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Biomedical subjects

Marilisa M Guerreiro

Publications and source records attributed to Marilisa M Guerreiro.

At least 19 recordsLinked to original sources

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↗

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↗

Voxel-based morphometry in patients with idiopathic generalized epilepsies.

Idiopathic generalized epilepsies (IGE) are a group of frequent age-related epilepsy syndromes. IGE are clinically characterized by generalized tonic-clonic, myoclonic and absence seizures. According to predominant seizure type and age of onset, IGE are divided in subsyndromes: childhood absence and juvenile absence epilepsy (AE), juvenile myoclonic epilepsy (JME) and generalized tonic-clonic seizures on awakening (GTCS). The limits between these subsyndromes are not well defined, supporting the existence of only one major syndrome. Visual assessment of routine magnetic resonance imaging (MRI) in patients with IGE is normal. MRI voxel-based morphometry (VBM) uses automatically segmented gray and white matter for comparisons, eliminating the investigator bias. We used VBM to study 120 individuals (47 controls, 44 with JME, 24 with AE and 15 with GTCS) to investigate the presence of subtle structural abnormalities in IGE subsyndromes. VBM was performed searching for abnormalities on gray matter concentration (GMC) between patients groups and controls. Compared to controls, JME presented increased GMC in frontobasal region and AE showed increased GMC in the superior mesiofrontal region. The GTCS group did not differ from controls. There were no areas of reduced GMC with the statistical level selected. Region of interest analysis showed increased GMC in the anterior portion of the thalamus in patients with absence seizures. Our results support subtle GMC abnormalities in patients with JME and AE when compared to controls. These findings suggest the existence of different patterns of cortical abnormalities in IGE subsyndromes.

Adolescent↗

[Dichotic listening and spect in dyslexic children].

Dichotic listening (DL) was evaluated in 36 children with: verbal dichotic listening test, alternating disyllable dichotic test and non-verbal dichotic listening test. Children were separated into two groups: experimental group with 18 dyslexic children and control group with 18 normal children. Both groups were comparable in gender, laterality and social-economic level. All dyslexic children underwent neuroimaging exam (SPECT). Our data showed that there was a statistical difference between both groups in all DL tests. Abnormal SPECT findings were seen in 50% of the dyslexic children, hypoperfusion of the left temporal lobe being the most frequent abnormality. We conclude that dyslexic children present an impairment of central neurologic processing that may be detected by DL tests, and by functional imaging exam, such as SPECT, as well.

Analysis of Variance↗

MRI volumetry shows increased anterior thalamic volumes in patients with absence seizures.

The interaction between thalamus and cortex appears to be critical to the pathophysiology of idiopathic generalized epilepsies (IGEs). The objective of this study was to investigate thalamic volumes of a group of patients with IGEs using high-resolution MRI. Thalamic segmentation was performed by the same rater, who was unaware of the diagnosis. Thalamic volumes were divided into anterior half and posterior half. One hundred forty-seven patients were scanned (71 with juvenile myoclonic epilepsy, 49 with generalized tonic-clonic seizures only, and 27 with absence epilepsy). Subgroup analyses with corrections for multiple comparisons showed that, when compared with those of controls, anterior thalamic volumes were increased in patients with absence epilepsy and juvenile myoclonic epilepsy with absence seizures, but not in patients with generalized tonic-clonic seizures only and juvenile myoclonic epilepsy without absence seizures. Our results demonstrated that the anterior thalamus is structurally different in patients with IGEs and absence seizures as compared with patients with IGEs without absence seizures.

Adult↗

EEG features in idiopathic generalized epilepsy: clues to diagnosis.

PURPOSE: To investigate the EEG profile and its contribution for diagnosis and management in a group of patients with a clinical diagnosis of idiopathic generalized epilepsy (IGE) who were referred to a tertiary hospital. METHODS: We retrospectively studied clinical and EEG features of 180 consecutive patients with IGE. Eighty patients were diagnosed with juvenile myoclonic epilepsy (JME), 35 had absence epilepsy (AE), 13 had generalized tonic-clonic seizures on awakening (GTCS-A), 28 had generalized tonic-clonic seizures only (TCS), and 24 had adult-onset idiopathic generalized epilepsy (AIGE). The EEGs were classified in typical (synchronous generalized spike or polyspikes-and-wave discharges with normal background), atypical (with clear focalities or asymmetries), and normal. RESULTS: The 493 EEG exams were analyzed. The first EEG was normal in 45% of the 180 patients, and only 33% had typical abnormalities. AE had a higher proportion of typical examinations and needed fewer sequential examinations to register a typical abnormality compared with the other groups. By contrast, the serial EEG profile of TCS and AIGE showed a higher proportion of normal and atypical EEG findings. CONCLUSIONS: These findings support previous recommendations that IGE patients should be treated with appropriate therapy based on clinical history. Waiting for a typical abnormal EEG pattern can generate an unacceptable delay in the correct diagnosis and treatment of these patients. In patients with long-term epilepsy, the diagnosis may be difficult. Furthermore, serial EEGs can help to elucidate the syndromic diagnosis, especially in patients with TCS and AIGE.

Adolescent↗

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↗

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↗

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↗

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↗

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↗

Comparison of short-term outcome between surgical and clinical treatment in temporal lobe epilepsy: a prospective study.

OBJECTIVE: To compare the efficacy of medical and surgical treatment for refractory mesial temporal lobe epilepsy associated with hippocampal sclerosis (MTLE). METHODS: A prospective controlled non-randomized study of 26 patients with MTLE who underwent surgical treatment and 75 patients with MTLE who underwent medical treatment between August 2002 and October 2004. All patients failed to achieve seizure control with at least two first line antiepileptic drugs (AED) for partial seizures before entering the study. We used Kaplan-Meier survival analyses as a function of time of seizure recurrence to obtain estimates of 95% confident interval of seizure freedom and log-rank test to compare the status of seizure control between the two groups. RESULTS: The cumulative proportion of patients free of all seizures (Engel's class IA) was higher in the surgical group (73%) compared to the clinical group (12%) (p<0.0001). In the surgical group, 2 of 26 patients (7.7%) had transient adverse effects and 2 of 26 patients (7.7%) had a permanent deficit related to the surgical procedure. In the clinical group 7 patients (9.3%) major adverse events during follow-up, including burns and status epilepticus. CONCLUSIONS: Surgical treatment for patients with MTLE who failed to achieve seizure control with two previous AED regimens was more efficient than medical treatment with further trials of AED.

Adult↗

[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↗

[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↗

[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↗

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↗

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↗

[Diagnosis of children with specific language impairment using a developmental scale].

Specific language impairment (SLI) should have an early diagnosis, since it can interfere with social and school adaptation of the child. The aim of this study was to verify the performance of children with SLI in comparison with normal children using the Behavior Developmental Scale of Gesell and Amatruda. Twenty-five SLI children, 3 to 6 years of age, were evaluated. This group was compared to 50 normal children of the same age. Children of control group showed better performance in all aspects of the scale. The medium value of the studied group was borderline in adaptative and social aspects, and was slightly below the medium in the language aspect. We conclude that language disorder may impair the assessment of other areas of development. Nevertheless, this scale may be useful in the evaluation of children with SLI.

Case-Control Studies↗