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Biomedical subjects

Fernando Cendes

Publications and source records attributed to Fernando Cendes.

At least 73 records · Page 4Linked to original sources

Unilateral hippocampal sclerosis with contralateral temporal scalp ictal onset.

PURPOSE: To investigate the clinical characteristics and surgical outcomes in patients with unilateral hippocampal sclerosis whose scalp ictal EEG recordings localize to the opposite temporal lobe. METHODS: We retrospectively reviewed the data of all adult patients who had undergone depth electrode implantation for suspected temporal lobe epilepsy (TLE) at UCLA (1993-2000) or the Montreal Neurological Institute (1991-1998) to identify patients who had (a) unilateral hippocampal atrophy, and (b) surface ictal recordings in which the majority of seizures appeared to initiate in the opposite temporal lobe, with few or none that were concordant with the hippocampal atrophy. RESULTS: Of 109 patients with suspected TLE who underwent depth electrode study at the two centers, five patients met the aforementioned criteria. Four of these five had very severe hippocampal atrophy, whereas the fifth had mild atrophy but extensive signal change on magnetic resonance imaging (MRI). Depth electrode recordings in four of the five patients yielded clear ictal onset in the mesial temporal lobe ipsilateral to the imaging abnormality (contralateral to apparent scalp ictal onset). One patient had an unusual bitemporal onset pattern, which was nonetheless suggestive of onset in the sclerotic hippocampus. No patient had intracranial ictal onset contralateral to the imaging abnormality. All patients underwent resection of the structurally abnormal temporal lobe. After follow-up of > or = 2 years, four (80%) of five patients were seizure free, while the fifth showed lesser improvement (class III). CONCLUSIONS: Some patients with severe hippocampal sclerosis (sometimes called a "burned-out hippocampus") have atypical spread of ictal discharges, resulting in apparent gross discordance between imaging and scalp ictal recordings. These patients nonetheless have excellent surgical outcomes on the whole. Whether such patients may forego intracranial recordings requires further study.

Adult↗

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↗

Proton magnetic resonance spectroscopy and magnetic resonance imaging findings in a patient with central nervous system paracoccidioidomycosis.

The authors describe a patient with brain paracoccidioidomycosis whose magnetic resonance image (MRI) showed multiple hypointense lesions on T2-weighted images with peripheral enhancement after gadolinium injection. Single-voxel proton magnetic resonance spectroscopy (1H-MRS) of one of the lesions showed 2 peaks at 0.9 and 1.32 ppm, corresponding to lipid signals, indicating intense necrosis. The other characteristic peaks of 1H-MRS were undifferentiated from baseline. These findings, although not specific, may help to differentiate fungal abscess from tumoral lesions and other types of abscess.

Adult↗

[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↗

Is low antiepileptic drug dose effective in long-term seizure-free patients?

OBJECTIVE: To investigate the value of leaving seizure-free patients on low-dose medication. METHOD: This was an exploratory prospective randomized study conducted at our University Hospital. We evaluated the frequency of seizure recurrence and its risk factors following complete or partial antiepileptic drug (AED) withdrawal in seizure free patients for at least two years with focal, secondarily generalized and undetermined generalized epilepsies. For this reason, patients were divided into two groups: Group 1 (complete AED withdrawal), and Group 2 (partial AED withdrawal). Partial AED withdrawal was established as a reduction of 50% of the initial dose. Medication was tapered off slowly on both groups. Follow-up period was 24 months. RESULTS: Ninety-four patients were followed up: 45 were assigned to complete (Group 1) AED withdrawal and 49 to partial (Group 2) AED withdrawal. Seizure recurrence frequency after two years follow-up were 34.04% in group 1 and 32.69% in Group 2. Survival analysis showed that the probability of remaining seizure free at 6, 12, 18 and 24 months after randomization did not differ between the two groups (p = 0.8). Group 1: 0.89, 0.80, 0.71 and 0.69; group 2: 0.86, 0.82, 0.75 and 0.71. The analysis of risk factors for seizure recurrence showed that more than 10 seizures prior to seizure control was a significant predictive factor for recurrence after AED withdrawal (hazard ratio = 2.73). CONCLUSION: Leaving seizure free patients on low AED dose did not reduce the risk for seizure recurrence. That is, once the decision of AED withdrawal has been established, it should be complete.

Adolescent↗

Temporal lobe hypogenesis associated with arachnoid cyst in patients with epilepsy.

OBJECTIVE: To determine the frequency of temporal lobe hypogenesis (TLH) associated with arachnoid cysts (AC) in patients with epilepsy. METHOD: We retrospectively revised 655 consecutive MRI scans from patients followed in our epilepsy clinic. We identified patients with temporal AC and then performed careful visual analysis in a workstation. Patients with evident expansive or destructive lesions were excluded. RESULTS: Only 4 (0.6%) patients had AC in the left temporal lobe, all associated with TLH. In addition, there were also ipsilateral dysgenetic characteristics in the ipsilateral hippocampus including abnormal shape and axis, and hyperintense T2 signal. In one patient this hippocampal abnormality was bilateral. CONCLUSION: AC with TLH was rarely found in our patients with epilepsy and it was always associated with hippocampal dysgenesis. Although volumetric reduction of the temporal lobe can be observed in patients with epilepsy and hippocampal abnormalities, the presence of adjacent AC points to a malformative etiology.

Adolescent↗

Effects of method and MRI slice thickness on entorhinal cortex volumetry.

We assessed the effect of the method of analysis and the MRI slice thickness on entorhinal cortex volumetry. A T1 gradient echo 3D volumetric acquisition was reformatted into different slice thickness and analyzed by edge-tracing. We performed two different forms of analysis of images with 3 mm slice thickness: edge-tracing and pixel by pixel. There was difference among the volumes obtained from different slice thickness (p < 0.001), and also difference between the two different methods of analysis (p < 0.05). The use of thick slices is time saving, but volumes are linearly increased; different methods of segmentation also yields different values. The form of volumetric analysis of the entorhinal cortex should be evaluated in advance to prevent false estimates in longitudinal studies.

Adult↗

Hypertrophic pachymeningitis: case report.

Hypertrophic pachymeningits is an unusual cause of neurological symptoms and is often secondary to infections, carcinomatosis or inflammatory diseases. It may also be idiopathic. We report a case of pachymeningitis which was manifested primarily by psychosis and visual loss with optic atrophy and destruction of nasal septum. The patient, a 45 year old woman was submitted to extensive investigation without evidence of any underlying disease. A meningeal biopsy was performed and showed a mostly unspecific inflammatory process with extensive fibrosis of the dura and few early stage granulomas. These findings suggest either neurosarcoidosis or idiopathic hypertrophic pachymeningitis.

Biopsy↗

Magnetic resonance imaging abnormalities in familial temporal lobe epilepsy with auditory auras.

BACKGROUND: Two forms of familial temporal lobe epilepsy (FTLE) have been described: mesial FTLE and FTLE with auditory auras. The gene responsible for mesial FTLE has not been mapped yet, whereas mutations in the LGI1 (leucine-rich, glioma-inactivated 1) gene, localized on chromosome 10q, have been found in FTLE with auditory auras. OBJECTIVE: To describe magnetic resonance imaging (MRI) findings in patients with FTLE with auditory auras. DESIGN AND METHODS: We performed detailed clinical and molecular studies as well as MRI evaluation (including volumetry) in all available individuals from one family, segregating FTLE from auditory auras. RESULTS: We evaluated 18 of 23 possibly affected individuals, and 13 patients reported auditory auras. In one patient, auditory auras were associated with déjà vu; in one patient, with ictal aphasia; and in 2 patients, with visual misperception. Most patients were not taking medication at the time, although all of them reported sporadic auras. Two-point lod scores were positive for 7 genotyped markers on chromosome 10q, and a Zmax of 6.35 was achieved with marker D10S185 at a recombination fraction of 0.0. Nucleotide sequence analysis of the LGI1 gene showed a point mutation, VIIIS7(-2)A-G, in all affected individuals. Magnetic resonance imaging was performed in 22 individuals (7 asymptomatic, 4 of them carriers of the affected haplotype on chromosome 10q and the VIIIS7[-2]A-G mutation). Lateral temporal lobe malformations were identified by visual analysis in 10 individuals, 2 of them with global enlargement demonstrated by volumetry. Mildly reduced hippocampi were observed in 4 individuals. CONCLUSIONS: In this family with FTLE with auditory auras, we found developmental abnormalities in the lateral cortex of the temporal lobes in 53% of the affected individuals. In contrast with mesial FTLE, none of the affected individuals had MRI evidence of hippocampal sclerosis.

Adolescent↗

Early development destructive brain lesions and their relationship to epilepsy and hippocampal damage.

Fifty-one consecutive adult patients with epilepsy and early development destructive brain lesions were divided into three main groups according to the topographic distribution of the lesion on magnetic resonance imaging: hemispheric (H) (n=9); main arterial territory (AT) (n=25) and arterial borderzone (Bdz) (n=17). Eight (89%) patients from group H presented status epilepticus in the first 5 years of life, five of them associated with fever. Seventeen of the 25 patients from group AT (76%) had an obvious hemiparesis observed early in life. In addition, major prenatal events were significantly more common in the group AT compared with the other two groups. Among patients from group Bdz, prenatal or postnatal events were not identified, except for one patient. Conversely, nine patients from group Bdz (60%) showed a history of perinatal complications. Hippocampal atrophy (HA) was determined by visual analysis in 74.5% of all patients and by volumetry in 92%. The frequency of HA was comparable among groups, but patients from group H presented the most severe atrophy and more frequent hyperintense T2 hippocampal signal. In conclusion, these three groups of patients with early destructive lesions and epilepsy (H, AT and Bdz), appear to have distinct pathogenic mechanisms. Our data show that there is a striking association of HA with different patterns of neocortical destructive lesions of early development. This association seems to be related to a common and synchronic pathogenic mechanism. The recognition of the pattern and degree of HA among these patients with intractable seizures may influence the surgical rationale.

Adult↗

Outcome of surgical treatment in familial mesial temporal lobe epilepsy.

PURPOSE: To describe postoperative outcome in patients with familial mesial temporal lobe epilepsy (FMTLE). METHODS: We studied FMTLE patients who underwent surgical treatment for refractory seizures. FMTLE was defined when at least two individuals in a family had a clinical EEG diagnosis of MTLE. Preoperative investigation included magnetic resonance imaging (MRI), interictal/ictal EEGs, and neuropsychological evaluation. We used Engel's classification for postoperative outcome. RESULTS: To date, 20 FMTLE patients have been operated on, with 1.6 to 9.8 years of follow-up (mean, 5.5 years). Hippocampal atrophy (HA) and other signs of mesial temporal sclerosis (MTS) were present in 18 patients (15 unilateral). Seizures were recorded in 19 patients. Seventeen (85%) patients are in class I. Two patients had normal hippocampal volumes (HcV): one (5%) is in class II and the other (5%) in class IV (extratemporal seizures developed after surgery). One (5%) patient had bilateral HA and is in class III. Qualitative histopathology showed MTS with different degrees of severity. CONCLUSIONS: Refractory FMTLE patients have good surgical outcome when unilateral or clearly asymmetric HA is identified. Preoperative investigation should be the same as that in patients with sporadic refractory MTLE.

Adult↗

Thalamic dysfunction in juvenile myoclonic epilepsy: a proton MRS study.

PURPOSE: To investigate neuronal dysfunction in the thalami of patients with juvenile myoclonic epilepsy (JME) by using proton magnetic resonance spectroscopy (MRS). METHODS: We performed single-voxel proton MRS over the right and the left thalami of 10 consecutive patients (five women) with JME (mean age, 31.6 years) and 10 age-matched healthy volunteers (five men). All patients had seizure onset in late childhood-teenage, normal neurologic examination, typical EEG of JME, and normal high-resolution MR imaging (MRI). We determined ratios of N-acetylaspartate (NAA) over creatine-phosphocreatine (Cr). Values <2 standard deviations from controls were considered abnormal. We performed analysis of variance to evaluate group differences. RESULTS: Group analysis showed that thalami NAA/Cr ratios were significantly decreased in JME patients (left side, 1.58 +/- 0.26; right side, 1.5 +/- 0.15) as compared with controls (left side, 1.98 +/- 0.18; right side, 1.88 +/- 0.15; p = 0.001 and p = 0.007, respectively). Individual analysis showed that nine of the 10 patients had abnormal NAA/Cr in at least one of the thalami. CONCLUSIONS: This study shows evidence of neuronal dysfunction in the thalami of patients with JME, which may have relevance for the mechanisms of seizure generation in this form of generalized epilepsy.

Adolescent↗

Texture analysis of hippocampal sclerosis.

UNLABELLED: Mesial temporal lobe epilepsy (MTLE) is frequently associated with refractory seizures and pathologic features of hippocampal sclerosis (HS). Quantitative magnetic resonance imaging (MRI) techniques can improve the detection and quantification of HS. The objective of this study was to evaluate whether MRI texture analysis can detect hippocampal abnormalities in patients with pathologically proven HS. METHODS: Nineteen consecutive patients who underwent surgery for refractory unilateral MTLE and had HS diagnosed on histopathology (12 right and seven left) had their preoperative MRIs evaluated. We performed texture analysis in 3-mm coronal T1-IR MRIs, focusing on the hippocampal head, by using the software MAZDA. Data were compared with those of a group of 78 normal hippocampi from 39 healthy adult volunteers through multivariate analysis of variance and selection of the most significant texture parameters. RESULTS: Overall, almost all parameters of texture could discriminate the group of hippocampi with HS and the group of contralateral hippocampi from the group of normal hippocampi, but the post hoc comparison showed no differences between HS and contralateral hippocampi. CONCLUSIONS: These results provide evidence of texture alteration in MRIs of hippocampi with HS and corroborate the hypothesis of bilaterality of hippocampal damage in patients with MTLE, but further studies are needed to investigate the lateralization power of texture analysis.

Adolescent↗

Quantification of hippocampal signal intensity in patients with mesial temporal lobe epilepsy.

BACKGROUND: Refractory mesial temporal lobe epilepsy (MTLE) is frequently associated with hippocampal atrophy (HA) and an abnormal hippocampal signal (Hsig) on magnetic resonance imaging (MRI). OBJECTIVE: To quantify Hsig on MRI using a simplified technique. METHODS: The authors included 15 patients with refractory MTLE who underwent surgery and had preoperative MRI with hippocampal volumetry. Hsig was quantified on preoperative coronal T1-weighted and T2-weighted MRI using the NIH-Image program. Hsig was determined for the head, tail, and entire hippocampal extension. Abnormal Hsig was defined when values were above (for T2) or below (for T1) 2 standard deviations from the mean of normal control group. RESULTS: The lateralization of abnormal Hsig values was concordant with electroencephalograms and HA in all patients. There was a significant difference in ipsilateral T2 Hsig between patients and controls (P < .0001), but not for contralateral T2 Hsig. T1 Hsig showed bilateral abnormalities more frequently, whereas T2 Hsig could lateralize better hippocampal abnormalities. Overall, there were no differences when comparing T2 Hsig for the entire hippocampus, head, or tail only. However, there were some individual differences. T2 Hsig abnormalities involved the entire hippocampus in most patients but were restricted to the head in one patient and to the tail in another. CONCLUSIONS: This simplified method for the quantification of Hsig using NHI-Image is an efficient method for the identification and quantification of hippocampal abnormalities in patients with MTLE. The assessment of the entire extension of the hippocampal formation may provide important additional data, compared to T2 relaxometry maps limited to one segment of the hippocampus.

Adult↗