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Maria Augusta Montenegro

Publications and source records attributed to Maria Augusta Montenegro.

32 records · Page 2Linked to original sources
PubMed2004

Abnormal behavior in children with temporal lobe epilepsy and ganglioglioma.

Temporal lobe epilepsy in childhood is characterized by great clinical, electroencephalographic, and etiological diversity. The prognosis after temporal lobe epilepsy surgery in childhood is usually good, with most patients achieving complete seizure control. However, in some children behavior deteriorates postoperatively. We report two girls (2 and 6 years of age) with refractory seizures due to temporal lobe ganglioglioma. They exhibited aggression and hyperactivity since the beginning of their epilepsy. In both patients, behavioral disturbances worsened postoperatively, despite complete seizure control. Patients and parents should be advised about possible behavioral disturbances after epilepsy surgery, especially in the presence of a temporal lobe developmental tumor, even when seizure control is achieved postoperatively.

Anticonvulsants↗
PubMed2004

Clinical, electroencephalographic, and behavioral features of temporal lobe epilepsy in childhood.

This study describes the clinical, electroencephalographic, and behavioral features of 36 children with temporal lobe epilepsy. Patients were divided into two groups: group A, with 6 patients (< 6 years), and group B, with 30 patients (6-18 years). Statistical analysis was performed considering the significance level of .05. Regarding the clinical features of the focal seizures, motor components were more frequently seen in children younger than 6 years of age (P < .01), whereas automatisms were more frequently seen in patients older than 6 years of age (P < .05). Associated myoclonic seizures were more frequent in the younger age group (P < .01). Behavioral disorders such as hyperactivity and aggressiveness and speech delay were more common in the younger age group (P < .05). Temporal lobe epilepsy in children younger than 6 years of age is more frequently associated with motor components, myoclonic seizures, behavioral disorders, and speech delay. Conversely, temporal lobe epilepsy in older patients has frequent automatisms.

Adolescent↗
PubMed2003

[Epilepsy surgery in childhood: neuropsychological and quality of life assessments].

PURPOSE: To perform neuropsychological and quality of life assessments before and after epilepsy surgery in children; to correlate these parameters with clinical findings. METHOD: Nine children with refractory epilepsy were evaluated before and six months after the surgical treatment with a comprehensive neuropsychological battery and quality of life questionnaire. Children had variable etiologies and surgical procedures. RESULTS: IQ changes did not occur; two children with temporal lobe epilepsy due to tumor had important behavior impairment after surgery despite complete seizure control; best neuropsychological improvement was seen in the child who had Rasmussen's encephalitis and underwent hemispherectomy. Social aspects, drug side effects, seizure perception and the overall level of quality of life improved after surgery. CONCLUSION: Quality of life improvement was clearly correlated with seizure control; nevertheless, neuropsychological improvement depended on several factors, such as etiology, type of epilepsy and surgery.

Adolescent↗
PubMed2002

Interrelationship of genetics and prenatal injury in the genesis of malformations of cortical development.

CONTEXT: Although the causes of some malformations of cortical development (MCD) have been established, others remain unclear. There are several lines of evidence supporting the theory of a complex mechanism that involves genetic and environmental factors. OBJECTIVE: To investigate the interrelationship of genetics and prenatal injury in the genesis of MCD. PATIENTS AND DESIGN: A series of 76 consecutive patients with MCD and their families were systematically questioned about their family histories of epilepsy or other neurological impairment and the occurrence of prenatal events. Whenever possible, magnetic resonance imaging was performed in other family members if MCD was suspected or in the presence of any neurological impairment. Patients were divided into 3 groups according to the type of MCD. Patients in group 1 had focal cortical dysplasia, group 2 had heterotopias (periventricular or subcortical) or agyria-pachygyria, and group 3 had polymicrogyria or schizencephaly. These findings were also compared with a disease-control group of 40 consecutive patients with epilepsy but without MCD. SETTING: Neurology clinic of a university hospital. RESULTS: Of the 76 patients with MCD, 21 (28%) had focal cortical dysplasia, 19 (25%) had heterotopias or agyria-pachygyria, and 36 (47%) had polymicrogyria or schizencephaly. There were 39 men and 37 women, aged 2 to 52 years (mean age, 13 years). In group 2, 6 patients (32%) had a family history of MCD, mental retardation, or miscarriages, suggesting a genetic predisposition. In group 3, family history of MCD was present in 5 patients (14%). Prenatal events occurred in 28 patients with MCD (37%) and 2 controls (5%) and were more frequent in patients with heterotopia or agyria-pachygyria and polymicrogyria (P<.001). Conversely, epilepsy occurred in all patients in group 1, in 17 patients (89%) in group 2, and in 17 patients (47%) in group 3. In group 3, epilepsy was less frequent (P<.001) and also more easily controlled (P =.005) than in other forms of MCD. CONCLUSIONS: Our findings support the idea of a spectrum among the different types of MCD. Focal cortical dysplasia (group 1) is associated with more frequent and severe epilepsy and less important genetic and prenatal events, heterotopias and agyria-pachygyria (group 2) are frequently associated with genetic predisposition, and polymicrogyria and schizencephaly (group 3) are less frequently associated with epilepsy but have a stronger association with genetic and detectable prenatal events.

Adolescent↗
PubMed2002

Daytime outpatient versus inpatient video-EEG monitoring for presurgical evaluation in temporal lobe epilepsy.

Video-EEG monitoring documentation of seizure localization is one of the most important aspects of a presurgical investigation in refractory temporal lobe epilepsy (TLE) patients. The objective of this study was to evaluate the efficacy of inpatient versus daytime outpatient telemetry. The authors evaluated prospectively 73 patients with medically intractable TLE. Ninety-one telemetry sessions were performed: 35 as inpatients and 56 as outpatients. Outpatient monitoring was performed in the EEG laboratory. They used 18-channel digital EEG. Medications were not changed in the outpatient group. For analysis of the data, time was counted in periods (12 hours = 1 period). Statistical analyses were performed using Student's t-test and the chi2 test. There were no differences between the two groups (outpatient versus inpatient) with respect to age and mean seizure frequency before monitoring, mean time to record the first seizure (1.1 versus 1.4 periods), mean number of seizures per period (0.6 for both groups), lateralization by interictal spiking (46% versus 57%), and lateralization by ictal EEG (59% versus 77%). Daytime outpatient video-EEG monitoring for presurgical evaluation is efficient and comparable with inpatient monitoring. Therefore, the improved cost benefit of outpatient monitoring may increase the access to surgery for individuals with intractable TLE.

Adolescent↗
PubMed2002

Neuroimaging characteristics of pseudosubcortical laminar heterotopia.

Subcortical laminar heterotopia (SLH) is a subtype of malformation of cortical development characterized by laminar gray matter between the cortex and ventricles, which can vary in thickness and may be continuous or discontinuous. The objective of this study is to describe a normal finding of high-resolution magnetic resonance imaging that may simulate an SLH. SLH is isointense to cortex on both T1- and T2-weighted/FLAIR images, usually both anteriorly and posteriorly in location. Conversely, pseudo-SLH is a normal variant present only at the posterior aspect of the brain, and with dark signal on both T1- and T2-weighted/FLAIR images.

Brain Diseases↗
PubMed2002

Neuroimaging of acute cerebellitis.

Acute cerebellitis is one of the main causes of acute cerebellar dysfunction in childhood and may be infectious, postinfectious, or postvaccination. The etiology of acute cerebellitis is usually viral. Varicella zoster, Epsten-Barr, rubeola, pertussis, diphtheria, and coxsackie viruses are the most frequently involved agents. Diagnosing of acute cerebellitis can sometimes be difficult because the patient may present only mild cerebellar signs and the examination of cerebrospinal fluid may be normal. The authors present the clinical and neuroimaging findings of 2 patients presenting with acute cerebellitis. Their magnetic resonance imaging showed hyperintense signal of cerebellar gray matter in T2-weighted sequences, which is a strong indication of a diagnosis of acute cerebellitis.

Acute Disease↗
PubMed2002

Rasmussen's encephalitis: the relevance of neuropsychological assessment in patient's treatment and follow up.

Rasmussen's encephalitis is characterized by refractory epilepsy, neurological deterioration and progressive atrophy of one cerebral hemisphere. The objective of this study is to describe the importance of neuropsychological evaluation in the treatment decision and follow-up of patients with Rasmusseńs encephalitis. Neuropsychological assessment was performed in two steps. Firstly, the clinical history was obtained and the Vineland adaptative behavior scale (VABS) was applied. After this first step, the patients with social maturity level equal or higher than the inferior limit underwent a battery of neuropsychological assessment. We evaluated three patients before any specific treatment was started, and six months after the intervention (surgery or plasmapheresis). Patient 1 underwent left hemispherectomy and had global improvement on second neuropsychological assessment. This suggests that the decision of performing surgery was adequate. Patients 2 and 3 underwent plasmapheresis. They did not present cognitive decline between both evaluations which suggest that our decision of postponing surgery was adequate as well. We conclude that neuropsychological assessment is important when evaluating patients with Rasmusseńs encephalitis. That is especially true for patients in whom disease progression is slow, and surgery timing has to be carefully planned.

Adaptation, Psychological↗
PubMed2002

Association of family history of epilepsy with earlier age at seizure onset in patients with focal cortical dysplasia.

OBJECTIVE: To establish the contribution of family history of epilepsy to seizure onset in patients with focal cortical dysplasia (FCD). PATIENTS AND METHODS: From January 1998 to January 2001, we prospectively evaluated 19 consecutive patients (10 male, 9 female) with a diagnosis of FCD based on magnetic resonance imaging. All patients and at least 1 family member were directly interviewed by the same observer after completion of a semistructured questionnaire. Initially, we classified patients into 2 groups: presence or absence of family history of epilepsy. Patients with a family history of epilepsy were subdivided into 2 groups: patients with a family history of epilepsy in first-degree relatives or multiple relatives (n=5) and patients with a family history of epilepsy in relatives who were not first-degree (n=4). Statistical analysis was performed with use of the nonparametric tests Kruskal-Wallis and Kaplan-Meier (survival analysis). P=.05 was considered statistically significant. RESULTS: The ages of the patients ranged from 3 to 41 years (mean, 15.6 years). All patients had similar type and extent of cortical dysgenesis. Ages at seizure onset varied from 1 month to 22 years, with a mean of 5.8 years. Nine patients had a family history of epilepsy. The mean age at the first seizure in patients with a family history of epilepsy was 2.6 years compared with 8.5 years in those with no relatives having epilepsy (P=.02). When patients with a family history of epilepsy were classified further, the mean age at first seizure was 1.9 years for patients with a family history of epilepsy in first-degree or multiple relatives and 3.9 years for patients with a family history of epilepsy in relatives who were not first-degree compared with 8.5 years for patients with no family history of epilepsy (P=.04). CONCLUSION: Our results show that a family history of epilepsy is associated with an earlier age at seizure onset in patients with FCD. Although this is a preliminary finding and a larger sample is needed to confirm these results, we believe these observations provide evidence that genetic modifiers could become an important issue in the clinical presentation of patients with dysplastic lesions.

Adolescent↗
PubMed2002

Focal cortical dysplasia: improving diagnosis and localization with magnetic resonance imaging multiplanar and curvilinear reconstruction.

OBJECTIVE: To establish the contribution of multiplanar reconstruction (MPR) and curvilinear reformatting (CR) to the MRI investigation of focal cortical dysplasia (FCD). METHODS: From a group of patients with intractable frontal lobe epilepsy, we selected patients with neuroimaging diagnosis of FCD. The diagnosis of FCD was based on the neuroimaging findings after a three step evaluation, always in the same order: (a) plain MRI films, (b) MPR, and (c) CR. After the selection of patients, the process of reviewing all the images in the three stages described above was performed by one of us, who did not take part on the selection of patients nor on the initial evaluation, and who was blind to the clinical and EEG findings of the patients. For data analysis, we first assessed the contribution of the additional findings of MPR analysis compared to the results of the evaluation using only plain MRI films, as is usually done in routine practice. Second, we assessed the contribution of CR to the findings of plain MRI films plus MPR. After completing the multistep evaluation, we all went back to review the plain MRI films with knowledge of lesion topography, in order to identify possible subtle features associated with FCD. RESULTS: Seventeen patients met the inclusion criteria. Twelve had imaging diagnosis of FCD and were included in the second step of this project. Plain films of high resolution MRI showed the lesion in 6 (50%) of the 12 patients. By adding MPR to the plain MRI films, we identified lesions in all 12 patients. Furthermore, we found that MPR provided a better lesion localization and ascertainment of its relationship to other cerebral structures in 5 of 6 (83%) patients who had a lesion identified on plain films. By adding CR to the plain MRI films plus MPR analysis, we observed that (a) CR also allowed the identification of the dysplastic lesion in all patients, (b) CR improved lesion localization in one patient, and (c) CR provided a better visualization of the lesion extent in 4 patients (33%), showed a larger lesion in 3, and demonstrated that part of the area suspected as abnormal was more likely volume averaging in 2. CONCLUSION: MPR and CR analysis add to the neuroimaging evaluation of FCD by improving the lesion diagnosis and localization. CR helps to establish the extent of the lesion more precisely, allowing the visualization of some areas not shown on high resolution MRI and MPR. These techniques are complementary and do not replace the conventional wisdom of MRI analysis.

Adolescent↗
PubMedDate not supplied

[Characterization of the linguistic profile of a family with Perisylvian Syndrome].

BACKGROUND: Perisylvian syndrome refers to a variety of clinical manifestations associated to lesions in the perisylvian or opercular regions. Polymicrogyria is the most common structural malformation found. The syndrome may be inherited and the clinical spectrum includes subtle language disturbances on one end and more severe characteristics such as prominent pseudobulbar signs and refractory epilepsy on the other end. Other studies have already associated perisylvian polymicrogyria with developmental language disorders or specific language impairment. AIM: to describe the language deficits of four members of a family with Perisylvian Syndrome, and to correlate these deficits to neuroimaging data. METHOD: The patients underwent neuroimaging investigation, psychological assessment using the Weschler Intelligence Scales, and specific speech-language evaluation. The following tests were used for the assessment of vocabulary, phonology, syntax, pragmatics, reading and writing: Thematical Pictures of Yavas, ABFW-Child Language Test, Peabody Picture Vocabulary Test (PPVT), and other specific protocols. RESULTS: Magnetic resonance imaging revealed perisylvian polymicrogyria in all of the subjects, with varied locations and extensions. Speech-language assessment indicated significant oral and written language deficits in all of the subjects. CONCLUSION: The obtained data indicate that language impairment can co-exist with reading deficits in members of the same family. Neuroimaging findings reveal cortical alterations that are associated to specific language impairments within the spectrum of the Perisylvian Syndrome. Another important aspect evidenced by this study is the similarities in the language profiles of siblings and mother, suggesting that a variety of linguistic manifestations exist within the spectrum of the syndrome. Perisylvian polymicrogyria can be one of the neurobiological malformations involved in the manifestation of these deficits.

Adolescent↗