Luiz Henrique Martins Castro

Human immune globulin infusion in Guillain‐Barré syndrome: Worsening during and after treatment

Because of an unexpectedly high rate of worsening, we report the results of treatment of Guillain-Barré syndrome with a standard regimen of human immune globulin (HIG) in 15 consecutive patients. Patients were treated with 1.5 g/kg HIG for 4 days beginning a mean of 7 days after the first symptoms. Eight stabilized or improved, but seven deteriorated during or within the first 5 days after treatment, and four remained ventilator-dependent for at least 2.5 months. One had a severe relapse 5 weeks after treatment. Five patients subsequently received plasma exchange and two improved. Our experience varies from previous reports that have endorsed HIG treatment for Guillain-Barré syndrome.

Prospective memory and mesial temporal epilepsy associated with hippocampal sclerosis

Episodic memory impairment is commonly observed in patients with epilepsy associated with mesial temporal sclerosis (MTS). Prospective memory (PM) is a set cognitive abilities that allow future performance of a present intention, in response to time- or event-based evocation cues, that trigger the intended action at the appropriate time. PM has not been evaluated in mesial temporal sclerosis. We evaluated the role of right and left hippocampal lesions on performance in both the retrospective and prospective PM components in patients with epilepsy secondary to mesial temporal sclerosis and correlated with performance in traditional neuropsychological tests, as well as with self-perception of memory impairment. We tested the hypotheses that a hippocampal lesion impacts on the prospective components of PM, and that a left-sided lesion had a greater impact on performance in the prospective component of PM than a right-sided lesion. We evaluated PM in 26 patients with right MTS, 22 left MTS patients, and 26 age-gender and education matched controls. The prospective component of PM was impaired in both patient groups, with both a lesion (patients performed significantly worse in the PM battery) and laterality effect (left MTS patients performed significantly worse than right MTS patients in the PM battery). Performance in the prospective component of the PM battery correlated with long-term delay performance in episodic verbal memory and self-perception of memory impairment in the left MTS group. The retrospective component was impaired in left MTS patients. Impaired performance was not accounted for solely by depression, anxiety or an antiepileptic drug effect. We conclude that mesial temporal lobe structures, including the hippocampus, play an important role in both the prospective and retrospective components of PM processes in tasks involving long delay intervals.

Factors associated with treatment non-adherence in patients with epilepsy in Brazil

PURPOSE To investigate factors associated with treatment non-adherence in Brazilian patients with epilepsy. METHODS Prospective cross-sectional study. We evaluated 385 epilepsy outpatients in a tertiary referral center, 18 years or older, literate, without cognitive impairment or active psychiatric disorders, who were independent in daily living activities. Data were analyzed with correlation tests and conjoint analysis using multivariate logistic regression. RESULTS Non-adherence rate, measured by the Morisky-Green Test, was 66.2%, a moderate-to-low adherence level. Non-adherence was higher in men, in younger patients and in patients with uncontrolled seizures. Increasing treatment complexity was also associated with decreased treatment adherence. CONCLUSION Strategies designed to improve treatment adherence should address peculiarities associated with younger ages and male gender. Physicians should be made aware that prescription of less complex treatment regimens may result in better treatment adherence, and, therefore, better seizure control. The challenge in adjusting AED treatment in this population is to minimize treatment complexity, thus increasing chances for treatment adherence.

GRAPH THEORETICAL CHARACTERIZATION AND TRACKING OF THE EFFECTIVE NEURAL CONNECTIVITY DURING EPISODES OF MESIAL TEMPORAL EPILEPTIC SEIZURE

Via a detailed case study of mesial temporal lobe epilepsy, we show that a method of determining the direction of information flow among signals is able to provide focal localization via the simultaneous analysis of multiple EEG channels. This determination is accomplished by representing information flow direction via directed graphs, where focal electrodes are associated with high observed rates of pertinence to strongly connected subgraphs. Further clinical support to this finding is provided by results for an additional 9 cases of focal epilepsy cases. The graph theoretical approach is a tool for describing and analyzing the effective connectivity dynamics behind epileptic seizures and may provide a common language for studying other complex dynamic relationships between neural structures.

HTLV-1 antibodies in serum and cerebrospinal fluid in tropical spastic paraparesis in Brazil

HTLV-1 antibodies were investigated in serum and in CSF of 150 patients with neurologic disorders mainly myelopathies. The patients were considered into three groups according to the possible relationship of their disease to the presence of HTLV-1 antibodies: no relationship risk (control group), occasional risk group, and possible risk group. In this latter are 56 patients with crural spastic paraparesis or paraplegia of unknown etiology (SP). HTLV-1 antibodies were tested by the passive particle-agglutination method for anti-ATLA antibody detection. The search was negative in all patients of the control group, and positive (serum and/or CSF) in 16.5% of the patients from the second group and in 55.4% of the SP patients group. Clinical patterns in SP cases with HTLV-1 antibodies were those of tropical spastic paraparesis (TSP). CSF patterns considered (cytology, protein content and gamma-globulins rate) were different between TSP group with HTLV-1 antibodies in CSF and SP group with no HTLV-1 antibodies detection either in serum or in CSF. The difference was significant. Results of this investigation confirm the high incidence of TSP in Brazil, and bring additional indication for searching HTLV-1 antibodies in the CSF.

Epilepsy syndromes associated with hypothalamic hamartomas

PURPOSE Hypothalamic hamartoma (HH) related epilepsy presents with gelastic seizures (GS), other seizure types and cognitive deterioration. Although seizure origin in GS has been well established, non-GS are poorly characterized. Their relationship with the HH and cognitive deterioration remains poorly understood. We analyzed seizure type, spread pattern in non-GS and their relationship with the epileptic syndrome in HH. METHODS We documented all current seizure types in six adult patients with HH-epilepsy with video-EEG monitoring, characterized clinical-electrographic features of gelastic and non-gelastic seizures and correlated these findings with cognitive profile, as well as MRI and ictal SPECT data. RESULTS Only four seizure types were seen: GS, complex partial (CPS), tonic seizures (TS) and secondarily generalized tonic-clonic seizures (sGTC). An individual patient presented either CPS or TS, but not both. GS progressed to CPS or TS, but not both. Ictal patterns in GS/TS and in GS/CPS overlapped, suggesting ictal spread from the HH to other cortical regions. Ictal SPECT patterns also showed GS/TS overlap. Patients with GS-CPS presented a more benign profile with preserved cognition and clinical-EEG features of temporal lobe epilepsy. Patients with GS-TS had clinical-EEG features of symptomatic generalized epilepsy, including mental deterioration. CONCLUSIONS Video-EEG and ictal SPECT findings suggest that all seizures in HH-related epilepsy originate in the HH, with two clinical epilepsy syndromes: one resembling temporal lobe epilepsy and a more catastrophic syndrome, with features of a symptomatic generalized epilepsy. The epilepsy syndrome may be determined by HH size or by seizure spread pattern.

Emotional trauma and abuse in patients with psychogenic nonepileptic seizures.

A history of childhood trauma and the presence of dissociative phenomena are considered to be the most important risk factors for psychogenic nonepileptic seizure disorder (PNESD). This case-control study investigated 20 patients with PNESD and 20 with temporal lobe epilepsy (TLE) diagnosed by video/EEG monitoring who were matched for gender and age. Patients with both conditions were not included in the study. Groups were evaluated for age at onset and at diagnosis, worst lifetime weekly seizure frequency, trauma history, and presence of dissociative phenomena. Age at onset (P=0.007) and age at diagnosis (P<0.001) were significantly higher in the PNESD group than the control group, as were the scores on the Dissociative Experiences Scale (P<0.001) and Childhood Trauma Questionnaire (P=0.014). Only the differences in scores on the Childhood Trauma Questionnaire subscales Emotional Neglect (P=0.013) and Emotional Abuse (P=0.014) reached statistical significance. Dissociative phenomena and a reported history of childhood trauma are more common in patients with PNESD than in those with TLE. However, only emotional neglect and abuse were associated with PNESD in this study.

Microsurgical Anatomy of the Temporal Lobe: Part 2—Sylvian Fissure Region and Its Clinical Application

OBJECTIVE We present observations of the anatomy of the sylvian fissure region and their clinical application in neuroimaging, microsurgery for middle cerebral artery aneurysms and insular lesions, frontobasal resections, and epilepsy surgery. METHODS Sixty adult cadaveric hemispheres and 12 adult cadaveric heads were studied after perfusion of the arteries and veins with colored latex. The anatomic information was applied in more than 200 microsurgeries in and around the sylvian fissure region in the past 15 years. RESULTS The sylvian fissure extends from the basal to the lateral surface of the brain and presents 2 compartments on each surface, 1 superficial (temporal stem and its ramii) and 1 deep (anterior and lateral operculoinsular compartments). The temporal operculum is in opposition to the frontal and parietal opercula (planum polare versus inferior frontal and precentral gyri, Heschls versus postcentral gyri, planum temporale versus supramarginal gyrus). The inferior frontal, precentral, and postcentral gyri cover the anterior, middle, and posterior thirds of the lateral surface of the insula, respectively. The pars triangularis covers the apex of the insula, located immediately distal to the genu of the middle cerebral artery. The clinical application of the anatomic information presented in this article is in angiography, middle cerebral artery aneurysm surgery, insular resection, frontobasal resection, and amygdalohippocampectomy, and hemispherotomy. CONCLUSION The anatomic relationships of the sylvian fissure region can be helpful in preoperative planning and can serve as reliable intraoperative navigation landmarks in microsurgery involving that region.

Evidence of acute ischemic tissue change in transient global amnesia in magnetic resonance imaging: case report and literature review.

Transient global amnesia is a benign syndrome of sudden‐onset alteration of behavior with temporary dysfunction of anterograde and recent retrograde memory. Its neural substrates remain uncertain. Possible causes include ischemia, migraine, and epilepsy. The authors report a case of a 62‐year‐old man with a transient attack of memory disturbance, suggestive of transient global amnesia, in which magnetic resonance imaging performed 48 hours after onset showed left mesial temporal lobe signal changes on diffusion‐weighted imaging and fluid‐attenuated inversion recovery images. The findings and a literature review lend further support to the ischemic pathogenesis of transient global amnesia as a possible etiology, and underscore the role of diffusion‐weighted imaging in the diagnosis of this condition.

Serial and prolonged EEG monitoring in anti-N-Methyl-d-Aspartate receptor encephalitis

OBJECTIVE To describe serial electroencephalographic (EEG) findings of three patients with anti-NMDAR encephalitis. METHODS Three women (age 15-34years) with confirmed anti-NMDAR encephalitis underwent serial EEG recordings. Continuous EEG for 72h was performed in one case and 3-day video-EEG monitoring was obtained in two cases. RESULTS Generalized rhythmic delta activity (GRDA) was found in all patients. GRDA persisted for hours, but was not continuous on a 24-h EEG recording, disclosed no frequency, voltage or field evolution, and was not seen on the first EEG of two patients. Extreme delta brush was noted in two patients who presented more severe disease. One patient presented seizures, which were electrographically and clinically different from the GRDA pattern and from dyskinetic movements. CONCLUSIONS Serial or continuous EEG may be necessary to detect GRDA in anti-NMDAR patients. To avoid unnecessary treatment, this pattern should not be interpreted as indicative of ictal activity, unless there is evidence of its ictal nature. SIGNIFICANCE Our findings may contribute to the diagnosis of anti-NMDAR encephalitis in cases with characteristic clinical picture.