Personality traits in patients with juvenile myoclonic epilepsy.

There is evidence of personality disorders in patients with juvenile myoclonic epilepsy (JME). To date, there have been no published quantitative studies on personality traits in JME. The aim of the work described here was to study a group of patients with JME and quantitatively measure personality traits. We evaluated 42 patients (mean age: 26.57 years, SD: 8.38) and 42 controls (mean age: 26.96, SD: 8.48) using a validated personality inventory, the Temperament and Character Inventory (TCI). We applied two scores, one for the Beck Depression Inventory and one for the State-Trait-Anxiety Inventory, as depression and anxiety may impact the performance of these patients on the TCI. We compared both groups on TCI scales using analysis of covariance with Beck Depression Inventory and State-Trait-Anxiety Inventory scores as covariates. Patients with JME obtained significantly higher scores on Novelty Seeking (P=0.001) and Harm Avoidance (P=0.002) and significantly lower scores on Self-Directedness (P=0.001). Patients with JME have a higher expression of impulsive personality traits that demand early recognition to avoid further consequences and facilitate social insertion, consequently avoiding future stigma.

A study of EEG and epilepsy profile in Wolf-Hirschhorn syndrome and considerations regarding its correlation with other chromosomal disorders.

Wolf-Hirschhorn syndrome (WHS) is a genetic disorder caused by a deletion of the short arm of chromosome 4. Sgrò et al. described an electroclinical profile for WHS, but data regarding this issue are scarce. We report an 8-year-old girl presenting the classic phenotype for WHS, confirmed by FISH test. Epilepsy started during infancy with myoclonic seizures. Later, she presented atypical absences, which gradually increased in frequency, and at the age of 2.5 years, she presented a non-convulsive status epilepticus. Epilepsy was controlled with valproate at the age of 6 years. Serial EEGs were performed and showed unusual bursts of generalized, high amplitude delta waves with superimposed low-moderate amplitude sharp waves. A literature review was performed and our case was compared to others, where EEG and/or epilepsy were addressed. Our case and previously published data show that WHS presents a stereotyped epilepsy profile and EEG patterns. A discussion concerning similarities between these findings and those observed in Angelman syndrome has been performed, since in both syndromes, GABA genes are involved and may play a role in the pathogenesis. Although fascinating, this theory is simplistic, since patients with Angelman syndrome without GABA deletion may present epilepsy and EEG abnormalities. Another issue is the striking overlap regarding these features, between WHS and Pitt-Rogers-Danks syndrome, which may be a key in showing that these disorders could be a spectral variation of the same entity.

Lamotrigine and valproate: efficacy of co-administration in a pediatric population.

This study aimed to assess the risks and benefits of the co-administration of lamotrigine and valproate in a pediatric population with refractory epilepsy. Twenty-eight children who received lamotrigine and valproate during co-medication were evaluated. Outcome measurements were established according to efficacy in seizure control, adverse effects, and tolerability. Treatment was considered effective when >50% frequency reduction was obtained. Adverse effects were also analyzed and in patients who presented them the mode of administration was compared with those who did not to verify the importance of this factor. Association of lamotrigine and valproate was considered effective in 64.3% of all patients, regardless of the seizure type. Seizure-free status was obtained in six patients. Drop attacks and secondary generalized tonic-clonic seizures were reduced in five patients, who remained under treatment despite less than the satisfactory (<50%) seizure decrease. Tremor occurred in six patients; urinary incontinence and ataxia in one. Skin rash also occurred, as an early manifestation, in two patients, both with a previous history of hypersensitivity to antiepileptic drugs. Causes for discontinuation were inefficacy of treatment in six patients and presence of adverse effects in two. In our series, seizure control was obtained in most children with refractory epilepsy, some of whom had a previous history of unsatisfactory response to lamotrigine and valproate, either in monotherapy or polytherapy. Adverse effects were uncommon, but skin rash was observed in higher proportions than in other series with lamotrigine or valproate. Nevertheless, these risks may be lessened with slow introduction and by exclusion of patients with a previous history of hypersensitivity.

The diagnostic role of short duration outpatient V-EEG monitoring in children.

Video-electroencephalographic monitoring enables correlation between behavioral and EEG data, however, because it requires prolonged hospitalization, it may be stressful and expensive. This study aimed to assess the benefits and limitations of this procedure in children. We analyzed 39 children classified according to clinical complaints: doubts about epilepsy classification in 23 (Group I); differential diagnosis with nonepileptic events in eight (Group II); and differential diagnosis between cognitive decline and subtle seizures in eight (Group III). Clinical episodes were recorded in 37 patients (94.9%). In Group I, seizure type was reclassified in 11 patients and epileptic syndrome in nine. In two patients a previously unnoticed seizure type was recorded. In Group II, four patients presented epileptic seizures. In Group III, nonconvulsive status was detected in five. Video-electroencephalographic monitoring enabled major modification of therapeutic approach in 21 patients and guided new neuroimaging studies in 10 patients. In conclusion, in patients with frequent seizures, short video-electroencephalographic monitoring allows proper classification of epileptic syndromes, and diagnosis of nonepileptic seizures, promoting introduction of appropriate treatment with a relatively low cost.

Mesial temporal lobe epilepsy with focal photoparoxysmal response.

UNLABELLED: Intermittent photic stimulation (IPS) may produce epileptiform discharges and seizures, most of which are generalized. There are several cases of focal seizures of occipital origin induced by IPS and only five reported cases originating from the temporal lobe (TL). We report an unusual case of TL epilepsy, supported by electroclinical and neuroimaging data, with rhythmic focal TL discharges precipitated by photic stimulus. CASE REPORT: A 37 year old male with refractory epilepsy due to a right mesial temporal sclerosis presented a focal right TL photoparoxysmal response during IPS. A routine, interictal SPECT study obtained with injection of HMPAO, carried out during continuous pulses of the photic stimulus at 16 Hz, disclosed hyperperfusion over the right TL. Presurgical ictal studies demonstrated seizures arising from the same region. A temporal lobectomy rendered the patient seizure-free and further EEGs with IPS were normal. CONCLUSION: To our knowledge, this is the sixth case in medical literature showing evidence of photosensitivity associated with a TL lesion and it is the first in which photic stimulation induced a focal electrographic response of the involved TL. We hypothesize that in rare cases, photosensitivity may be related to a mesial TL lesion without occipital cortex involvement.

[The diagnostic value of short-term video-EEG monitoring childhood epilepsy]. / O vídeo-EEG dia no diagnóstico de eventos paroxísticos na infância.

OBJECTIVE: To investigate the value of short-term video-EEG monitoring in a pediatric population with distinct clinical complaints in order to verify the benefits and limitations of this procedure. PATIENTS AND METHODS: A prospective protocol, developed in the University of São Paulo, analyzed 38 consecutive patients (age ranging from 4 months to 17 years; mean 6.9 years). All patients were referred in order to establish the diagnosis. The patients were divided in the following groups according to the main clinical complaint: doubts about seizure/syndromic classification (Group I, n = 22); differential diagnosis with non-epileptic events (Group II, n = 8) and differential diagnosis between cognitive decline and status epilepticus (Group III, n = 8). RESULTS: Clinical episodes were observed in 36 patients (94.7%). In group I, seizures were reclassified in 11/22 (50%) patients and confirmed in eight (36.4%). One patient presented a sleep disorder and two did not present clinical events during monitoring. Syndromic classification was modified in nine (40.9%). In group II, four patients (50%) presented epileptic seizures; two had movement disorders and two, non-epileptic events. The cognitive deterioration was correlated with non-convulsive status epilepticus in five children (62.5%) of group III. Alterations of therapeutic and/or diagnostic approaches, as a consequence of monitoring, were performed in 21/38 (55.3%) patients. CONCLUSION: In our series, short-term video-EEG monitoring established a reliable diagnosis in most patients due to correlation between clinical and EEG data. This procedure was well tolerated by children, including infants and those with psychiatric disorders.

Surgical treatment of temporal lobe epilepsy with interictal psychosis: results of six cases.

We describe the postsurgical outcome of six patients with medically intractable temporal lobe epilepsy and interictal psychosis who underwent temporal lobe resection. All patients were submitted to a comprehensive presurgical investigation, including prolonged video-EEG monitoring. Despite their psychotic disorders, all patients were able to provide informed consent and we were able to complete the investigation of all cases. Surgical complications occurred in two cases. Seizure outcome was Engel class I (free from incapacitating seizures) in all except one patient. There was no worsening of their psychoses. Until now, there has been relative improvement in the mental conditions of five patients. Although psychosis has been considered by some authors as a contraindication to epilepsy surgery, with appropriate psychiatric intervention, patients with refractory epilepsy and chronic interictal psychosis may be submitted to prolonged presurgical investigation and undergo surgery successfully.

O vídeo-EEG dia no diagnóstico de eventos paroxísticos na infância / The diagnostic value of short-term video-EEG monitoring in childhood

Objetivo: este estudo tem como objetivo investigar o valor do vídeo-EEG dia numa população pediátrica, com queixas clínicas diversas, verificando os benefícios e as limitações deste método.Casuística e métodos: um protocolo prospectivo, desenvolvido na Universidade de São Paulo, analisou 38 pacientes consecutivos (quatro meses a 17 anos; média 6,9 anos). Todos os pacientes foramencaminhados para elucidação do seu quadro clínico. Estes foram classificados, segundo sua queixa clínica principal, em: dúvidas sobre a classificação das crisesl síndromes epilépticas em 22 pacientes (grupo I); diagnóstico diferencial entre eventos epilépticos e não epilépticos em 8 (grupo II); e diagnóstico diferencial entre declíniocognitivo e estado de mal epiléptico (EME) não convulsivo em 8 pacientes (grupo III).Resultados: episódios clínicos foram registrados em 36 pacientes (94,7por cnto ). No grupo I, as crises epilépticas foram reclassificadasem 11/22 (50por cento) pacientes e confirmadas em oito (36,4por cento). Deste grupo, um paciente apresentou distúrbio do sono, e dois não apresentaram eventos clínicos durante a monitorização. A classificaçãosindrômica foi modificada em nove pacientes (40,9por cento). No grupo II, quatro pacientes (50por cento) apresentaram eventos epilépticos. A deterioração cognitiva estava associada com EME em cinco crianças (62,5por cento) do grupo III. Mudanças na conduta terapêutica e diagnóstica, como conseqüência da monitorização, ocorreram em 21/38(55,3por cento) pacientes.Conclusão: em nossa série, o vídeo-EEG dia estabeleceu o diagnóstico na maioria dos pacientes, relacionando os dados clínicoscom os eletrencefalográficos. Este procedimento foi bem tolerado pelas crianças, incluindo lactentes e aquelas com doenças psiquiátricas

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

Analisamos as manifestaçöes clínicas de 27 crises parciais complexas do lobo temporal registradas em vídeo-EEG com ênfase nas manifestaçöes motoras, principalmente postura distônica, paresia crítica e versäo forçada dos olhos e cabeça. A origem das crises no lobo temporal foi baseada na concordância dos exames pré cirúrgicos, que incluiam vários EEGs intercríticos, alteraçöes na TC e RM do crânio, SPECT intercríticos e, em alguns pacientes, também críticos. 8 pacientes tiveram ressecçöes temporais; em 7 foi feita lobectomia temporal sendo demonstrada esclerose mesial temporal em 5; 1 paciente foi submetido a amígdalo-hipocampectomia seletiva e o achado anátomo-patológico consistiu de hemossiderose e glicose hipocampal, possivelmente decorrente de sangramento de um aneurisma gigante da artéria cerebral posterior. Todos os pacientes estäo sem crises após a cirurgia. Enquanto postura distônica e paresia crítica, presentes em 18(66,6 por cento) das crises, foram sempre contralaterais à área epileptôgênica e assim, de valor indiscutível para lateralizaçäo, versäo de olhos e cabeça ocorreu tanto ipso como contralateralmente

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

Vinte e oito estudos intercríticos e 9 críticos/pós críticos de tomografia por emissäo de fóton único (SPECT) foram realizados em 27 pacientes com epilepsia do lobo temporal e esclerose mesial temporal. Estudos intercríticos localizaram corretamente a área epileptogênica em 19 (67 por cento) dos pacientes pela análise visual e 16 (61,5 por cento) pela semi quantitativa, com 10,7 e 15,3 por cento, de falsa lateralizaçä respectivamente. Todos os 9 estudos críticos/ pós críticos detectaram o hemosfério epileptogênico e permitiram localizaçäo correta da área epileptogênica em 5 de 9 pacientes cujos exames intercríticos haviam sido inonclusivos. Três estudos realizados durante crises secundariamente generalizadas e simétricas mostraram hiperfluxo restrito ao lobo temporal