Congenital bilateral perisylvian syndrome: familial occurrence, clinical and psycholinguistic aspects correlated with MRI.

OBJECTIVE: Congenital bilateral perisylvian syndrome (CBPS) is frequently caused by polymicrogyria (PMG). The aim of this study was to correlate the clinical and psycholinguistic aspects with neuroradiological data of patients with CBPS. METHODS: Thirty-one patients were studied. We performed a clinical investigation of the patients and their families, including MRI scanning, neuropsychological tests and language evaluation. RESULTS: The statistical analysis showed that: a) prenatal events are associated with the non-familial type of PMG; b) diffuse PMG is associated with pseudobulbar signs, as opposed to BPPP; c) motor deficit is associated with diffuse PMG; d) epilepsy is equally present in patients with both familial or non-familial PMG, but is more frequently seen in patients with diffuse PMG; e) dyslexia and SLI can be a feature of both the diffuse or BPPP, and either familial or sporadic cases of PMG. CONCLUSIONS: The severity of clinical manifestations in CBPS is correlated with the extent of cortical involvement. Most patients with CBPS have a history of speech delay or language difficulties and no epilepsy. Dyslexia can be found in patients with PMG.

MRI reveals structural abnormalities in patients with idiopathic generalized epilepsy.

OBJECTIVE: To evaluate MRI findings in a large group of patients with idiopathic generalized epilepsies. METHODS: Idiopathic generalized epilepsies were diagnosed according to clinical and EEG criteria following International League Against Epilepsy recommendations. MRI was performed in a 2.0 T scanner using a previously established epilepsy protocol. Images were reviewed, and any abnormality was reported. Patients were divided in those with and without MRI abnormalities. Comparisons were made between these groups concerning age, age at seizure onset, subsyndrome, EEG findings, and seizure control. RESULTS: Of the 134 MRIs evaluated, 33 (24%) showed abnormalities, most of which (88%) were nonspecific. There were eight main abnormalities: arachnoid cyst, diffuse cortical atrophy, basal ganglia abnormalities (signal alterations and prominent perivascular spaces), ventricular abnormalities (uni- or bilateral increased volume of the lateral ventricles), white matter abnormalities (increased T2 signal in the frontal lobes), reduced hippocampal volume, focal gyral abnormality, and area of gliosis in the frontal lobe. Comparisons between the groups showed a higher proportion of EEG focalities in patients with abnormal MRI, which were in most part concordant with the location of the MRI abnormalities. CONCLUSIONS: Twenty-four percent of patients with idiopathic generalized epilepsies had MRI abnormalities. However, the majority of these abnormalities were nonspecific.

Patterns of hippocampal abnormalities in malformations of cortical development.

OBJECTIVE: To assess whether different types of malformation of cortical development (MCD) are associated with specific patterns of hippocampal abnormalities. METHODS: A total of 122 consecutive patients with MRI diagnosis of MCD (53 males, age range 1-58 years) were included in the study. Hippocampal measurements were made on 1-3 mm coronal T1-weighted MRIs and compared with MRIs of normal controls. RESULTS: A total of 39 patients had focal cortical dysplasia, 5 had hemimegalencephaly, 5 had lissencephaly-agyria-pachygyria, 11 had SLH, 11 had PNH, 12 had bilateral contiguous PNH, 5 had schizencephaly, and 34 had polymicrogyria. The frequency of hippocampal abnormalities in these patients with MCD was 29.5%. A small hippocampus was present in all types of MCD. Only patients with lissencephaly and SLH had an enlarged hippocampus. Abnormalities in hippocampal rotation and shape were present in all types of MCD; however, these predominated in PNH. None of the patients with lissencephaly-agyria-pachygyria or SLH had hyperintense signal on T2 or FLAIR images or abnormal hippocampal internal architecture. CONCLUSION: A small hippocampus was present in all types of MCD; however, the classic MRI characteristics of hippocampal sclerosis were often lacking. Abnormal enlargement of the hippocampus was associated with only diffuse MCD due to abnormal neuronal migration (lissencephaly-agyria-pachygyria and SLH).

Childhood epilepsy due to neurocysticercosis: a comparative study.

PURPOSE: To assess the clinical profile of pediatric patients with epilepsy and neurocysticercosis (NC), and compare them with a group of pediatric patients with benign partial epilepsy to determine clinical differences, response to treatment, and prognosis. METHODS: We studied 28 patients (16 girls) with probable or definitive diagnosis of NC and epilepsy and 32 patients (16 girls) with partial benign epilepsy (BE). All patients had normal neurologic examination. We compared NC and BE patients looking for differences in demographics (age at first seizure, gender, family history); clinical presentation (type, frequency, duration, and total number of seizures, duration of epilepsy, status epilepticus, cluster, and postictal deficit); treatment [duration, number of antiepileptic drugs (AEDs), maximal dose, drug association, number of seizure-free patients, time to obtain control and recurrence after medication discontinuation]; complementary examinations (the first and the last EEG). RESULTS: The mean follow-up was 5.4 years for the 28 NC patients and 4.6 years for the 32 BE patients (p=0.98). We did not find statistical differences between NC and BE in gender, family history, types of seizures, frequency and length of seizures, previous status epilepticus, seizure clustering, and presence of postictal deficits. However, we found that NC compared with BE patients had significant longer AED treatment, more seizures after AED introduction, tried more AEDs and at maximal dose, and in 20%, required polytherapy. The recurrence rate in NC was 54.4% and this was not significantly associated with number of lesions and disease activity seen on CT scans or the presence of EEG abnormalities. CONCLUSIONS: NC presents with a mild form of epilepsy in terms of seizure severity; however, it is more challenging in regard to drug management and has a less favorable long-term prognosis in terms of seizure remission. The number of lesions or disease activity seen on computed tomography (CT) as well as EEG abnormalities have no prognostic value in childhood epilepsy due to NC.

Predisposition to metabolic acidosis induced by topiramate.

RATIONALE: Metabolic acidosis induced by topiramate is a well documented but infrequent adverse event. The objective was to demonstrate the lowering of carbon dioxide serum levels, which is usually asymptomatic but may facilitate the occurrence of metabolic acidosis in patients using topiramate. METHODS: We evaluated, prospectively, the carbon dioxide serum levels of 18 patients seen at the epilepsy clinic of our university hospital, before and 3 months after introducing topiramate. RESULTS: Five patients were female and 13 were male, age ranging from 2 to 16 years old (mean=9. 3). Carbon dioxide mean serum levels were 25 and 21.2 mmol/L (normal = 22 to 30), before and 3 months after introducing topiramate, respectively. Dose ranged from 2.08 to 11.76 mg/kg/day (mean=6. 7mg/kg/day). Adverse events were anorexia, nausea and somnolence. CONCLUSION: We conclude that the lowering of carbon dioxide serum levels induced by topiramate is mostly asymptomatic, but may facilitate the occurrence of metabolic acidosis. Since patients in use of topiramate have refractory epilepsy, they may need epilepsy surgery, and must be carefully monitored for the risk of metabolic acidosis during surgery.

De novo psychogenic seizures after epilepsy surgery: case report.

The occurrence of de novo psychogenic seizures after epilepsy surgery is rare, and is estimated in 1.8% to 3.6%. Seizures after epilepsy surgery should be carefully evaluated, and de novo psychogenic seizures should be considered especially when there is a change in the ictal semiology. We report a patient with de novo psychogenic seizures after anterior temporal lobe removal for refractory temporal lobe epilepsy. Once psychogenic seizures were diagnosed and psychiatric treatment was started, seizures stopped.

Surgical treatment of epilepsy in tuberous sclerosis: strategies and results in 18 patients.

BACKGROUND: Seizures in patients with tuberous sclerosis complex (TSC) are often intractable to antiepileptic medications and searching investigation may provide evidence that surgical treatment can be considered. OBJECTIVE: To review the results of investigation and surgical therapy, a treatment modality not generally considered in patients with medically refractory seizures and TSC. METHODS: We report 18 patients (9 male) with TSC who underwent surgical treatment of medically refractory epilepsy. Twelve patients had a well-localized epileptogenic lesion and were treated by lesionectomy or focal resection. Resections were: 7 frontal, 4 temporal, 1 frontotemporal, 1 occipital, and 1 frontoparietal. Four patients underwent more than one operation. Six patients had corpus callosotomy (CC). RESULTS: Follow-up ranged from 1 month to 47 years. Outcome of the patients treated by resection was excellent in 7 (5 were seizure-free and 2 had auras only), good in 1, fair in 3, and 1 was lost to follow-up. Best outcome was obtained in patients who had focal seizures and good imaging and EEG correlation, although they might have multiple seizure types, other imaging abnormalities, and multifocal or generalized EEG findings. When there was no such correlation, CC was found to be an option as five patients had at least some improvement and only one showed no change. CONCLUSION: Surgical treatment of patients with TSC and intractable epilepsy is most effective when a single tuber or epileptogenic area can be identified as the source of seizures and resected. This may be possible even when other tubers or diffuse EEG abnormalities are present. In patients with unlocalizable epileptic abnormalities, palliation may be obtained by CC.

Epilepsias recém-diagnosticadas: aderência, tolerância e prognóstico com a primeira droga / Recently diagnosed epilepsy: adherence, tolerance, and prognostic with the first drug

Há poucos dados no nosso meio sobre epilepsias recém-diagnosticadas (ERD). Com o objetivo de avaliar a aderência, a tolerância e a eficácia da primeira droga antiepiléptica receitada, seguimos 78 pacientes de 6 a 61 anos de idade (média: 17.96 anos) com ERD por um tempo médio de 12.68 meses (1 a 29 meses). Estes pacientes apresentavam crises parciais, com ou sem generalizaçäo secundária, e crises generalizadas tônico-clônicas com um tempo médio de epilepsia de 7.68 meses (4 dias a 7 anos). Encontramos 11 pacientes (14.10 por cento ) näo aderentes ao tratamento e 14 (17,94 por cento ) com efeitos colaterais que justificaram a troca da medicaçäo. Os efeitos colaterais mais prevalentes foram alteraçöes dismórficas como hirsutismo e hiperplasia gengival, síndrome dispéptica, reaçöes idiossincrásicas e sedaçäo. Sessenta e seis por cento dos pacientes mantiveram-se completamente controlados por um período de 8 semanas e 63.88 por cento por 56 semanas. Estes dados säo consistentes com os achados da literatura internacional. A taxa de näo aderência ao tratamento foi relativamente alta (14,10 por cento ), possivelmente devido aos aspectos sócio-econômicos e culturais da populaçäo estudada. Ressaltamos que 17,94 por cento dos pacientes näo toleraram a primeira droga, necessitando de substituiçäo. Aproximadamente 2/3 dos pacientes com ERD obtém controle satisfatório com a primeira medicaçäo

Discite em crianças: estudo de oito casos / Discitis en children: study of 8 cases

Os autores apresentam o estudo de oito crianças com diagnóstico de discite estabelecido através da avaliaçäo clínica, neurológica e ortopédica. A comprovaçäo de alteraçöes inflamatórias em um espaçäo discal, vertebral e em tecidos moles foi possível em estudos por imagem, sendo discutidos aqueles obtidos por radiologia convencional, tomografia computadorizada e ressonância magnética

[Congenital hemiparesis: complementary neuropsychological evaluation with computer]. / Hemiparesia congênita: avaliação neuropsicológica complementar utilizando computador.

We present the neuropsychological assessment with computer aid of six cerebral palsy children. Three children had right hemiparesis and three, left hemiparesis. The tomographic examination showed parietal cavities (porencephalic cyst in 4 children, ischemic injury in 1 case and subarachnoid cyst in 1 case). We have proposed to assess the visuo-spatial function since we suspected the children could have disturbance of this function. We did not detect this disturbance. On the other hand, the children had astereognosia and the right hemiparetic children preferred to execute signs on the right part of the computer visor. We discuss and propose explanations for both findings.

[Basal ganglia calcifications in children]. / Calcificações dos gânglios da base na infância.

We report the study of four children with bilateral basal ganglia calcifications (BGC) visualized on CT scan. Epilepsy was the clinical manifestation of three patients whose laboratory investigation revealed abnormal calcium metabolism. The first aim of this paper is to call attention to a treatable entity that can cause epileptic syndromes in infancy and childhood. The second purpose is to review the literature comparing with our fourth child who presented encephalopathy with BGC.

[Cranial computed tomography aspects in neurocysticercosis in childhood]. / Aspectos da tomografia computadorizada craniana na neurocisticercose na infância.

The authors present the analysis of 27 computed tomography scans (CT) of 18 children which were divided in three groups according to clinical and tomographic criteria. Group 1 was characterized mainly by epilepsy and calcifications. Group 2 was characterized by intracranial hypertension and several tomographic aspects: edema, cysts and nodules were seen in three patients; hydrocephaly and calcifications were seen in two patients and CT was normal in one patient. Group 3 had patients with epilepsy or headache and variable tomographic patterns. The results are discussed based on the available literature.

[Aneurysmal bone cyst of the orbit: report of a case]. / Cisto ósseo aneurismático orbitário: relato de um caso.

The authors report a case of aneurysmatic osseous cyst of the orbit in a 10 year-old girl. The tumor was totally removed and the reconstruction of the orbital roof with acrylic was performed.

[Infratentorial malignant neurinoma: report of a case]. / Neurinoma maligno infratentorial: relato de um caso.

A solitary malignant schwannoma of the foramen jugular, unassociated with von Recklinghausen's disease in a two years and nine months old girl is presented. A partial removal of the tumor was carried out. The low incidence in this age group is emphasized in report to the literature reviewed.

Herpes zoster oftalmico e posterior acidente vascular cerebral: relato de caso / Ocular herpes zoster and delayed cerebrovascular disorder: report of a case

Um caso de acidente vascular cerebral (AVC) 14 semanas apos a instalacao de herpes zoster oftalmico (HZO) e apresentado.A tomografia computadorizada craniana documentou comprometimento em territorio de arteria cerebral media ipsilateral ao HZO.O diagnostico de probabilidade e o de arterite por herpes zoster com posterior trombose. Os autores reviram a literatura e enfatizam o longo intervalo entre o HZO e a instalacao da hemiplegia. Citam as novas drogas antivirais que tornam esta causa de AVC potencialmente passivel de ser prevenida