Rasmussen encephalitis associated with segmental vitiligo of the scalp: clinicopathologic report.

A 3-year-old boy with segmental vitiligo, poliosis, and alopecia over the right side of the scalp developed epilepsia partialis continua involving the left side of the body and progressive atrophy of the right cerebral hemisphere. There was a right ear dysacusia and a perilimbal vitiligo associated with an area of iris depigmentation in the right eye. Pleocytosis and hypergammaglobulin were detected in cerebrospinal fluid. Because medical treatment that included phenobarbital, phenytoin, carbamazepine, oxcarbazepine, benzodiazepines, corticosteroids, gamma-globulin, and a ketogenic diet was ineffective, he underwent a right hemispherectomy. Neuropathologic examination showed a widespread scattered inflammatory process with numerous microglial nodules and perivascular lymphocytic cuffing associated with degenerative changes with severe neuronal loss, loosening of the neuropil, and microcystic changes with tissue collapse. The coexistence of vitiligo and possibly Vogt-Koyanagi-Harada syndrome in this child reinforces the autoimmune theory as the pathogenesis of Rasmussen syndrome.

Clinical characteristics and surgical outcome of patients with temporal lobe tumors and epilepsy.

This is a retrospective study of 21 surgically treated patients with temporal lobe tumors and epilepsy. Evaluation included clinical data, EEG findings, structural scans, pathological diagnosis and post-surgical follow-up. There were 9 cases of ganglioglioma, 5 pilocytic astrocytoma, 3 ganglioneuroma, 2 dysembryoplastic neuroepithelial tumor, 1 pleomorphic xantoastrocytoma, and 1 meningioangiomatosis. Mean follow-up time was 22 months and outcome was evaluated according to Engel's classification; 76.2% were classified in class I and 23.8% in II and III. All patients classes II and III had been submitted to mesial and neocortical resections. There were no differences related to clinical characteristics, pathological diagnosis or duration of follow-up in patients seizure-free or not. All patients had abnormal MRI and ten of these had normal CT; the MRI characteristics were compared to pathological diagnosis and specific histological characteristics of the tumors were not discernible by MRI. We concluded that MRI was essential for the diagnosis and precise location of TL tumors. Ganglioglioma was the most frequent tumor and lesionectomy associated to mesial resection doesn't guarantee a better prognosis.

[Bradycardia as an epileptic manifestation in temporal epilepsy: report of a case]. / Bradicardia como manifestação epiléptica em epilepsia temporal: relato de caso.

We describe a patient who had cardiac arrhythmia as epileptic manifestation. In a 34-year-old woman who had many episodes of loss of consciousness, the simultaneous ECG and video-EEG monitoring recorded bradycardia with a short episode of asystole (4 seconds) and left temporal rhythmic theta activity on EEG. MRI showed a small mass lesion in the left parahippocampal gyrus. Alterations in cardiac rhythm have been reported in epileptic seizures and tachycardia is the most common finding associated with them; bradyarrhythmia during seizures was uncommon. Many interconnections among insular cortex, limbic system and hypothalamus, may be responsible for vegetative manifestations in temporal lobe epilepsy.

Cerebellar hemorrhage after supratentorial surgery for treatment of epilepsy: report of three cases.

OBJECTIVE AND IMPORTANCE: We report three cases of cerebellar hemorrhage complicating supratentorial craniotomies for the treatment of epilepsy. In a literature review, we identified only four similar cases of cerebellar hemorrhage after temporal lobectomy for the treatment of epilepsy. CLINICAL PRESENTATION AND RESULTS: Three young and otherwise healthy patients underwent frontal, occipital, and temporal resections for the treatment of refractory epilepsy. The hemorrhage manifested as peduncular tremor, ataxia, and decerebrate posturing presenting early in the postoperative period. The diagnosis was established by computed tomography and/or magnetic resonance imaging. Benign outcomes were observed for all patients. CONCLUSION: Based on the available data, it is our opinion that brain dislocation resulting from excessive intraoperative cerebrospinal fluid drainage is a possible mechanism for this rare complication of supratentorial craniotomy. The overdrainage seems to be less hazardous when the procedure is performed for the removal of space-occupying mass lesions. In contrast, the resection of nonexpanding tissues, such as in lobectomies for the treatment of epilepsy, may be an additional risk factor, because the incidence of this complication seems to be higher in these situations.

[Sexual disorders in epilepsy. Results of a multidisciplinary evaluation]. / Alterações sexuais na epilepsia. Resultados de uma avaliação multidisciplinar.

Eleven epileptic men who complained of epilepsy and sexual dysfunction were submitted to a multidisciplinary evaluation. Mean age was 27 years (20-34), mean epilepsy duration was 19 years (0.5-32) and the mean seizure frequency was two by week (0-7). Ten patients had partial seizures and one other had myoclonic epilepsy. Ten patients were treated with antiepileptic drugs (phenytoin--1, carbamazepine--8, clonazepam--3, clobazam--2, valproic acid--3, vigabatrin--1). As defined in the DSM III-R, the complaints were: erectile disorder (9), hypoactive sexual desire disorder (4), frotteurism (4), inhibited orgasm (3), premature ejaculation (3), fetishism (2), voyeurism (2), exhibitionism (2), pedophilia (1) and sexual aversion disorder (1). Two patients showed hypogonadotropic hypogonadism on endocrinologic screening. Urological evaluation disclosed organic erectile dysfunction in other two. One patient had a diagnosis of psychogenic sexual disorder. In six patients a conclusive etiologic diagnosis was not reached. This report shows the multifactorial nature of sexual disorder in epilepsy and underlies the need of a multidisciplinar evaluation.

Clinical and EEG analysis of mesial and lateral temporal lobe seizures.

The analysis of the temporal lobe seizures through video-EEG systems shows that they often consist of a sequence of clinical and EEG features which may suggest the localization and the lateralization of the epileptogenic lobe. We analyzed clinical and EEG features of 50 temporal lobe seizures which were separated in group 1 with 25 patients (related to mesial temporal sclerosis) and group 2 with 25 patients (other neocortical temporal lesions). Among the auras, the epigastric type was the most frequent and predominated in group 1. There were differences between the two groups, considering dystonic and tonic posturing and versive head and eye movements. Dystonic posturing was always contralateral to the ictal onset and was considered the most useful lateralizing clinical feature. Ictal speech, spitting and blinking automatisms, prolonged disorientation for place and a greatest percentage of postictal language preservation occurred in right temporal seizures. Postictal aphasia and global disorientation predominated in left temporal seizures. EEG was important for lateralizing the epileptogenic lobe, specially considering rhythmic ictal activity and postictal findings.

Neuroimaging findings in Rasmussen's syndrome.

Rasmussen's syndrome is a progressive childhood disease of unknown cause characterized by severe epilepsy, hemiparesis, mental deterioration, inflammation of one cerebral hemisphere, and brain atrophy. Computed tomography, single-photon emission computed tomography (SPECT), and magnetic resonance (MR) neuroimaging findings of 8 patients with pathologically confirmed Rasmussen's syndrome were evaluated retrospectively. All patients showed a predominance of the atrophy in the temporoinsular region and cerebral hemispheric alterations on MR images in a similar extension as seen on SPECT studies. Focal increase in regional cerebral blood flow was observed in the 4 patients presenting with epilepsia partialis continua at the time of hexamethylpropyleneamineoxime injection. Extensive cortical hypoperfusion was noted in the other 4 patients who received the injection during the interictal state. Cerebellar functional abnormalities were present in 6 patients, 2 of them with structural damage.

Double pathology in Rasmussen's encephalitis: etiologic considerations.

In a 7-year-old girl with epilepsia partialis continua (EPC) involving the left face, arm, and leg for 1 year, serial neuroimaging studies showed progressive, brain atrophy. Because medical treatment was ineffective, she underwent a large fronto-temporal surgical resection. Neuropathological examination showed loss of lamination and dysplastic neurons, gliosis, microglial nodules, and perivascular cuffing. Such "double pathology" (dysgenesia and a chronic inflammatory process) may have implications for the pathophysiology of Rasmussen's syndrome.

Neurophysiological characteristics of Lafora's Disease

Avaliamos as características neurofisiológicas (EEG e potenciais evocados) de 2 pacientes na fase III da doença de Lafora, No EEG, desorganizaçäo da atividade de base foi associada a descargas epileptiformes difusas e bilaterais, com acentuaçäo em regiöes pccipitais onde também ocorreram descargas isoladas nos exames de ambos os pacientes. Resposta fotoconvulsiva foi evidenciada nos 2 casos e, em 1 deles, foram registradas descargas occipitais seguindo a estimulaçäo fótica com baixa frequência (1-3 flashes/s) correspondendo a grande amplitude de N2-P2 na resposta evocada visual por flash

[Single photon emission tomography in temporal epilepsy: interictal and ictal studies with visual and semi quantitative analysis]. / Tomografia por emissão de fóton único em epilepsia temporal: estudos intercríticos e críticos com análise visual e semi quantitativa.

Single photon emission tomography (SPECT) was performed in 27 patients with refractory complex partial seizures from the temporal lobes due to mesial temporal sclerosis. Independent blinded observers assessed the 28 interictal studies and 9 ictal/postictal studies. Visual analysis of interictal studies detected hypoperfusion in 22, ipsilateral to the epileptogenic zone in 19 (67%) and contralateral in 3 (10.7%). Quantified temporal lobe asymmetry, greater than a previously derived normal range, correctly identified the epileptogenic zone in 16 (61.5%) with false lateralization in 4 (15.3%). In all 9 cases in which they were performed, ictal/postictal studies showed hyperperfusion at the region of epileptic focus. In 3 patients with complex partial seizures followed by symmetric generalized tonic-clonic seizures, hyperperfusion restricted to the temporal lobe was demonstrated. In 5 of these patients the interical studies were unable to demonstrate localized changes. There were no significant correlations between SPECT findings and clinical parameters or EEG slowing in the temporal lobes.

Anatomia patológica em 33 pacientes com epilepsia do lobo temporal / Anatomopathology in 33 patients with temporal lobo epilepsy

As crises parciais complexas do lobo temporal säo associadas a vários tipos de lesöes anátomo-patológicas, como esclerose mesial temporal, tumores, malformaçöes, infartos e infecçöes. Relataremos achados histopatológicos em 33 espécimes cirúrgicos consecutivos de pacientes com epilepsia temporal refratária a drogas antiepilépticas em todos, com exceçäo de 1 paciente. O único achado patológico em 20 deles foi esclerose mesial temporal, lesäo caracterizada por perda neuronal e gliose das estruturas mesiais do lobo temporal principalmente do hipocampo. Nove destes pacientes apresentaram crises nos 2 primeiros anos de vida, representadas por convulsöes febris complicadas em 8 deles, 4 traumatismos cranianos e 3 tiveram antecedentes de anorexia perinatal. A formaçäo hipocampal foi adequadamente estudada em 11 espécimes e se pôde verificar distribuiçäo näo uniforme das lesöes, as quais acometiam principalmente o setor de Sommer em 5, o hilo do giro denteado em 3 e estas duas regiöes nos outros 3 casos. Em 5 pacientes o hipocampo näo esteve disponível para análise e em outros 4 se encontrava destruído. Apenas 1 dos 13 pacientes desta série, com outras lesöes anátomo-patológicas, tinha antecedente de convulsäo febril. Os achados anátomo-patológicos destes 13 pacientes incluíram: Tumores (8 casos representados por gangliogliomas em 5, tumor neuroepitelial disembrioplástico em 1, astrocitoma pilocítico em 1 e oligodendrogioma em 1), microdisgenesia, granulomas cisticercótico e tuberculoso, hemossiderose e acúmulo de corpos psamomatosos. Este último achado foi considerado inespecífico

Intractable complex partial seizures associated with posterior cerebral artery giant aneurysm: a case report.

Giant aneurysms have rarely been reported in association with intractable complex partial seizures (CPS). We report a 30-year-old man with intractable CPS since age 18 years. Seizure onset was electrically localized to right temporal lobe. Preoperative neuroimaging studies showed a partially thrombosed giant aneurysm of the right posterior cerebral artery. Selective amygdalohippocampectomy and occlusion of the posterior cerebral artery did not cause deficits. The patient has been seizure-free for 15 months after operation. We review the relevant literature on aneurysms as a cause of epilepsy.

Functional significance of MRI defined mesial temporal sclerosis in temporal lobe epilepsy.

The functional significance of MRI-defined mesial temporal sclerosis in temporal lobe epilepsy (TLE) is not completely established. In order to study the possible relationship between signals of mesial temporal sclerosis on MRI and interictal SPECT findings we selected 18 patients with complex partial seizures, unilateral temporal EEG focus and normal CT. The EEG focus was defined by the presence of interictal sharp waves and slow background on several scalp EEG obtained during many years of follow up in all patients and by ictal recordings with sphenoidal electrodes in 12 patients. Group I comprised patients (n = 11) in whom MRI showed mesial temporal sclerosis; group II patients (n = 7) had normal MRIs. All patients were submitted to interictal 99m-Tc HMPAO injections with concomitant EEG monitoring. Lateralized hypoperfusion ipsilateral to the EEG was found in 13 patients (72%). In all Group II and in 6 Group I patients a temporal hypoperfusion was found. This SPECT study showed a higher positivity rate in patients with normal MRI than previously reported. On the other hand, in all these group II patients a neocortical origin of epileptic focus was suspected on clinical or electroencephalographic basis. Positive SPECT findings may be at least as prevalent in neocortical as in mesiolimbic epilepsy.

[Clinical manifestations in complex partial crisis of the temporal lobe: a video-electroencephalographic study]. / Manifestações clínicas de crises parciais complexas do lobo temporal: um estudo vídeo-eletrencefalográfico.

We analysed 27 complex partial seizures arising from the temporal lobes recorded on videotape simultaneously with the EEG emphasizing the motor manifestations specially dystonic posturing, ictal paresis and head and eye forced deviation (version). The temporal lobe origin of the seizures was based on the agreement of many scalp-sphenoidal or zygomatic interictal and ictal EEG recordings, CT and MRI findings, interictal and, in some patients, ictal SPECT studies. 8 patients had surgery. In 5 from 7 patients who had temporal lobectomy, mesial temporal sclerosis was the anatomopathological finding and in one patient who had selective amigdalohippocampectomy, hemosiderosis and gliosis probably due to bleeding of a posterior cerebral artery giant aneurysm was found. All patients have been seizure free after surgery. While dystonic posturing and ictal paresis, present in 18 seizures (66.6%), were excellent as lateralizing seizure signs, since they were always contralateral to the ictal onset, contralateral and ipsilateral versive head and eye movements were observed.

MRI and brain spect findings in patients with unilateral temporal lobe epilepsy and normal CT scan.

26 patients with temporal lobe epilepsy clinically documented by several abnormal interictal surface EEGs with typical unitemporal epileptiform activity and a normal CT scan were studied. Interictal 99mTC HMPAO brain SPECT and MRI were performed in all subjects. Abnormalities were shown in 61.5% of MRI (n = 16) and 65.4% of SPECT (n = 17). Hippocampal atrophy associated to a high signal on T2-weighted MRI slices suggesting mesial temporal sclerosis was the main finding (n = 12; 75% of abnormal MRI). MRI correlated well to surface EEG in 50% (n = 13). There was also a good correlation between MRI and SPECT in 30.7% (n = 8). SPECT and EEG were in agreement in 57.7% (n = 15). MRI, SPECT and EEG were congruent in 26.9% (n = 7). These results support the usefulness of interictal brain SPECT and MRI in detecting lateralized abnormalities in temporal lobe epilepsy. On the other hand, in two cases, interictal SPECT correlated poorly with surface EEG. This functional method should not be used isolately in the detection of temporal lobe foci. MRI is more useful than CT as a neuroimaging technique in temporal lobe epilepsy. It may detect small structural lesions and mesial temporal lobe sclerosis which are not easily seen with traditional CT scanning.

MRI and brain SPECT findings in patients with unilateral temporal lobe epilepsy and normal CT scan

Foram estudados 26 pacientes com epilepsia do lobo temporal clinicamente documentada por vários EEGs com atividade epileptiforme intercrítica e TC de crânio normal SPECT com TC HMPAO e ressonância magnética (RM) foram realizados em todos os casos 61,5 por cento (n=17) dos SPECTs revelaron algum tipo de anormalidade. Atrofia hipocampal, nas imagens enfatizadas em T1 e presença de hipersinal nas imagens enfatizadas em T2, sugerindo esclerose mesial temporal, foram o principal achado (n=12,75 por cento das RM anormais). RM correlacionou-se ao ECG em 50 por cento (n=13). Concordância entre RM e SPECT ocorreu em 30.7 por cento (n=8) e entre SPECT e EEG em 57.7 por cento (n=15), RM, SPECT e EEG foram congruentes em 26.9 por cento (n=7). Estes resultados refletem o valor do SPECT intercrítico e RM na detecçäo e lateralizaçäo de anormalidades em epilepsia do lobo temporal. Por outro lado, em dois casos, o SPECT correlacionou-se mal com o EEG. Este método funcional näo deve ser utilizado isoladamente na detecçäo de focos temporais. RM é mais útil do que a TC como recurso de neuroimagem na epilepsia do lobo temporal. Ela pode detectar pequenas lesöes estruturais e esclerose mesial temporal, näo facilmente identificáveis pela TC

Características clínicas de pacientes com epilepsia temporal e esclerose mesial / Clinical characteristcs of patients with temporal lobe epilepsies and mesial sclerosis

Entre as epilepsias do lobo temporal, a síndrome temporal mesial é caracterizada por manifestaçöes clínicas homogêneas e achados específicos em exames paraclínicos. Descrevemos características clínicas de 22 pacientes, com 14 a 51 anos de idade (média 31,8 anos) e duraçäo da epilepsia de 6 a 41 anos (média 22,08 anos), com epilepsia do lobo temporal e sinais de esclerose mesial na RM e/ou no estudo anátomo-patológico. Crises epilépticas nos primeiros anos de vida estavam presentes em 19 (86,3 por cento ) dos pacientes; dentre estas, 11 foram convulsöes febris (em 10 complicados), as quais, quando apresentaram manifestaçöes motoras dimidiadas, constituíram importante dado para a lateriazaçäo da área epileptogênica, sendo sempre contralaterais ao lado da esclerose mesial temporal. Ocoreu intervalo livre de 8 meses a 24 anos (média 7,58 anos) entre o possível fator etiológico e o aparecimento de crises recorrentes sugestivas de epilepsia temporal, que incluiam crises parciais simples, com manifestaçöes viscerais, e crises parciais complexas, durante as quais sinais motores lateralizados foram importantes na lateralizaçäo do lobo epileptogênico de 9/71 pacientes. Crises tônico-clônicas generalizadas infreqüentes foram relatadas por 13 pacientes (59 por cento ), ocorrendo geralmente no início da epilepsia