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The hematological manifestations of systemic lupus erythematous (SLE) are causes of morbidity and increased risk of mortality. Young patient, female, with SLE and antiphospholipid syndrome (APS) had severe pancytopenia after urinary tract infection. A biopsy of the bone marrow (BM) showed necrosis and fibrosis. The most common pathophysiological mechanism for pancytopenia is the production of peripheral antibodies. However, pancytopenia with BM aplasia or necrosis is rare. BM necrosis is more common with neoplastic diseases, severe infections or sickle cell anemia but is also reported for patients with SLE. It is seen more rarely in patients with primary APS. Changes in the BM microcirculation lead to ischemia and subsequent necrosis. The main complications are pancytopenia and embolism. BM necrosis has been appointed in few clinical studies as a possible cause for pancytopenia in SLE patients. Among the findings, BM necrosis was present in 19% of the patients. BM necrosis is a relatively rare and poor prognosis entity.

The hematological manifestations of systemic lupus erythematous (SLE) are causes of morbidity and increased risk of mortality. Young patient, female, with SLE and antiphospholipid syndrome (APS) had severe pancytopenia after urinary tract infection. A biopsy of the bone marrow (BM) showed necrosis and fibrosis. The most common pathophysiological mechanism for pancytopenia is the production of peripheral antibodies. However, pancytopenia with BM aplasia or necrosis is rare. BM necrosis is more common with neoplastic diseases, severe infections or sickle cell anemia but is also reported for patients with SLE. It is seen more rarely in patients with primary APS. Changes in the BM microcirculation lead to ischemia and subsequent necrosis. The main complications are pancytopenia and embolism. BM necrosis has been appointed in few clinical studies as a possible cause for pancytopenia in SLE patients. Among the findings, BM necrosis was present in 19% of the patients. BM necrosis is a relatively rare and poor prognosis entity.

INTRODUCTION: Patients with idiophatic generalized epilepsy (IGE) may occasionally have frequent generalized tonic-clonic seizures (GTCS) which are not adequantely controlled by antiepileptic drugs. Frequent GTCS pose a significant risk of injury and other complications. In symptomatic generalized epilepsies, corpus callosotomy (CC) has been shown to be effective in reducing the number of generalized seizures. OBJECTIVE: We report a patient with refractory, weekly GTCS in the context of a primary generalized epilepsy syndrome who underwent subtotal CC. RESULT: In the 3 months since operation, no GTCS occurred. CONCLUSION: Corpus callosotomy can be helpful in reducing medically-refractory GTCS, even in patients with primary generalized epilepsies.

Patients with temporal lobe epilepsy due to hippocampal sclerosis (TLE/HS) have a distinct neuropsychological profile, but there is still debate on whether executive dysfunction is part of this profile and also whether temporal lobe surgery can modify this dysfunction. Objective: To study the presence and reversibility of executive dysfunction in patients with unilateral TLE/HS. Methods: Twenty-five patients with refractory seizures due to TLE/HS underwent presurgical evaluation which included the application of the Wiconsin Card Sorting Test (WCST). Nineteen were re-evaluated in follow up, at least 6 months after selective amygdalo-hippocampectomy (SAH). Twenty-two control subjects matched for age and education also performed the WCST. Results: Sixteen of the 25 patients (64%) completed fewer than four categories in the WCST whereas only 4 of the 22 controls (18%) did not complete at least four categories (p

Object The aim of this study was to compare seizure and memory outcome in patients with medically refractory mesial temporal lobe epilepsy due to hippocampal sclerosis (MTLE/HS) treated using an anterior temporal lobectomy (ATL) or a selective amygdalohippocampectomy (SA). Methods Surgical outcome data were prospectively collected for 2 to 11 years in 161 consecutive patients with MTLE/HS. Eighty patients underwent an ATL and 81 an SA. Seizure control achieved with each technique was compared using the Engel classification scheme. Postoperative memory testing was performed in 86 patients (53%). At the last follow up, 72% of the patients who had undergone an ATL (mean follow up 6.7 years) and 71% of those who had undergone an SA (mean follow up 4.5 years) were seizure free (Engle Class IA). Estimated survival in patients in Engel Classes I, IA, and I and II combined did not differ between the two surgical techniques. Preoperatively, 58% of the patients had verbal memory scores one standard deviation (SD) below the normal mean. One third of the patients with preoperative scores in the normal range worsened after surgery, although this outcome was not related to the surgical technique. In contrast, one third of those whose preoperative scores were less than −1 SD experienced improvement after surgery. Nine (18%) of the 50 patients whose left side had been surgically treated improved their verbal memory scores by more than one SD. Seven (78%) of these nine underwent an SA (p = 0.05). Conclusions Both ATL and SA can lead to similar favorable seizure control in patients with MTLE/HS. Preliminary data suggest that postoperative verbal memory scores may improve in patients who undergo selective resection of a sclerotic hippocampus in the dominant temporal lobe.

Grandes avanços tecnológicos tem ocoíido nas tecnicas de avaliação píé-ciÍúrgica de pacientes com epilepsia refratária. Coín O intuito de del'-ilar a zona epileotogènrca (/.e. a regiào co(icalduja remoção e necessária e suticiente para o conlÍole das crises), devê,se saber InterpretaÍ e hierarquizar os dados oblidos na 'nvesligação. já que esles nem sempre sáo comolelamêrle convergentes. lsso Íaz a necessidade de que se âvance Ìambem conceitualmenle. pati pasu com as inovações tecnológicâs. Este arligo taz uma revisão sobae as técnicas e concêilos que permitem delimitar esla zona, discuìe a necessidade de obler um diagnóstico lri-axial (topográÍico, etiológico e sindrômico) e comenta sobre novos conceilos concernenles

The aim of the study was to evaluate excessive daytime sleepiness and subjective sleep quality in patients who undergo epilepsy surgery for treatment of refractory partial seizures. Forty-eight patients were enrolled in this research study. All of them were evaluated 2 days before and 3 months after the surgery. Two questionnaires were used to assess daytime sleepiness (Epworth Sleepiness Scale [ESS]) and sleep quality (Pittsburgh Sleep Quality Index [PSQI]). Global PSQI was high (mean=5.65 SD=3.71) before the surgical procedure (P

INTRODUÇÃO: Estima-se que um terço dos casos de pacientes com espectro autista vai apresentar ao menos uma crise epiléptica até a adolescência. Esta associação entre transtornos invasivos do desenvolvimento e epilepsia vem sendo amplamente estudada, mas ainda com inúmeros questionamentos sem resposta na literatura. OBJETIVO: Os autores apresentam o caso de uma criança, com quadro de Transtorno Invasivo do Desenvolvimento - espectro autista - e epilepsia de difícil controle submetida à cirurgia, revisando aspectos fundamentais desta associação. CONCLUSÃO: A partir do caso em questão e de estudos existentes, é pertinente questionar quais são os indícios que nos fazem acreditar que crises epilépticas recorrentes ou uma atividade elétrica anormal sejam responsáveis por alterações cognitivas, de linguagem ou de conduta e qual o tratamento ideal para estas crianças nas quais co-existem os dois diagnósticos. INTRODUCTION: Some reports have indicated that one third of children with autistic spectrum disorder will present at least one seizure untill early adolescence. The association between autism and epilepsy is recognized but remains unresolved and poorly understood. OBJECTIVE: The authors present a child with autistic spectrum disorder and infantile spasms in the first year of life who underwent resective surgery for intractable epilepsy and discuss aspects of this relationship. CONCLUSION: Despite a growing number of studies involving autism and epilepsy we must point out how is the evidence that recurrent seizures or abnormal electrical activity can cause cognitive, language or behavioral abnormalities and what is the ideal treatment for these children in whom a neurodevelopmental disorder coexists with epilepsy.

Background: Patients with hypothalamic hamartomas (HH) often have severe refractory epilepsy, incapacitating behavioral abnormalities, and cognitive decline. Attempts to control the seizure disorder by resection of apparently epileptogenic mesial temporal or other cortical structures have failed consistently. Objective: To report a series of 13 patients in whom the hamartoma itself was resected. Methods: All patients underwent preoperative evaluation between ages 2 and 33 years and had subtotal or complete resection of the hamartoma. Follow-up ranged from 1 to 5.5 years (mean: 2.8 y). Results: Preoperatively, all patients had variable combinations of gelastic, complex partial, and generalized seizures. Eight had drop attacks. In addition, all had marked behavior abnormalities and cognitive impairment. Postoperatively, two patients are completely seizure-free and 11 are either seizure-free or have achieved a greater than 90% reduction of drop attacks and generalized tonic-clonic seizures. However, minor gelastic, complex partial, and atypical absence seizures have persisted in 11 patients, although at significantly reduced rates. In addition, there has been a dramatic improvement in behavior and cognition. Three patients had an anterior thalamic and one a capsular infarct, which left only minimal long-term deficits. Exact location of the lesion in relation to the interpeduncular fossa and the walls of the third ventricle correlated with extent of excision, seizure control, and complication rate. Conclusion: Resection can alleviate both the seizures and the behavioral and cognitive abnormalities of hypothalamic hamartomas, but complications are frequent.

Summary: Purpose: Surgical results in patients with mesial temporal lobe epilepsy due to hippocampal sclerosis (MTLE/HS) are often reported in conjunction with other etiologies of TLE. Methods: We prospectively collected surgical outcome data for 2 to 11 years for 134 consecutive patients who specifically had MTLE and unilateral HS, according to magnetic resonance imaging (MRI) and confirmed by histopathology. Sixty-five had postoperative neuropsychological testing. Outcome was analyzed by using Engel’s classification (a) through Kaplan‐Meier estimated survival curves (as a function of the time to seizure recurrence), (b) as percentage of patients in each outcome class on a yearly basis, and (c) at the last updated follow-up. Results: Kaplan‐Meier estimates of complete seizure freedom (Engel’s class IA) for years 1, 2, 5, and 10 were 85%, 77%, 74%, and 66%, and of Engel’s class I were 89%, 86%, 83%, and 81%. Only nine (6.7%) patients had outcome classes III or IV at any point during follow-up. Of the patients tested, 26% of those operated on the left side and 22% of those operated on the right had postoperative decline of >1 SD in verbal or visual memory, respectively. Conclusions: High rates of seizure freedom can be obtained and remain stable over the years in patients operated on for unilateral MTLE/HS, even in countries with limited resources. Key Words: Temporal Lobe epilepsy—Hippocampal sclerosis— Epilepsy surgery—Long-term outcome.