Biomedical subjects

Maria Augusta Montenegro

Publications and source records attributed to Maria Augusta Montenegro.

At least 19 recordsLinked to original sources

PubMed2006

What about us? Siblings of children with epilepsy.

RATIONALE: It is known that epilepsy has a severe impact in the quality of life of the patients; however, it affects the lives of all family members. The psychosocial repercussions of epilepsy are often of greater significance than the seizures themselves. METHODS: This was a prospective study, conducted from January 2005 to December 2005 at the pediatric epilepsy clinic of our University Hospital. Parents were interviewed by one of the authors according to a structured questionnaire about the impact of epilepsy in the life of the siblings of children with epilepsy. RESULTS: One hundred and twenty-seven children, siblings of 78 patients with epilepsy were evaluated. From the 127 siblings of children with epilepsy, 60 were girls and 67 were boys. Ages ranged from 5 to 18 years old (mean=11.7 years). After the diagnosis of epilepsy, the siblings had only negative feelings toward the disease, mostly sadness and fear. CONCLUSION: Our data showed that the impact of epilepsy in the lives of siblings of children with epilepsy is much more severe than previously suspected.

Adolescent↗

PubMed2006

Specific language impairment: linguistic and neurobiological aspects.

Specific language impairment (SLI) occurs when children present language maturation, at least 12 months behind their chronological age in the absence of sensory or intellectual deficits, pervasive developmental disorders, evident cerebral damage, and adequate social and emotional conditions. The aim of this study was to classify a group of children according to the subtypes of SLI and to correlate clinical manifestations with cortical abnormalities. Seventeen children with SLI were evaluated. Language assessment was based on standardized test (Peabody) and a non-standardized protocol, which included phonological, syntactical, semantical, pragmatical and lexical aspects of language. All children, except one, had abnormal MRI. Thirteen children presented perisylvian polymicrogyria. The MRI findings in the remaining three patients were: right frontal polymicrogyria, bilateral fronto-parietal atrophy, and hypogenesis of corpus callosum with Chiari I. The data show that patients with posterior cortical involvement tended to present milder form of SLI (no sign of articulatory or bucofacial praxis disturbance), while diffuse polymicrogyric perisylvian cortex usually was seen in patients who presented severe clinical manifestation, mainly phonological-syntactic deficit. In conclusion, SLI may be associated with perisylvian polymicrogyria and clinical manifestation may vary according to the extent of cortical anomaly.

Adolescent↗

PubMed2006

Pharmacovigilance in epileptic patients using antiepileptic drugs.

OBJECTIVE: To investigate the occurrence of adverse effects of antiepileptic drugs (AED) in chronic epileptic patients in mono or polytherapy. METHOD: We evaluated consecutive patients that met the following inclusion criteria: age of 18 years or older, diagnosis of epilepsy for at least one year, stable dose of AED for at least three months. Patients were asked if they had any adverse event related to the AED. After that, they were interviewed according to a detailed semi-structure questionnaire. We also assessed specifically the adverse events in the last four weeks. The data were analyzed regarding the use of monotherapy x polytherapy, and the presence of probable depression (score>15) according to the Center for Epidemiologic Studies Depression Scale. RESULTS: Sixty-five patients were evaluated, 35 women, mean age 38.3 years; 35 patients were in use of monotherapy and 35 in polytherapy. Only 45 (69.2%) patients spontaneously reported adverse effects. After the formal questionnaire, 63 (97%) patients referred experiencing an adverse event (p<0.001). Seventeen men had adverse events, as opposed to 28 women (p=0.042). When the last four weeks were evaluated, patients with probable depression presented adverse events more frequently (p<0.0001). CONCLUSION: Our data suggest that adverse events are highly prevalent when a detailed questionnaire is applied and that depression may aggravate the number and intensity of side effects in patients using AEDs.

Adult↗

PubMed2006

Voxel-based morphometry reveals excess gray matter concentration in patients with focal cortical dysplasia.

PURPOSE: Many patients with focal cortical dysplasia (FCD) continue to have seizures after surgical treatment. The usual explanation for the poor surgical outcome is the presence of residual dysplastic tissue missed by the preoperative neuroimaging investigation and therefore not resected during surgery. We apply a voxel-based morphometry (VBM) analysis to the magnetic resonance imaging (MRI) scans from patients with epilepsy and visually detected FCD to investigate whether (a) VBM is able to detect gray-matter concentration (GMC) abnormalities in patients with FCD, and (b) whether the extent of GMC abnormalities in the brain of these patients differs from the regions observed by using visual inspection. METHODS: We studied 11 patients with visually detected FCD (eight of them with histologic confirmation of FCD). The GMC from each one of these patients was compared with the mean GMC from a control group of 96 normal healthy subjects by using an optimized VBM protocol. RESULTS: Ten of 11 patients showed statistically significant GMC excess, and among patients with GMC excess, only one showed GMC excess that was not exactly correspondent to the visually detected FCD. Seven patients exhibited excess in GMC extending beyond the area of visually detected FCD. CONCLUSIONS: This preliminary neuroimaging study suggests that (a) VBM can detect GMC excess in patients with FCD, and (b) GMC excess in these patients can extend to brain areas not visually defined as abnormal. Abnormal areas detected by VBM can possibly correspond to mild malformations of cortical development, supporting the notion that the surgical refractoriness observed in patients with FCD can be due to the incomplete resection of the dysplastic tissue.

Adolescent↗

PubMed2006

Coexistence of childhood absence and rolandic epilepsy.

The coexistence of absence and rolandic epilepsy is extremely rare. This coexistence has been attributed to either the treatment or an atypical course of the disease. However, for some authors, this issue remains controversial. We report the case of a 9-year-old girl presenting with both absence seizures and focal seizures consistent with rolandic epilepsy.

Anticonvulsants↗

PubMed2006

Epileptic features of patients with unilateral and bilateral schizencephaly.

The extent of cortical maldevelopment might correlate with the severity of the clinical manifestation, such as cognitive delay or motor dysfunction. The objective of this study was to investigate the clinical features of epilepsy in patients with unilateral and bilateral schizencephaly. We studied 44 consecutive patients with schizencephaly diagnosed by magnetic resonance imaging (MRI). The epileptic features were analyzed in detail: frequency of epilepsy, median age at onset of epilepsy, semiology of seizures, characteristic features of electroencephalographic (EEG) abnormalities, epileptic syndromes, and antiepileptic drug treatment. We also verified the presence of motor disabilities. Data were analyzed according to the presence of unilateral or bilateral clefts and to the presence of open-lip versus closed-lip schizencephaly. We used the chi-square test and Fisher exact test for statistical analysis. Twenty-four patients had a unilateral cleft (group 1) and 20 patients had bilateral clefts (group 2). Ages ranged from 1 to 37 years (mean 10.6 years). Epilepsy was present in 15 (63%) patients in group 1 and in 11 (55%) patients of group 2; a history of status epilepticus occurred in 13% of group 1 and in 27% in group 2; and a history of clusters of seizures occurred in 40% of group 1 and in 45% of group 2. Eight (53%) patients in group 1 and 6 (54%) patients in group 2 were in monotherapy. Ten (67%) patients in group 1 and 7 (64%) patients in group 2 had seizures controlled with antiepileptic drugs. The frequency of EEG abnormalities was similar between groups (75% and 85%, groups 1 and 2, respectively). Statistical analysis showed no difference between the two groups in the variables mentioned above. However, motor disability was significantly more frequent and more severe in group 2. Regarding the type of schizencephaly (open lip versus closed lip), there was no difference in the frequency of patients with epilepsy, and severe motor deficit was more frequently found in bilateral and open-lip schizencephaly. The extent of the cortical maldevelopment in patients with schizencephaly does not correlate with the severity of the clinical and EEG features of epilepsy, unlike the cognitive and motor manifestations. In addition, the type of schizencephaly (open lip versus closed lip) does not correlate with the presence of epilepsy or seizure control, unlike motor deficit.

Adolescent↗

PubMed2006

Interictal electroencephalographic findings in children and adults with temporal lobe tumors.

OBJECTIVE: To characterize clinical and interictal electroencephalographic aspects of children and adults with temporal lobe epilepsy (TLE) due to tumoral lesions. METHOD: We performed a retrospective analysis of the clinical and interictal electroencephalographic aspects of 16 children (64 exams) and 12 adults (78 exams) with lesions in the temporal lobe. RESULTS: The most frequent etiologies were gangliogliomas, DNETs, followed by astrocytomas. Auras occurred in both groups, the most common being epigastric sensation. Other findings such as myoclonias, behavioral arrest and vomiting were more frequent in children. Temporal epileptiform and nonepileptiform activities, mostly unilateral, were found in both groups. Extratemporal epileptiform activities (frontal, parietal, central, occipital and generalized) were also found equally in both groups. CONCLUSION: Our data show that children and adults with TLE due to expansive lesions present with similar EEG findings.

Adult↗

PubMed2006

Effectiveness of clobazam as add-on therapy in children with refractory focal epilepsy.

The objective of this study was to evaluate the safety and efficacy of clobazam in children with refractory focal epilepsy. We investigated 100 consecutive patients concerning etiology of epilepsy, previously used antiepileptic drugs, seizure frequency and adverse events. Clobazam was introduced as add-on therapy in patients with previous failure of at least two monotherapies. Mean age was eight years-old and 39 patients were girls. Clobazam mean dosage was 23.6 mg/day. Mean use of clobazam was 18.6 months. Twenty-two patients had adverse events. Twenty-six patients became seizure-free, 11 had an improvement of >75% and in 58 there was no modification in seizure frequency. Five patients had an increase in seizure frequency. Clobazam efficacy lasted for more than one year in 42% of the seizure-free patients. Clobazam seems to be safe and effective in the treatment of focal epilepsy in childhood and should be considered in patients with refractory seizures.

Adolescent↗

PubMed2006

Clobazam as add-on therapy in children with epileptic encephalopathy.

RATIONALE: Clobazam has been used successfully in adults and children with partial epilepsy. The purpose of this study was to evaluate the safety and efficacy of clobazam as add-on therapy in children with epileptic encephalopathy. METHODS: This was a retrospective study conducted at the pediatric epilepsy clinic of our university hospital. Children less than 18-years of age with epileptic encephalopathy were included in the study. Clobazam was introduced as add-on therapy, starting with 5 mg/Kg/day and increased in minimally effective doses, up to the maximum tolerated dose. Data were obtained from clinical files and follow-up visits. RESULTS: Ninety-seven patients were included in the study (39 girls), aged between 1 and 17-years-old (mean = 9.9). Twenty-six patients had Lennox-Gastaut syndrome, seven had myoclonic astatic epilepsy, nine had West syndrome and, in 57 patients, the type of epileptic encephalopathy could not be determined. Clobazam dosage ranged from 5 to 60 mg/day (mean = 37.5 mg/day). Forty (41%) patients presented with adverse events, most of which were mild and transitory, and clobazam needed to be withdrawn in only 11 patients. Nine (9.2%) patients were seizure-free after clobazam adjunctive therapy. In 11 (11.3%) patients seizure improvement was >75%, in 16 (16.5%) it was >50%, in 17 (17.5%) improvement was <50% and in 44 (45.5%) there was no change in seizure frequency. Three patients were lost to follow-up. In 85% of the patients with seizure improvement, the results lasted for more than one year. CONCLUSION: Clobazam is safe and effective in the treatment of epileptic encephalopathies of childhood.

Adolescent↗

PubMed2005

Menstrual cycle worsening of epileptic seizures in women with symptomatic focal epilepsy.

INTRODUCTION: Hormonal fluctuation is responsible for worsening of epileptic seizures during the menstrual cycle. OBJECTIVE: To identify irregularities in the menstrual cycles of women with mesial temporal lobe epilepsy (MTLE) and extratemporal focal epilepsy (ETFE) and correlate the frequency of seizures during the menstrual cycles. METHOD: We evaluated prospectively women in the menacme with MTLE and ETFE. Calendars were provided for these patients, and they were asked to mark their seizure frequency according to the menses. Calendars were reviewed in each routine medical appointment. RESULTS: Thirty-nine patients with MTLE and 14 with ETFE were evaluated. We registered 211 cycles in the patients with MTLE and 49 in those with ETFE. Irregular menstrual cycles were found in 28 (28/39, 71.7%) patients with MTLE and 6 (6/14, 42.8%) with ETFE (p=0.052). Premenstrual seizure worsening was observed in 46 (21.8%) patients with MTLE and 9 (18.3%) with ETFE (p=0.596). Menstrual worsening was observed in 47 (22.2%) patients with MTLE and 15 (30.6%) with ETFE (p=0.217). Ovulatory worsening was observed in 36 (17%) patients with MTLE and 13 (26.5%) with ETFE (p=0,126). Catamenial worsening was observed in 58 (27.4%) of the patients with MTLE and in 17 (34.7%) of the patients with ETFE (p=0.315). CONCLUSION: There was no difference between the group of patients with MTLE and ETFE regarding the frequency of irregular cycles and seizure worsening during the premenstrual, menstrual, catamenial or ovulatory periods.

Adolescent↗

PubMed2005

[Neuropsychomotor development of infants born of mothers with gestational hypertension].

INTRODUCTION: Gestational hypertension is a major cause of maternal death in our country and may be associated with neonatal hypoxic-ischemic encephalopathy with serious neurological complications. OBJECTIVE: To correlate gestational hypertension with risk factors of neuropsychomotor development in infants. METHOD: This was a prospective study. We evaluated 30 consecutive infants born of mothers with gestational hypertension. The following risk factors were considered: small for gestational age; fetal asphyxia; age of onset of gestational hypertension; term/preterm newborn; Apgar scores; central cyanosis; O2 mask; meconium. The study followed two steps. In the first step, newborns underwent neurological examination soon after birth (48-72 hours of life). In the second step, children underwent another neurological assessment between 7 and 15 months of life. RESULTS: Six newborns presented neurological signs on the first evaluation. The only risk factor that showed a significant correlation with the neurologic examination was the Apgar score. Other risk factors did not show any correlation. All children evaluated on the second step of the study showed normal neurological development and examination, which did not allow any correlation with risk factors. CONCLUSION: Our data suggest that gestational hypertension per se is not sufficient to cause fetal neurological impairment.

Adult↗

PubMed2005

[Perisylvian syndrome: report of one Brazilian family with focus on the genetic mode of inheritance and clinical spectrum].

Perisylvian syndrome (PS) refers to a variety of clinical manifestations associated with lesions in the perisylvian or opercular region. Acquired lesions such as cerebrovascular diseases or virus encephalitis and congenital lesions such as polymicrogyria (PMG) may be implied as etiological factors. The onset of the PS may occur in early childhood. The aim of this study was to report one family with PS in order to draw attention to this rarely diagnosed entity. Our family has five affected patients, three children and two male adults. All of them had developmental language disorder. Epilepsy, motor deficit and pseudobulbar signs (such as drooling) were detected in one child who had diffuse PMG along the Sylvian fissure. Subtle clinical manifestations correlated with either subtle MRI findings or normal MRI. Most reported families provide evidence suggestive of X-linked transmission. However, the most likely mode of inheritance in our family is autosomal dominant, since a male to male transmission was documented.

Adult↗

PubMed2005

[Efficacy and tolerability of vigabatrin in West syndrome].

UNLABELLED: West syndrome (WS) is a severe epileptic encephalopathy of childhood, characterized by spasms, developmental deterioration and hipsarhythymia. OBJECTIVE: To evaluate the safety and efficacy of vigabatrin (VGB) in the treatment of WS. METHOD: We evaluated every patient diagnosed with WS seen at the pediatric epilepsy clinic and exposed to VGB. Patients were interviewed according to a semistructured questionnaire and we analyzed gender, age, etiology (cryptogenic or symptomatic), associated diseases, age of seizure onset, neuroimaging findings, EEG prior and after VGB, use of other antiepileptic drugs, time for seizure control, electroretinogram, visual complaints, adverse events and family history of epilepsy. RESULTS: Twenty-three patients were evaluated, 16 boys, ages ranging from 1.25 years to 11.5 years (mean=5y3m). Sixteen (69.5%) patients were seizure free, five (22%) had partial seizure control and in two (8.5%) there was no improvement. Only one patient presented gabaergic retinopathy. Six (26%) patients presented adverse events: somnolence, aggressivity or retinopathy. Patients with seizure onset after 6 months of age presented better results after VGB introduction (p<0.05). There was no difference in seizure control according to duration of epilepsy before VGB treatment or etiology of the seizures (p>0.05). After VGB, no patient presented hipsarrhythymia and 50% had a normal EEG. CONCLUSION: Although VGB may be associated with serious adverse events such as gabaergic retinopathy, our results show that it should be considered in the treatment of WS.

Anticonvulsants↗

PubMed2005

Clobazam as add-on therapy for temporal lobe epilepsy and hippocampal sclerosis.

BACKGROUND: Clobazam is a benzodiazepine with known antiepileptic action; however, it is not considered first line therapy in the treatment of epilepsy. The objective of this study was to evaluate the efficacy of clobazam as add-on therapy in adults with temporal lobe epilepsy associated with MRI evidence of hippocampal sclerosis (HS). METHOD: This is a retrospective study, conducted at our epilepsy clinic which evaluated clobazam as add-on therapy in patients with temporal lobe epilepsy and MRI signs of HS. Clobazam was prescribed based on the minimum effective dose up to the maximum tolerated dose. RESULTS: Seventy-eight patients met the inclusion criteria (51 women), ages ranging from 16 to 76 years old (mean=42.2). Dosage of clobazam ranged from 5 to 60 mg/day (mean=22.6 mg/day). Clobazam was used from one month to eight years (mean=29 months). Sixteen (20.5%) patients were seizure-free, 20 (25.5%) had more than 75% improvement in seizure control, eight (10%) had more than 50% and 20 (26%) were non responders to clobazam. In 14 (18%) we could not determine seizure frequency during follow-up. The improvement in seizure control lasted for more than one year in 30 (68%) patients. CONCLUSION: Our data suggest that clobazam should be considered as add-on therapy in the treatment of patients with temporal lobe epilepsy associated with MRI signs of HS.

Adolescent↗

PubMed2005

Antiepileptic drug withdrawal in childhood: does the duration of tapering off matter for seizure recurrence?

There is no consensus on how long the duration of antiepileptic drug withdrawal after seizure control should be. The objective of the present study was to verify if the duration of tapering off matters for seizure recurrence in children with controlled epilepsy. In this prospective study, patients were randomized to taper the antiepileptic drug during a period of 1 or 6 months. Fifty-seven patients met the inclusion criteria; 24 were girls, and ages ranged from 2 to 16 years (mean 9.45 years). There was no difference between the two groups according to age, gender, type of epileptic syndrome, electroencephalogram abnormality, and antiepileptic drug (P > .05). In the 1-month group, seizure recurred in 10 patients, as opposed to 12 patients in the 6-month group. The analysis of seizure recurrence in each group showed no difference (P > .05). We conclude that the duration of antiepileptic drug tapering off does not influence the risk of seizure recurrence in childhood.

Adolescent↗

PubMed2005

Intrapartum complications associated with malformations of cortical development.

Birth asphyxia can be an early sign of cerebral palsy, the effect of an antenatal anomaly rather than its cause. The objective of this study was to evaluate the occurrence of intrapartum complications in patients with malformation of cortical development. The data were retrospectively assessed and compared with a control group of children with idiopathic epilepsy. Intrapartum complications were reported in 22 (31.5%) patients, as opposed to only 2 in the control group (p < 0.001). Patients with cortical malformations frequently present intrapartum complications, which could lead to the misdiagnosis of hypoxic-ischemic encephalopathy.

Adolescent↗

PubMed2004

The role of neuroimaging in the investigation of patients with single seizures, febrile seizures, or refractory partial seizures.

Seizures are a symptom of an underlying brain disorder. The accurate identification of the nature of the disease generating the seizures facilitates treatment and can prevent further brain damage. The understanding of the physiopathology underlying the seizures has been greatly enhanced by the possibility to examine these patients with advanced forms of neuroimaging. Among different forms of investigation, neuroimaging techniques play a major role in diagnosis and decision-making in the care of patients with epilepsy. We review the contributions of current methods of imaging in the investigation of patients with single seizures, febrile seizures or refractory seizures, assessing the practical aspects, benefits, and operational costs of various neuroimaging techniques. The focus is on MRI, which is the imaging modality of choice for outpatient investigation. We also assessed the benefits from CT scans, which remain the first option for emergency seizure assessment. The aim was to evaluate how the structural, functional and metabolic information provided by modern techniques of neuroimaging decreases the need for additional investigation and speeds the management of these epileptic disorders. This makes it possible to accurately define the location and the nature of the epileptogenic lesions related to seizures.

Brain↗

PubMed2004

[Treatment of epilepsy: consensus of the Brazilian specialists].

Epilepsy is a frequent condition in the world. Recently a study in Brazil showed prevalence of 18/1000 inhabitants in São José do Rio Preto, São Paulo State. In the last decade, new therapeutic options were discovered or developed. The main therapeutic decision method is based on randomized clinical trials. This method represents the higher level of evidence. However, even these studies have limitations and in some cases the treatment of choice remains controversial. In these instances, the epilepsy experts' opinions become helpful. In 2001 a similar study had been conducted in USA. The aim of this study is to create guidelines for epilepsy treatment based on the opinion of the Brazilian experts. These guidelines can be used to create manuals and strategies for the treatment of some epileptic syndromes according to Brazilian experts. As compared to the North-American guidelines our study better reflects the resources available in our country.

Anticonvulsants↗