The executive profile of children with Benign Epilepsy of Childhood with Centrotemporal Spikes and Temporal Lobe Epilepsy.

RATIONALE: Benign Epilepsy of Childhood with Centrotemporal Spikes (BECTS) and temporal lobe epilepsy (TLE) represent two distinct models of focal epilepsy of childhood. In both, there is evidence of executive dysfunction. The purpose of the present study was to identify particular deficits in the executive function that would distinguish children with BECTS from children with TLE. METHODS: We prospectively evaluated 19 consecutive children and adolescents with TLE with hippocampal sclerosis (HS) (57.9% male; mean 11.74years [SD 2.05]; mean IQ 95.21 [SD 15.09]), 19 with BECTS (36.8% male; mean 10.95years [SD 2.33]; mean IQ 107.40 [SD 16.01]), and 21 age and gender-matched controls (33.3% male; mean 11.86years [SD 2.25]; mean IQ 108.67 [15.05]). All participants underwent a neuropsychological assessment with a comprehensive battery for executive and attentional functions. We used ANOVA and chi-square to evaluate differences on demographic aspects among groups (BECTS, TLE-HS, and control groups). Group comparisons on continuous variables were complemented by MANOVA and Bonferroni posthoc comparisons. RESULTS: Patients with BECTS had worse performance than controls in: Matching Familiar Figures Test, time (p=0.001); Matching Familiar Figures Test, time×errors index (p<0.001); Verbal Fluency for foods (p=0.038); Trail Making Test, part B time (p=0.030); Trail Making Test, part B number of errors (p=0.030); and WCST, number of categories achieved (p=0.043). Patients with BECTS had worse performance than patients with TLE-HS on Matching Familiar Figures Test, time (p=0.004), and Matching Familiar Figures Test, time×errors index (p<0.001). Patients with TLE-HS had worse performance than controls on the following tests: Verbal Fluency for foods (p=0.004); Wisconsin Card Sorting Test, the number of categories achieved (p<0.001); and Wisconsin Card Sorting Test, the number of perseverative errors (p=0.028). Patients with TLE-HS had worse performance than patients with BECTS on Digit Backward (p=0.002); and the Wisconsin Card Sorting Test, the number of perseverative errors (p<0.001). CONCLUSIONS: Patients with TLE and BECTS present distinct cognitive profiles. Patients with TLE-HS had worse performance in mental flexibility, concept formation, and working memory compared to BECTS. Patients with BECTS had worse inhibitory control compared to children with TLE-HS. Both TLE-HS and BECTS had a higher number of errors on an inhibitory control test. However, patients with BECTS had a slower mental processing even when compared to patients with TLE-HS. Rehabilitation programs for children with epilepsy must include children with benign epilepsies and must take into account the epileptic syndrome and its particular neurocognitive phenotype.

The impact of intelligence on memory and executive functions of children with temporal lobe epilepsy: Methodological concerns with clinical relevance.

PURPOSE: Patients with TLE are prone to have lower IQ scores than healthy controls. Nevertheless, the impact of IQ differences is not usually considered in studies that compared the cognitive functioning of children with and without epilepsy. This study aimed to determine the effect of using IQ as a covariate on memory and attentional/executive functions of children with TLE. METHODS: Thirty-eight children and adolescents with TLE and 28 healthy controls paired as to age, gender, and sociodemographic factors were evaluated with a comprehensive neuropsychological battery for memory and executive functions. The authors conducted three analyses to verify the impact of IQ scores on the other cognitive domains. First, we compared performance on cognitive tests without controlling for IQ differences between groups. Second, we performed the same analyses, but we included IQ as a confounding factor. Finally, we evaluated the predictive value of IQ on cognitive functioning. RESULTS: Although patients had IQ score in the normal range, they showed lower IQ scores than controls (p = 0.001). When we did not consider IQ in the analyses, patients had worse performance in verbal and visual memory (short and long-term), semantic memory, sustained, divided and selective attention, mental flexibility and mental tracking for semantic information. By using IQ as a covariate, patients showed worse performance only in verbal memory (long-term), semantic memory, sustained and divided attention and in mental flexibility. IQ was a predictor factor of verbal and visual memory (immediate and delayed), working memory, mental flexibility and mental tracking for semantic information. CONCLUSION: Intelligence level had a significant impact on memory and executive functioning of children and adolescents with TLE without intellectual disability. This finding opens the discussion of whether IQ scores should be considered when interpreting the results of differences in cognitive performance of patients with epilepsy compared to healthy volunteers.

Rolandic epilepsy and dyslexia.

OBJECTIVE: Although benign epilepsy with centrotemporal spikes (BECTS) is an idiopathic, age-related epilepsy syndrome with favorable outcome, recent studies have shown impairment in specific neuropsychological tests. The objective of this study was to analyze the comorbidity between dyslexia and BECTS. METHOD: Thirty-one patients with clinical and electroencephalographic diagnosis of BECTS (group A) and 31 paired children (group B) underwent a language and neuropsychological assessment performed with several standardized protocols. Our findings were categorized as: a) dyslexia; b) other difficulties; c) without difficulties. Our results were compared and statistically analyzed. RESULTS: Our data showed that dyslexia occurred in 19.4% and other difficulties in 74.2% of our patients. This was highly significant when compared with the control group (p<0.001). Phonological awareness, writing, reading, arithmetic, and memory tests showed a statistically significant difference when comparing both groups. CONCLUSION: Our findings show significant evidence of the occurrence of dyslexia in patients with BECTS.

Rolandic epilepsy and dyslexia / Epilepsia rolândica e dislexia

Objective Although benign epilepsy with centrotemporal spikes (BECTS) is an idiopathic, age-related epilepsy syndrome with favorable outcome, recent studies have shown impairment in specific neuropsychological tests. The objective of this study was to analyze the comorbidity between dyslexia and BECTS. Method Thirty-one patients with clinical and electroencephalographic diagnosis of BECTS (group A) and 31 paired children (group B) underwent a language and neuropsychological assessment performed with several standardized protocols. Our findings were categorized as: a) dyslexia; b) other difficulties; c) without difficulties. Our results were compared and statistically analyzed. Results Our data showed that dyslexia occurred in 19.4% and other difficulties in 74.2% of our patients. This was highly significant when compared with the control group (p<0.001). Phonological awareness, writing, reading, arithmetic, and memory tests showed a statistically significant difference when comparing both groups. Conclusion Our findings show significant evidence of the occurrence of dyslexia in patients with BECTS. .

Objetivo Apesar da epilepsia benigna da infância com espículas centrotemporais (EBICT) ser uma síndrome epiléptica considerada idiopática, idade-relacionada e de evolução favorável, estudos recentes têm mostrado que essas crianças apresentam prejuízo em testes neuropsicológicos específicos. O objetivo desse estudo foi analisar a comorbidade entre EBICT e dislexia. Método Trinta e um pacientes com diagnóstico clínico e eletrencefalográfico de EBICT (grupo A) e 31 crianças pareadas (grupo B) foram submetidos à avaliação neuropsicológica e de linguagem com vários protocolos estandardizados. Nossos achados foram categorizados em: a) dislexia; b) outras dificuldades; c) sem dificuldades. Nossos resultados foram comparados e analisados estatisticamente. Resultados Os dados mostraram que dislexia ocorreu em 19,4% e outras dificuldades em 74,2% dos nossos pacientes. Esses números foram altamente significativos quando comparados com o grupo controle (p<0,001). Consciência fonológica, leitura, escrita, aritmética e testes de memória mostraram diferença estatisticamente significante quando foram comparados os dois grupos. Conclusão Nossos dados mostraram que há evidência da ocorrência de dislexia em pacientes com EBICT. .

Voxel-based morphometry and intellectual assessment in patients with congenital bilateral perisylvian syndrome.

Congenital bilateral perisylvian syndrome (CBPS) presents with heterogeneous clinical manifestations such as pseudobulbar palsy, language disorder, variable cognitive deficits, epilepsy, and perisylvian abnormalities (most frequently polymicrogyria) on imaging studies. We investigated the relationship between seizures and extent of gray matter (GM) and white matter (WM) abnormalities using voxel-based morphometry (VBM) of brain magnetic resonance imaging (MRI) as well the association between seizures, structural abnormalities and cognitive ability. In this cross-sectional study, we evaluated 51 healthy volunteers and 18 patients with CBPS with epilepsy (seizure group, n = 7) and without (non-seizure group, n = 11). We used VBM (SPM8/DARTEL) to investigate areas with excess and atrophy of both gray and white matter, comparing groups of patients with controls. Intellectual ability of patients was assessed by the WISC-III or WAIS-III. Both groups with CBPS and the control group were homogeneous with respect to gender (p = 0.07) and age (p = 0.065). Besides perisylvian polymicrogyria, the seizure group exhibited areas with GM and WM reduction including temporal, frontal, parietal and occipital lobes. In contrast, we identified fewer areas with GM and WM reduction in the non-seizure group. The seizure group presented worse intellectual performance (performance IQ and global IQ) than the non-seizure group. The seizure group presented with a more widespread pattern of cortical and sub-cortical abnormalities, as well as worse cognition. Our results suggest that patients with CBPS and epilepsy appear to have widespread neuronal damage that goes beyond the areas with MRI-visible perisylvian polymicrogyria.

ADHD in idiopathic epilepsy.

Our aim was to clarify the correlation of attention deficit hyperactivity disorder (ADHD) with epilepsy and behavior problems. This was a cross-sectional study. Sixty children with idiopathic epilepsy were interviewed using the MTA-SNAP IV Teacher and Parent Rating Scale, Vineland Adaptive Behavior Scales and Conners' Rating Scales. We used the chi-square test to analyze the correlation of epilepsy variables in patients with and without ADHD with a significance level of 0.05. Eight patients had ADHD symptoms (13%), seven had the inattentive ADHD subtype and only three had behavioral problems. When epileptic patients with and without ADHD symptoms were compared we found no significant difference in regard to epilepsy variables. All patients were controlled and 43% were either without AED or undergoing withdrawal. Our study revealed a low comorbidity of ADHD symptoms and epilepsy due to low interference of seizures and drug treatment on the comorbid condition.

Memory in children with symptomatic temporal lobe epilepsy.

In children with temporal lobe epilepsy (TLE), memory deficit is not so well understood as it is in adults. The aim of this study was to identify and describe memory deficits in children with symptomatic TLE, and to verify the influence of epilepsy variables on memory. We evaluated 25 children with TLE diagnosed on clinical, EEG and MRI findings. Twenty-five normal children were compared with the patients. All children underwent a neuropsychological assessment to estimate intellectual level, attention, visual perception, handedness, and memory processes (verbal and visual: short-term memory, learning, and delayed recall). The results allowed us to conclude: besides memory deficits, other neuropsychological disturbances may be found in children with TLE such as attention, even in the absence of overall cognitive deficit; the earlier onset of epilepsy, the worse verbal stimuli storage; mesial lesions correlate with impairment in memory storage stage while neocortical temporal lesions correlate with retrieval deficits.

Memory in children with symptomatic temporal lobe epilepsy / Memoria em criancas com epilepsia de lobo temporal sintomatica

In children with temporal lobe epilepsy (TLE), memory deficit is not so well understood as it is in adults. The aim of this study was to identify and describe memory deficits in children with symptomatic TLE, and to verify the influence of epilepsy variables on memory. We evaluated 25 children with TLE diagnosed on clinical, EEG and MRI findings. Twenty-five normal children were compared with the patients. All children underwent a neuropsychological assessment to estimate intellectual level, attention, visual perception, handedness, and memory processes (verbal and visual: short-term memory, learning, and delayed recall). The results allowed us to conclude: besides memory deficits, other neuropsychological disturbances may be found in children with TLE such as attention, even in the absence of overall cognitive deficit; the earlier onset of epilepsy, the worse verbal stimuli storage; mesial lesions correlate with impairment in memory storage stage while neocortical temporal lesions correlate with retrieval deficits.

Em crianças com epilepsia de lobo temporal (ELT) os problemas de memória não são tão bem compreendidos como em adultos. O objetivo desse estudo foi identificar e descrever déficits de memória em crianças com ELT sintomática e verificar a influência de variáveis da epilepsia na memória. Avaliamos 25 crianças com ELT com diagnóstico baseado em aspectos clínicos, eletrencefalográficos e de neuroimagem. Vinte e cinco crianças normais foram comparadas com os pacientes. Todas as crianças foram submetidas à avaliação neuropsicológica para estimar nível intelectual, atenção, percepção visual, dominância manual, e processos de memória (verbal e visual: memória a curto prazo, aprendizado e recuperação tardia). Os resultados nos permitiram concluir que: além de déficit de memória, outros distúrbios neuropsicológicos podem ser encontrados em crianças com ELT, tais como déficit de atenção, mesmo na ausência de déficit cognitivo global; quanto mais precoce o início da epilepsia, pior o armazenamento verbal; lesões mesiais se correlacionam com prejuízo no armazenamento de memória enquanto lesões temporais neocorticais se correlacionam com prejuízos de evocação.

ADHD in idiopathic epilepsy / TDAH em epilepsia idiopatica

Our aim was to clarify the correlation of attention deficit hyperactivity disorder (ADHD) with epilepsy and behavior problems. This was a cross-sectional study. Sixty children with idiopathic epilepsy were interviewed using the MTA-SNAP IV Teacher and Parent Rating Scale, Vineland Adaptive Behavior Scales and Conners’ Rating Scales. We used the chi-square test to analyze the correlation of epilepsy variables in patients with and without ADHD with a significance level of 0.05. Eight patients had ADHD symptoms (13%), seven had the inattentive ADHD subtype and only three had behavioral problems. When epileptic patients with and without ADHD symptoms were compared we found no significant difference in regard to epilepsy variables. All patients were controlled and 43% were either without AED or undergoing withdrawal. Our study revealed a low comorbidity of ADHD symptoms and epilepsy due to low interference of seizures and drug treatment on the comorbid condition.

Nosso objetivo foi clarificar a correlação entre transtorno do déficit de atenção (TDAH) com epilepsia e problemas comportamentais. Este foi um estudo transversal. Sessenta crianças com epilepsia idiopática foram entrevistadas com a Escala para Pais e Professores MTA-SNAP IV, Escala de Comportamento Adaptativo Vineland e Escala Conners. Utilizamos o teste do qui-quadrado para analisar a correlação das variáveis de epilepsia em pacientes com e sem TDAH com um nível de significância de 0,05. Oito pacientes apresentaram sintomas de TDAH (13%), sete tiveram o subtipo inatento e três deles tinham problemas comportamentais. Quando os pacientes epilépticos com e sem TDAH foram comparados, não encontramos diferença significativa em relação às variáveis de epilepsia. Todos os pacientes estavam controlados e 43% deles estavam sem medicação ou em retirada da droga. O nosso estudo mostrou baixa comorbidade entre epilepsia e TDAH devido à baixa interferência de crises e tratamento na condição comórbida.

Memory in children with temporal lobe epilepsy is at least partially explained by executive dysfunction.

An association between memory and executive dysfunction (ED) has been demonstrated in patients with mixed neurological disorders. We aimed to investigate the impact of ED in memory tasks of children with temporal lobe epilepsy (TLE). We evaluated 36 children with TLE and 28 controls with tests for memory, learning, attention, mental flexibility, and mental tracking. Data analysis was composed of comparison between patients and controls in memory and executive function; correlation between memory and executive function tests; and comparison between patients with mild and severe ED in memory tests. Children with TLE had worse performance in focused attention, immediate and delayed recall, phonological memory, mental tracking, planning, and abstraction. Planning, abstraction, and mental tracking were correlated with visual and verbal memory. Children with severe ED had worse performance in verbal and visual memory and learning tests. This study showed that ED was related to memory performance in children with TLE.

Clinical and neuropsychological correlation in patients with rolandic epilepsy.

OBJECTIVES: To evaluate the presence of neurological soft signs (NSS) and to correlate them with the Wechsler Intelligence Scale for Children (WISC III) in patients with rolandic epilepsy (RE). METHODS: Forty children and adolescents aged between 9 and 15 years were studied. They were divided into two groups: G1 - patients with RE (n=20) - and G2 - healthy controls without epilepsy (n=20). They were assessed with the Quick Neurological Screening Test (QNST II) - clinical trial to search for NSS -, and the WISC III - neuropsychological test. RESULTS: No statistical difference between groups was found in WISC III and QNST II. However, children with poorer motor skills had worse performance in the QNST II and also in the execution intelligence quotient - IQ (p=0.001) and in total IQ (p=0.004), thus showing a positive correlation between them. CONCLUSIONS: The QNST II is a good screening tool for the neurologist to detect abnormalities in fine motor skills.

Clinical and neuropsychological correlation in patients with rolandic epilepsy / Correlação entre achados clínicos e neuropsicológicos em pacientes com epilepsia rolândica

OBJECTIVES: To evaluate the presence of neurological soft signs (NSS) and to correlate them with the Wechsler Intelligence Scale for Children (WISC III) in patients with rolandic epilepsy (RE). METHODS: Forty children and adolescents aged between 9 and 15 years were studied. They were divided into two groups: G1 - patients with RE (n=20) - and G2 - healthy controls without epilepsy (n=20). They were assessed with the Quick Neurological Screening Test (QNST II) - clinical trial to search for NSS -, and the WISC III - neuropsychological test. RESULTS: No statistical difference between groups was found in WISC III and QNST II. However, children with poorer motor skills had worse performance in the QNST II and also in the execution intelligence quotient - IQ (p=0.001) and in total IQ (p=0.004), thus showing a positive correlation between them. CONCLUSIONS: The QNST II is a good screening tool for the neurologist to detect abnormalities in fine motor skills.

OBJETIVOS: Avaliar a presença de sinais neurológicos menores (SNM) e correlacioná-los com o Escala de Inteligência de Wechsler para Crianças (WISC III) em pacientes com epilepsia rolândica (ER). MÉTODOS: Foram estudados 40 crianças ou adolescentes entre 9 e 15 anos, divididos em dois grupos: G1 - pacientes com ER (n=20); G2 - controles saudáveis sem epilepsia (n=20). Foram avaliados por meio do QNST II - teste clínico que pesquisa SNM - e do WISC III - teste neuropsicológico. RESULTADOS: Não foi encontrada nenhuma diferença estatística entre os grupos no WISC III e QNST II. Entretanto, crianças com habilidades motoras pobres tiveram pior desempenho no QNST II, assim como no quociente de inteligência (QI) de execução (p=0,001) e no QI total (p=0,004), mostrando, portanto, correlação positiva entre os dois instrumentos. CONCLUSÕES: O QNST II é uma boa ferramenta de rastreamento para o neurologista detectar anormalidades nas habilidades motoras finas.

Neuropsychological assessment of children with rolandic epilepsy: executive functions.

Rolandic epilepsy (RE) is the most common type of childhood focal epilepsy. Although there is no intellectual deficit, children with RE may have specific cognitive impairments. The aims of this study were to identify changes in executive functions in children with RE and to verify the influence of epilepsy and seizure variables. We evaluated 25 children with RE and 28 healthy controls. A comprehensive neuropsychological battery was utilized. The results showed that the RE children had worse performance than the control group in some categories of the Wisconsin Card Sorting Test, the Trail Making Test part B, and the Verbal Fluency Test (FAS). Children with earlier onset of epilepsy had worse performance when compared with children with later onset of epilepsy. We conclude that children with RE may show a deficit in executive function despite their normal IQ. The set of tests was more extensive than what was previously used in other studies. Our study suggests that early seizures can interfere with brain development. Regarding cognition, the term benign should be used cautiously in RE.

Quality of life and epilepsy surgery in childhood and adolescence.

UNLABELLED: Epilepsy can affect the quality of life (QOL) of patients. The temporal lobe epilepsy (TLE) is often refractory to medication, which has an adverse impact on QOL. The surgery can be a form to control the seizures and to improve the QOL of the patients. OBJECTIVE: The aim of this study was to verify the QOL of children and adolescents with TLE who underwent surgery for epilepsy, comparing QOL before and after surgery and investigating which parameters showed improvement. METHOD: We used semi-structured questionnaire in the pre-and post-surgery in 13 patients. The data were analyzed using the Wilcoxon test. RESULTS: The analysis showed that there was general improvement in the QOL postoperatively. There was improvement in general health issues, adverse effects of antiepileptic drugs and the relationship with parents. CONCLUSION: When properly indicated, epilepsy surgery improves quality of life of patients with TLE.

Quality of life and epilepsy surgery in childhood and adolescence / Qualidade de vida e cirurgia de epilepsia na infância e adolescência

Epilepsy can affect the quality of life (QOL) of patients. The temporal lobe epilepsy (TLE) is often refractory to medication, which has an adverse impact on QOL. The surgery can be a form to control the seizures and to improve the QOL of the patients. OBJECTIVE: The aim of this study was to verify the QOL of children and adolescents with TLE who underwent surgery for epilepsy, comparing QOL before and after surgery and investigating which parameters showed improvement. METHOD: We used semi-structured questionnaire in the pre-and post-surgery in 13 patients. The data were analyzed using the Wilcoxon test. RESULTS: The analysis showed that there was general improvement in the QOL postoperatively. There was improvement in general health issues, adverse effects of antiepileptic drugs and the relationship with parents. CONCLUSION: When properly indicated, epilepsy surgery improves quality of life of patients with TLE.

Epilepsia pode afetar a qualidade de vida (QV) de pacientes. A epilepsia de lobo temporal (ELT) é frequentemente refratária ao tratamento medicamentoso, o que tem impacto negativo na QV. A cirurgia pode trazer controle das crises e melhorar a QV de pacientes com epilepsia. OBJETIVO: O objetivo desse estudo foi verificar a QV de crianças e adolescentes com ELT que foram submetidos à cirurgia para epilepsia, comparando a QV antes e após a intervenção cirúrgica, verificando quais foram os parâmetros que apresentaram melhora. MÉTODO: Um questionário semi-estruturado foi aplicado durante a avaliação pré e pós cirúrgica em 13 pacientes. Os dados foram analisados utilizando-se o teste de Wilcoxon. RESULTADOS: A análise mostrou que houve melhora global da QV no pós-operatório. Houve melhora em aspectos gerais da saúde, eventos adversos de drogas antiepilépticas e relacionamento com os pais. CONCLUSÃO: Quando adequadamente indicada, a cirurgia para epilepsia melhora a QV de pacientes com ELT.

Executive dysfunction in children and adolescents with temporal lobe epilepsy: is the Wisconsin Card Sorting Test enough?

The Wisconsin Card Sorting Test (WCST) is the gold standard in the evaluation of executive dysfunction (ED) in patients with temporal lobe epilepsy (TLE). We evaluated 35 children with TLE and 25 healthy controls with the WCST and with a more comprehensive battery. Among the children with TLE, 77.14% showed impairment on the WCST. On other tests (Wechsler Intelligence Scale for Children-Digit Forward, Matching Familiar Figures Test, Trail Making Test, Word Fluency, Finger Windows, and Number-Letter Memory), impairment was demonstrated in 94.29%. The authors concluded that the WCST is a good paradigm to measure executive impairment in children with TLE; however, it may be not enough. Evaluation performed only with the WCST not only underestimated the number of patients with ED, but also missed relevant information regarding the type of ED.

Landau-Kleffner syndrome: long-term follow-up.

PURPOSE: Landau-Kleffner syndrome (LKS) is a rare entity characterized by epilepsy and aphasia. It occurs in previously normal children, usually between three and seven years of age. The long-term outcome of LKS is not completely clear. The aim of this study is to verify the long-term follow-up of a group of patients with LKS, focusing on clinical and electroencephalographic (EEG) aspects, and quality of life. METHODS: This was a transversal study. Between November 2006 and April 2007 seven patients with previous diagnosis of LKS were interviewed. They had had a follow-up of three to 16 years after their disease onset. They were all males between the ages of eight and 27 years old. All patients had normal MRI. Parents and/or patients were interviewed by one of the authors using a structured questionnaire. The Vineland Adaptive Behavior Scales, the Conner's Rating Scales-Revised, and Short-Form Health Survey (SF 36) were used. Each patient had a prolonged interictal EEG recording. All patients had normal MRI. RESULTS: The present investigation revealed that two patients still have seizures several years after epilepsy onset. One patient had total and three others had partial remission of language disturbance, while three patients still have aphasia and verbal auditory agnosia. With respect to quality of life, only one of our patients has a normal life at present. The remaining six patients with some sort of limitation consider the aphasia/agnosia to be the main difficulty in their lives. Five patients have normal EEGs. CONCLUSIONS: The long-term follow-up of patients with LKS shows that epilepsy and EEG abnormalities do not always disappear. Language disturbances tend to persist in most patients. The age of onset of language dysfunction does not seem to correlate with the prognosis for recovery of language function. Patients with LKS have an overall poor quality of life, mostly due to language difficulties.

A new candidate locus for bilateral perisylvian polymicrogyria mapped on chromosome Xq27.

Polymicrogyria (PMG) is characterized by an excessive number of small and prominent brain gyri, separated by shallow sulci. Bilateral perisylvian polymicrogyria (BPP) is the most common form of PMG. Clinical signs include pseudobulbar paresis, mental retardation, and epilepsy. Familial forms of BPP have been described and a candidate locus was previously mapped to chromosome Xq28, distal do marker DXS8103. The objective of this study was to perform linkage analysis in one family segregating BPP. A total of 15 individuals, including 8 affected patients with BPP were evaluated. Family members were examined by a neurologist and subjected to magnetic resonance imaging scans. Individuals were genotyped for 18 microsatellite markers, flanking a 42.3 cM interval on ch Xq27-q28. Two-point and multipoint linkage analysis was performed using the LINKAGE package and haplotype reconstruction was performed by GENEHUNTER software. Our results showed a wide spectrum of clinical manifestations in affected individuals with BPP, ranging from normal to mild neurological abnormalities. Two-point linkage analysis yield a Zmax = 2.06 at theta = 0.00 for markers DXS1205 and DXS1227. Multipoint lod-scores indicate a candidate interval of 13 cM between markers DSXS1205 and DXS8043, on ch Xq27.2-Xq27.3. These results point to a new locus for BPP in a more centromeric location than previously reported.