pain intensity in pregnant patients. The etiologies most frequent were complex regional pain and failed back pain syndromes, which together represented 94% of analyzed cases. The technical complications most frequent were lead migration (3%, n = 1). Regarding the risks, the authors did not show significative factors among the categorical variables that can suggest a teratogenicity, while the maternal risks have been associated to the consequences of technical complications due to, among other factors, improvement of abdominal pressure during pregnancy and delivery. Finally, although there are not significative cohorts of pregnant patients, the procedure is still an effective surgical approach of neuropathic pain associated to lower rates of complications and significative improvement in the quality of life of patients during pregnancy.

Brazilian Consensus for the Treatment of Multiple Sclerosis: Brazilian Academy of Neurology and Brazilian Committee on Treatment and Research in Multiple Sclerosis.

The expanding therapeutic arsenal in multiple sclerosis (MS) has allowed for more effective and personalized treatment, but the choice and management of disease-modifying therapies (DMTs) is becoming increasingly complex. In this context, experts from the Brazilian Committee on Treatment and Research in Multiple Sclerosis and the Neuroimmunology Scientific Department of the Brazilian Academy of Neurology have convened to establish this Brazilian Consensus for the Treatment of MS, based on their understanding that neurologists should be able to prescribe MS DMTs according to what is better for each patient, based on up-to-date evidence and practice. We herein propose practical recommendations for the treatment of MS, with the main focus on the choice and management of DMTs, as well as present a review of the scientific rationale supporting therapeutic strategies in MS.

A disturbed processing of graviceptive pathways may be involved in the pathophysiology of balance disorders in patients with multiple sclerosis.

The purpose of this study was to determine the relationship between perception of verticality and balance disorders in multiple sclerosis patients. We evaluated patients and healthy controls. Patients were divided into two groups according to their risk of fall, with or without risk of fall, measured by a Dynamic Gait Index scale. Graviceptive perception was assessed using the subjective visual vertical test. Patients with risk of fall showed worse perception than those without risk of fall, p < 0.001. Misperception of verticality was correlated with the dynamic gait index scores (p < 0.001), suggesting that the larger the error for verticality judgment, the greater risk for falling. Considering that the perception of verticality is essential for postural control, our results suggested that the disturbed processing of graviceptive pathways may be involved in the pathophysiology of balance disorders in these patients.

Alternatives for reducing relapse rate when switching from natalizumab to fingolimod in multiple sclerosis.

Natalizumab is a therapeutic option for treating multiple sclerosis (MS) and is particularly efficacious for patients with highly active disease. A long washout period has been recommended between withdrawal of natalizumab and start of fingolimod (another option for treating MS). This long washout period has been associated with a significant increase in MS activity. In the present study, a group of 96 patients who were switched from natalizumab to fingolimod had short washout periods between drugs, or monthly corticosteroid pulse therapy if longer washout periods were recommended. This therapeutic approach led to the lowest reported relapse rate so far, among patients with MS switching from natalizumab to fingolimod (8.3%). No complications from short withdrawal were observed in this group of patients.

Natalizumab treatment in multiple sclerosis: the experience from two Brazilian MS centers.

Objective Analyze the demographics, clinical characteristics, efficacy and safety of natalizumab treatment in Brazilian patients with multiple sclerosis (MS) followed up for at least 12 months, in two tertiary MS care centers in São Paulo.Method We evaluated the effect of natalizumab treatment on annualized relapse rate and disability progression in 75 patients with MS treated with natalizumab for at least 12 months. A subgroup analysis was performed to evaluate efficacy of natalizumab treatment in patients with Expanded Disability Status Scale (EDSS) ≤ 3.0 vs patients with EDSS > 3.Results Patients treated for at least one year with natalizumab showed a 91% reduction in aRR, as well and an improvement in neurological disability. The impact of natalizumab treatment was greater in patients with EDSS < 3.0. Overall, natalizumab was safe but one patient developed progressive multifocal leukoencephalopathy.Conclusion Natalizumab as a third line therapy is safe and efficacious, especially in patients with mild neurological disability.

Distinction between MOG antibody-positive and AQP4 antibody-positive NMO spectrum disorders.

OBJECTIVE: To evaluate clinical features among patients with neuromyelitis optica spectrum disorders (NMOSD) who have myelin oligodendrocyte glycoprotein (MOG) antibodies, aquaporin-4 (AQP4) antibodies, or seronegativity for both antibodies. METHODS: Sera from patients diagnosed with NMOSD in 1 of 3 centers (2 sites in Brazil and 1 site in Japan) were tested for MOG and AQP4 antibodies using cell-based assays with live transfected cells. RESULTS: Among the 215 patients with NMOSD, 7.4% (16/215) were positive for MOG antibodies and 64.7% (139/215) were positive for AQP4 antibodies. No patients were positive for both antibodies. Patients with MOG antibodies represented 21.1% (16/76) of the patients negative for AQP4 antibodies. Compared with patients with AQP4 antibodies or patients who were seronegative, patients with MOG antibodies were more frequently male, had a more restricted phenotype (optic nerve more than spinal cord), more frequently had bilateral simultaneous optic neuritis, more often had a single attack, had spinal cord lesions distributed in the lower portion of the spinal cord, and usually demonstrated better functional recovery after an attack. CONCLUSIONS: Patients with NMOSD with MOG antibodies have distinct clinical features, fewer attacks, and better recovery than patients with AQP4 antibodies or patients seronegative for both antibodies.

Brazilian Consensus for the Treatment of Multiple Sclerosis: Brazilian Academy of Neurology and Brazilian Committee on Treatment and Research in Multiple Sclerosis / Consenso Brasileiro para o Tratamento da Esclerose Múltipla: Academia Brasileira de Neurologia e Comitê Brasileiro de Tratamento e Pesquisa em Esclerose Múltipla

ABSTRACT The expanding therapeutic arsenal in multiple sclerosis (MS) has allowed for more effective and personalized treatment, but the choice and management of disease-modifying therapies (DMTs) is becoming increasingly complex. In this context, experts from the Brazilian Committee on Treatment and Research in Multiple Sclerosis and the Neuroimmunology Scientific Department of the Brazilian Academy of Neurology have convened to establish this Brazilian Consensus for the Treatment of MS, based on their understanding that neurologists should be able to prescribe MS DMTs according to what is better for each patient, based on up-to-date evidence and practice. We herein propose practical recommendations for the treatment of MS, with the main focus on the choice and management of DMTs, as well as present a review of the scientific rationale supporting therapeutic strategies in MS.

RESUMO O crescent arsenal terapêutico na esclerose múltipla (EM) tem permitido tratamentos mais efetivos e personalizados, mas a escolha e o manejo das terapias modificadoras da doença (TMDs) tem se tornado cada vez mais complexos. Neste contexto, especialistas do Comitê Brasileiro de Tratamento e Pesquisa em Esclerose Múltipla e do Departamento Científico de Neuroimunologia da Academia Brasileira de Neurologia reuniram-se para estabelecer este Consenso Brasileiro para o Tratamento da EM, baseados no entendimento de que neurologistas devem ter a possibilidade de prescrever TMDs para EM de acordo com o que é melhor para cada paciente, com base em evidências e práticas atualizadas. Por meio deste documento, propomos recomendações práticas para o tratamento da EM, com foco principal na escolha e no manejo das TMDs, e revisamos os argumentos que embasam as estratégias de tratamento na EM.

Hemispherectomy in treatment of seizures: current perspectives of the indications, techniques and complications: literature review

Introducción: A hemisferectomía es un procedimiento valioso en el tratamiento de trastornos convulsivos causados por desordenes hemisféricos unilaterales. El hemisferectomía anatómica se ha utilizado para este fin desde 1938, sin embargo, se abandonó este procedimiento después de informes de complicaciones postoperatorias causadas por hemosiderosis superficial, ependimitis e hidrocefalia obstructiva. Así que, se ha mostrado en la literatura modificaciones en las indicaciones y técnicas de hemisferectomía anatómica cuya finalidad es la de reducir la incidencia de esta complicación sin dejar de lograr control de las convulsiones. Sobre la base de la literatura, la hemisferectomía mejora la calidad de vida de los pacientes que tiene la indicación para realizar este procedimiento, ya que permite reducir la frecuencia de las convulsiones, si tónica o átona, tónico-clónicas Objetivo: El objetivo de esta revisión de la literatura es discutir los detalles técnicos, modalidades, riesgos, complicaciones, resultados y de pronóstico de hemisferectomía basado en la revisión crítica de la literatura. Casuística y Métodos: Se realizó la consulta bibliográfica, utilizando la base de datos MEDLINE, LILACS, SciELO, que utiliza el lenguaje como criterios de selección, la elección de los artículos recientes preferiblemente en portugués, español o inglés. Conclusión: Según las referencias, hemisferectomía es un procedimiento con buen resultado para las personas con convulsiones derivadas cuando está indicado para casos seleccionados y la tasa de éxito no es proporcional a la extensión de la resección del tejido neuronal. A mayor resección puede o no reducir la frecuencia de las crisis, sin embargo, la incidencia de la morbilidad puede ser mayor.

Background: The hemispherectomy is a valuable procedure in the management of seizure disorders caused by unilateral hemispheric disease. The anatomical hemispherectomy has been used for this purpose since 1938, however, it was abandoned after reports of postoperative complications caused by superficial hemosiderosis, ependymitis and obstructive hydrocephalus. So that, it has been showed modifications in the techniques of hemispherectomy whose the purpose is reduce the incidence of this complications while still achieving seizure control. Based on literature, the hemispherectomy improves the quality of life of patients that has the indication to perform this procedure because it allows reducing the frequency of seizures, whether tonic or atonic, tonic-clonic. Aim: The aim of this literature review is discuss the indications, technical details, modalities, risks, complications, results as well de prognosis of callosotomy based on critical literature review and the authors experience. Casuistry and Methods: It was performed bibliographical consultation, using the databases MEDLINE, LILACS, SciELO, utilizing language as selection criteria, choosing preferably recent articles in Portuguese, Spanish or English. Conclusion: According to references, the functional hemispherectomy has a good outocome for those with seizures arisin when indicated to selected cases and the success rate is not proportional to the extent of neuronal tissue resection. So that, a greater resection cannot necessarily reduce the seizure frequency, however the morbidity may also be larger.

Complex regional pain syndrome: new concepts regarding diagnosis and treatment

Antecedentes: Los autores presentan una revisión crítica sobre el cuadro clínico, el diagnóstico, clasificación y tratamientodel síndrome de dolor regional complejo, discutiendo todos los métodos de tratamiento y haciendo hincapié en que la reabilitación debe ser empleada con el fin de obtener un mejor resultado. Aspecto psicológico debe ser discutido en el tratamiento y también se anima equipo multidisciplinario para participar en él.

Background: The authors presented a critical review about the clinical picture, diagnosis, classification and treatment ofcomplex regional pain syndrome, discussing all methods of treatment and emphasizing that the reabiltation must be employed in order to obtain a better result. Psychological aspect must be involved in the treatment and also multidisciplinary team is encouraged to take part on it.

Targets for treatment of dystonia caused by several etiologies. Meta analysis

Introducción: Para utilizar un meta-análisis de todos los casos reportados de la estimulación cerebral profunda (DBS) para ladistonía para determinar cuáles son los factores significativos resultados influencia relacionada con el destino. La escala demovimiento Burke-Fahn-Marsden (BFM), la medida más informado, fue elegida como la principal medida de resultado paraeste análisis. Material y Métodos: Una búsqueda en MEDLINE identificaron 137 pacientes que se sometieron a DBS para ladistonía en 24 estudios que tenían puntuaciones individuales BFM. Datos de los pacientes individuales, incluyendo la edad deinicio de la distonía, la edad de la cirugía, el género, la distribución de la distonía, la etiología de la distonía, la presencia decaracterísticas asociadas, anormalidad de las imágenes preoperatorias, cirugías estereotáxica anteriores, el núcleo estimulado,el tipo de anestesia que se utiliza, el tiempo de respuesta a la estimulación, y el momento de la evaluación de resultadosse introdujeron en una base de datos de SPSS para el análisis estadístico. Resultados: La media BFM cambio porcentual(mejora en la puntuación postoperatoria de la línea de base) fue 51,8% (rango - 34% a 100%). Significativamente se lograronmejores resultados con la estimulación del globo pálido interno (GPI) que con la estimulación de la parte posterior del núcleolateral ventral (VLP) del tálamo (p = 0,0001)...

Introduction: To use a meta-analysis on all reported cases of deep brain stimulation (DBS) for dystonia to reevaluate the good effect using the GPi as a target, which factors significant influence outcome related to the target. The Burke-Fahn-Marsden (BFM) movement scale, the most reported measure, was chosen as the primary outcome measure for this analysis. Material and Methods: Computerized MEDLINE searches on English literature search identified 137 patients who underwent BBS for dystonia in 24 studies that had individual BFM scores. The study was done with statistical analysis by intention to treat. Statistical analysis was made with a significant p- value of 0.05. For the comparison of pre- and postoperative scores, a test Wilcoxon signed was used. Results: The mean BFM percentage change (improvement in postoperative score from baseline) was 46.3 percent(range - 34 percent to 100 percent)...