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Biomedical subjects

F Cendes

Publications and source records attributed to F Cendes.

At least 55 records · Page 3Linked to original sources

De novo psychogenic seizures after epilepsy surgery: case report.

The occurrence of de novo psychogenic seizures after epilepsy surgery is rare, and is estimated in 1.8% to 3.6%. Seizures after epilepsy surgery should be carefully evaluated, and de novo psychogenic seizures should be considered especially when there is a change in the ictal semiology. We report a patient with de novo psychogenic seizures after anterior temporal lobe removal for refractory temporal lobe epilepsy. Once psychogenic seizures were diagnosed and psychiatric treatment was started, seizures stopped.

Adolescent↗

[Familial partial epilepsies].

OBJECTIVE: To investigate the clinical and genetic characteristics of familial partial epilepsies. METHOD: Family history of seizures was questioned in all patients followed in our epilepsy clinics, from October 1997 to December 1998. Those with positive family history were further investigated and detailed pedigrees were obtained. All possibly affected individuals available underwent clinical evaluation. Seizures and epilepsy syndromes were classified according to the ILAE recommendations. Whenever possible, EEG and MRI were performed. RESULTS: Positive family history was identified in 32 unrelated patients. A total of 213 possibly affected individuals were identified, 161 of whom have been evaluated. The number of affected subjects per family ranged from two to 23. Temporal lobe epilepsy (TLE) was identified in 22 families (68%), frontal lobe epilepsy in one family (3%), partial epilepsy with centrotemporal spikes in five families (15%), and other benign partial epilepsies of childhood in four families (12%). Most of the affected individuals in the TLE families (69%) had clinical and/or EEG characteristics of typical TLE. However, the severity of epilepsy was variable, with 76% of patients with spontaneous seizure remission or good control with medication and 24% with refractory seizures, including 7 patients that underwent surgical treatment. In the other 10 families, we identified 39 possibly affected subjects, 23 of whom were evaluated. All had good seizure control (with or without medication) except for one patient with frontal lobe epilepsy. Pedigree analysis suggested autosomal dominant inheritance with incomplete penetrance in all families. CONCLUSION: Family history of seizures is frequent among patients with partial epilepsies. The majority of our families had TLE and its expression was not different from that observed in sporadic cases. The identification of genes involved in partial epilepsies may be usefull in classification of syndromes, to stablish prognosis and optimal treatment.

Adolescent↗

Congenital destructive hemispheric lesions and epilepsy: clinical features and relevance of associated hippocampal atrophy.

We studied the clinical, EEG and MRI findings in 19 patients with epilepsy secondary to congenital destructive hemispheric insults. Patients were divided in two groups: 10 with cystic lesions (group 1), and 9 with atrophic lesions (group 2). Seizure and EEG features, as well as developmental sequelae were similar between the two groups, except for the finding that patients of group 2 more commonly presented seizures with more than one semiological type. MRI showed hyperintense T2 signal extending beyond the lesion in almost all patients of both groups, and it was more diffuse in group 2. Associated hippocampal atrophy (HA) was observed in 70% of group 1 patients and 77.7% of group 2, and it was not correlated with duration of epilepsy or seizure frequency. There was a good concordance between HA and electroclinical localization. The high prevalence of associated HA in both groups suggests a common pathogenesis with the more obvious lesion. Our findings indicate that in some of these patients with extensive destructive lesions, there may be a more circumscribed epileptogenic area, particularly in those with cystic lesions and HA, leading to a potential rationale for effective surgical treatment.

Adolescent↗

Cysticidal therapy: impact on seizure control in epilepsy associated with neurocysticercosis.

OBJECTIVE: To evaluate the clinical features and seizure control of epilepsy related to neurocysticercosis. METHOD: 18 patients with partial epilepsy and neurocysticercosis were treated with albendazol or praziquantel and followed from 3 months to 12 years. We analyzed results from the CSF exam, interictal electroencephalogram (EEG), head computerized tomography and/or magnetic resonance imaging. RESULTS: The patients' mean age was 36.4 years. The mean duration of epilepsy was 16 years. 83% patients had simple partial seizures; 17% had complex partial seizures. All patients underwent routine EEGs: 62% had abnormalities and 38% were normal. A relationship was observed between focal EEG abnormality and the location of cyst in 28% of the patients. The CSF exams showed pleocytosis in 33% of the patients, and 28% had elevated protein levels. Only 22% of patients had positive titer for cysticercosis in the CSF. In all patients who had somatosensory and special sensory seizures there was a relationship between location of the cysts and seizure semiology (n=11). After cysticidal therapy, 83% patients had a significant improvement in controlling seizures. CONCLUSION: In this group, we found a predominance of simple partial seizures and a relationship between somatosensory and special sensory seizures and the location of the cysts. Cysticidal therapy was effective in controlling seizures in these patients and should be considered for patients with partial seizures and semiology related to cyst location.

Adult↗

Prognostic value of proton magnetic resonance spectroscopic imaging for surgical outcome in patients with intractable temporal lobe epilepsy and bilateral hippocampal atrophy.

The objective of this study was to assess which features of temporal lobe proton magnetic resonance spectroscopic imaging (1H-MRSI) are associated with satisfactory surgical outcome in patients with intractable temporal lobe epilepsy and bilateral hippocampal atrophy. We studied 21 patients with intractable temporal lobe epilepsy and bilateral hippocampal atrophy defined by magnetic resonance imaging volumetric measurements who underwent surgical treatment. 1H-MRSI was used to determine the relative resonance intensity ratio of the neuronal marker N-acetylaspartate to creatine + phosphocreatine (NAA/Cr) for mid and posterior temporal lobe regions of the left and right hemisphere, as well as an asymmetry index. Values lower than 2 SDs below the normal mean were considered abnormal. We used Engel's classification to assess surgical outcome with respect to seizure control. Eleven patients (52%) were in class I-II and 10 (48%) were in class III-IV. All 21 were operated on the side of maximal electroencephalographic (EEG) lateralization. Concordant lateralization of decreases in NAA/Cr to the side of surgery and normal NAA/Cr values in the contralateral posterior-temporal region were significantly associated with good surgical outcome: 11 (69%) of 16 patients with 1H-MRSI abnormalities concordant with EEG lateralization and none of the 5 patients with nonconcordant 1H-MRSI had a good outcome (class I-II); 10 (77%) of 13 patients with normal NAA/Cr contralateral to the EEG lateralization versus 1 (12.5%) of 8 of those with NAA/Cr reduction contralateral to EEG lateralization were in class I-II. Regression correlation analysis showed significant linear correlation between the midtemporal NAA/Cr relative asymmetry ratio and surgical outcome; the greater the asymmetry, the better the outcome. We conclude that discriminant 1H-MRSI features associated with favorable surgical outcome in patients with temporal lobe epilepsy and bilateral hippocampal atrophy were (1) concordant 1H-MRSI lateralization, (2) a greater side-to-side asymmetry of NAA/Cr, and (3) an absence of contralateral posterior NAA/Cr reduction.

Adolescent↗

Familial perisylvian polymicrogyria: a new familial syndrome of cortical maldevelopment.

Two familial X-linked dominant syndromes of cortical maldevelopment have recently been described: double cortex/lissencephaly syndrome and bilateral periventricular nodular heterotopia. We report on 12 kindreds with familial perisylvian polymicrogyria (FPP) presenting at 10 centers, examine the clinical presentation in these familial cases, and propose a possible mode of inheritance. The clinical and radiological pattern was variable among the 42 patients, with clinical differences among the families and even within members of the same family. Pseudobulbar signs, cognitive deficits, epilepsy, and perisylvian abnormalities on imaging studies were not found in all patients. When present, they displayed a spectrum of severity. The only clear correlation in this study was between bilateral imaging findings and abnormal tongue movements and/or pronounced dysarthria. Most of the families provided evidence suggestive of, or compatible with, X-linked transmission. On the other hand, the pedigrees of 2 families ruled out X-linked inheritance. The most likely mode of inheritance for these 2 families was autosomal dominant with decreased penetrance; however, autosomal recessive inheritance with pseudodominance could not be ruled out in 1 family. We conclude that FPP appears to be genetically heterogeneous. However, most of the families probably represent a third previously undescribed X-linked syndrome of cortical maldevelopment.

Adolescent↗

Neuroimaging evidence of progressive neuronal loss and dysfunction in temporal lobe epilepsy.

Whether temporal lobe epilepsy is the result of an isolated, early injury or whether there is ongoing neuronal dysfunction or loss due to seizures is often debated. We attempt to address this issue by using magnetic resonance techniques. Proton magnetic resonance spectroscopic imaging can detect and quantify focal neuronal dysfunction or loss based on reduced signals from the neuronal marker N-acetylaspartate (NAA), and magnetic resonance imaging (MRI)-based measurements of hippocampal volumes (MRIvol) can quantify the amount of atrophy in this structure. We performed magnetic resonance spectroscopic imaging and MRIvol in 82 consecutive patients with medically intractable temporal lobe epilepsy to determine whether there was a correlation between seizure frequency, or type or duration of epilepsy, with NAA to creatine (Cr) values or hippocampal volumes. Volumes and spectroscopic resonance intensities were categorized as to whether they were measured from the temporal lobe ipsilateral or contralateral to the predominant electroencephalographic focus. Ipsilateral and contralateral NAA/Cr was negatively correlated with duration of epilepsy. Hippocampal volumes were negatively correlated with duration ipsilaterally but not contralaterally. Frequency of complex partial seizures was not correlated with any of the magnetic resonance measures. However, patients with frequent generalized tonic-clonic seizures had lower NAA/Cr bilaterally and smaller hippocampal volumes ipsilaterally than patients with none or rare generalized tonic-clonic seizures. The results suggest that although an early, fixed injury may cause asymmetric temporal lobe damage, generalized seizures may also cause progressive neuronal dysfunction or loss.

Adult↗

Diagnosis of subtle focal dysplastic lesions: curvilinear reformatting from three-dimensional magnetic resonance imaging.

Focal cortical dysplasia is a frequent cause of medically intractable partial epilepsy. These lesions are being increasingly identified by high quality images provided by magnetic resonance imaging (MRI), resulting in improved seizure control of surgically treated patients. Small dysplastic lesions are often missed by conventional MRI methods. The identification of subtle structural abnormalities by rectilinear slices is often limited by the complex convolutional pattern of the brain. We developed a method of curvilinear reformatting of three-dimensional MRI data that improves the anatomical display of the gyral structure of the hemispheric convexities. It also reduces the asymmetric sampling of gray-white matter that may lead to false-positive results. We present 5 patients in whom conventional two-dimensional and three-dimensional MRI with multiplanar reformatting was initially considered normal. Subsequent studies using curvilinear reformatting identified lesions in all. Four patients underwent surgery with histological diagnosis of focal cortical dysplasia. Three patients are seizure-free and 1 had significant improvement in seizure control. These results indicate that an increase in the detection of subtle focal dysplastic lesions may be accomplished when one improves the anatomical display of the brain gyral structure by performing curvilinear reformatting.

Adolescent↗

Surgical outcome in patients with epilepsy and dual pathology.

High-resolution MRI can detect dual pathology (an extrahippocampal lesion plus hippocampal atrophy) in about 5-20% of patients with refractory partial epilepsy referred for surgical evaluation. We report the results of 41 surgical interventions in 38 adults (mean age 31 years, range 14-63 years) with dual pathology. Three patients had two operations. The mean postoperative follow-up was 37 months (range 12-180 months). The extrahippocampal lesions were cortical dysgenesis in 15, tumour in 10, contusion/infarct in eight and vascular malformation in five patients. The surgical approach aimed to remove what was considered to be the most epileptogenic lesion, and the 41 operations were classified into lesionectomy (removal of an extrahippocampal lesion); mesial temporal resection (removal of an atrophic hippocampus); and lesionectomy plus mesial temporal resection (removal of both the lesion and the atrophic hippocampus). Lesionectomy plus mesial temporal resection resulted in complete freedom from seizures in 11/15 (73%) patients, while only 2/10 (20%) patients who had mesial temporal resection alone and 2/16 (12.5%) who had a lesionectomy alone were seizure-free (P < 0.001). When classes I and II were considered together results improved to 86, 30 and 31%, respectively. Our findings indicate that in patients with dual pathology removal of both the lesion and the atrophic hippocampus is the best surgical approach and should be considered whenever possible.

Adolescent↗

Clinical patterns of patients with temporal lobe epilepsy and pure amygdalar atrophy.

PURPOSE: MRI volumetric measurements (MRIvol) have been proven reliable in determining mesial temporal atrophy in patients with TLE. We attempted to correlate the clinical features with different patterns of hippocampal formation (HF) and amygdala (AM) atrophy in patients with TLE without foreign tissue lesion. METHODS: We studied 65 patients with refractory TLE. They were divided into five groups according to MRIvol results: pure AM atrophy (n = 11, 10 unilateral and one bilateral), unilateral HF atrophy (n = 16), bilateral HF atrophy (n = 12), unilateral AM + HF atrophy (n = 13), and patients with normal volumes of AM and HF (n = 13). MRIvol of AM and HF were performed by using a protocol previously described by Watson et al. (Neurology 1992;42:1743-50). RESULTS: Patients with AM atrophy had later onset of seizures compared with those with unilateral HF atrophy (p < 0.01). History of febrile convulsions (p < 0.0001) and frequent secondarily generalized tonic-clonic seizures (GTCSs) were more often found in patients with HF atrophy compared with those with pure AM atrophy and those with normal volumes (p = 0.04). Prolonged postictal confusion was more often found with AM atrophy (p = 0.05). Memory impairment was more severe in patients with HF atrophy than in those with AM atrophy only or in those with normal volumes (p = 0.03). There were no significant differences among the five groups in the following parameters: age, duration of epilepsy, seizure frequency, and presence and type of aura. CONCLUSIONS: Prolonged postictal confusion appeared to be related to AM atrophy, in keeping with previous clinical observations. These patients also had a lower incidence of early febrile convulsions, older age at epilepsy onset, lower frequency of secondary GTCS, and lesser memory dysfunction compared with patients with hippocampal atrophy.

Adolescent↗

EEG background delta activity in temporal lobe epilepsy: correlation with volumetric and spectroscopic imaging.

PURPOSE: With quantitative electroencephalogram (EEG) and neuroimaging methods, we examined delta activity, atrophy, and neuronal-axonal dysfunction of the cerebral gray and white matter in patients with intractable temporal lobe epilepsy (TLE). Based on evidence that lesions of the white matter result in EEG delta activity, we postulated that background abnormalities in patients with TLE are related to changes of the temporal lobe white matter. METHODS: We measured interictal delta activity in 34 TLE patients and 10 controls. Spike-free and artifact-free EEG samples were selected by visual inspection. A spectral analysis was used to compute the energy in the delta frequency band. We compared the results of the spectral analysis to magnetic resonance imaging- (MRI) based volumes of the temporal lobe white and gray matter, the hippocampus and the amygdala; and N-acetyl aspartate (NAA) in the lateral and posterior temporal lobe by using proton magnetic resonance spectroscopic imaging (1H-MRSI). The degree of correlation between delta activity and the neuroimaging measurements was assessed by using the Pearson correlation coefficient (r). An analysis of variance (ANOVA) was used to examine the influence of the seizure-focus lateralization on the delta activity and the neuroimaging parameters. RESULTS: There was no significant difference in the amount of delta activity in the temporal lobe between the controls and patients. We found no correlation between delta activity and the neuroimaging measures (p>0.05). The ANOVA showed significant differences between the patients and controls for the volume of the gray and white matter of the temporal lobe and for the NAA in the lateral and posterior temporal lobe (p<0.002). CONCLUSIONS: The interictal background delta activity was not explained by reduced volume of the temporal lobe white matter, gray matter, or by abnormalities seen in 1H-MRSI.

Adult↗

Dyskinesia induced by phenytoin.

UNLABELLED: Phenytoin is an effective antiepileptic drug, although, it can be associated with many side effects, including dyskinesia. OBJECTIVE: To describe the clinical characteristics of phenytoin induced dyskinesia. METHODS: We investigated the occurrence of involuntary movements in patients followed at our adult and pediatric epilepsy clinics during the period of one year. RESULTS: Three patients presented with phenytoin-induced dyskinesia: one adult with axial and orofacial dyskinesia, and two children with choreoathetosis. They did not have other signs of phenytoin intoxication and had complete recovery after phenytoin withdrawal. CONCLUSION: Phenytoin induced dyskinesia may occur during either chronic or initial treatment and with normal serum phenytoin levels. However, it occurs most often in patients on polytherapy, usually after increasing dosage and with toxic serum levels. Other signs of phenytoin intoxication may be present in these patients, but often the dyskinesia is the only side effect, which may delay the diagnosis and treatment. The clinical characteristics of the involuntary movements vary and may be focal or generalized, most often characterized by choreoathetosis and dyskinesias. These may last for hours, days or even years, but frequently disappear completely after phenytoin withdrawal.

Anticonvulsants↗

[Magnetic resonance imaging and clinical features in adults with monophasic demyelinating disease. Acute disseminated encephalomyelitis or a variant of multiple sclerosis?].

Acute disseminated encephalomyelitis (ADEM) is a widespread monophasic inflammatory disease affecting the central nervous system, that usually follows an infection or vaccination. In this study, we present an analysis of magnetic resonance imaging (MRI), cerebrospinal fluid (CSF) and clinical aspects in four patients with clinical diagnosis of ADEM. The presence of MRI demyelinating lesions was crucial, but not in itself sufficient for definitive diagnosis. Clinical and MRI follow up, in order to exclude new lesions and to reevaluate the former ones, as well as CSF, were important for the differential diagnosis with other demyelinating diseases, particularly multiple sclerosis. In addition, we have shown that early treatment with methylprednisolone after the initial symptoms was effective for improving clinical manifestations as well as for reducing MRI lesions.

Acute Disease↗

Spectroscopic imaging of frontal neuronal dysfunction in hyperekplexia.

We used proton magnetic resonance spectroscopic imaging (MRSI) to assess in vivo cortical neuronal involvement in hyperekplexia. Cerebral neuronal function was measured using proton MRSI in four unrelated patients with hyperekplexia and 20 healthy controls. All patients had the major form of hyperekplexia, with additional atypical clinical features in two of them. Family history was positive in three patients and absent in one. The neuronal marker N-acetylaspartate (NAA), choline-containing compounds (Cho) and creatine (Cr) were measured in frontal, central and parietal areas. The MRSI showed a reduction of the relative resonance intensity of NAA/(Cr + Cho) in frontal and central regions in three patients, and in the right frontal region of the fourth. In one patient a second MRSI showed normal relative NAA resonance intensities over both temporal lobes as well as in the brainstem. In two subjects the topography of EEG abnormalities in the frontal lobes coincided with the MRSI findings. This proton MRSI study indicates the presence of frontal neuronal dysfunction in hyperekplexia. Whether this represents cortical dysfunction or an epiphenomenon of diencephalic or brainstem abnormalities remains open. However, the observation of normal proton MRSI in the temporal regions and brainstem in one of the patients seems to concur with the hypothesis of a facilitatory role of cortical dysfunction within areas of sensorimotor representation in the generation of the pathological startle reaction in hyperekplexia.

Adolescent↗

Intracranial aneurysms and sickle cell anemia: multiplicity and propensity for the vertebrobasilar territory.

OBJECTIVE: We describe a case of sickle cell anemia and multiple intracranial aneurysms and review the English-language-reported cases of sickle cell disease associated with intracranial aneurysms proven angiographically or by autopsy, to assess whether there are associations with aneurysm multiplicity and sites of aneurysm occurrence. CLINICAL PRESENTATION: A 28-year-old woman with sickle cell disease and a subarachnoid hemorrhage underwent successful clipping of three intracranial aneurysms. RESULTS: Among 44 reviewed cases, 57% of patients demonstrated multiple aneurysms, and aneurysms from patients with multiple aneurysms comprised nearly 80% of the total number of aneurysms. There were, on average, three aneurysms per patient for patients with multiple aneurysms. There was a predominance of female patients (female/male ratio, 1.6:1), although there existed no significant differences in age or gender for patients with single or multiple aneurysms. None of the patients with multiple aneurysms was older than 40 years of age at the time of presentation. Patients with multiple aneurysms and sickle cell disease showed a significant difference in the distribution of the aneurysm sites, with a significantly large number occurring in the vertebrobasilar axis. Multiple aneurysms associated with sickle cell disease showed a higher rate of simultaneous occurrence in the posterior and anterior circulation, compared with multiple aneurysms in the general population. CONCLUSION: There are strong statistical associations for aneurysm multiplicity and sites of aneurysm occurrence among reported patients with sickle cell disease. Patients with sickle cell anemia and neurological symptoms should undergo magnetic resonance angiography or four-vessel angiography to detect potentially harmful, but neurosurgically treatable, pathological conditions.

Adolescent↗

Proton magnetic resonance spectroscopic imaging in patients with extratemporal epilepsy.

PURPOSE: Reduced levels of N-acetylaspartate (NAA) in temporal lobes responsible for temporal lobe epilepsy have been observed consistently in proton magnetic resonance spectroscopy (MRS) studies. METHODS: We investigated the potential of proton MRS to detect low NAA outside of the temporal lobes in patients with non-lesional partial extratemporal epilepsy. Proton MR spectroscopic imaging (MRSI) data of both frontal lobes and central/postcentral regions were obtained in 20 such patients and 16 normal control subjects. The epileptogenic region was determined by an extensive clinical-EEG investigation, including the recording of habitual seizures in each patient, and intracranial EEG recordings in 10 patients. RESULTS: The relative NAA resonance intensities (i.e., NAA/phosphocreatine plus creatine (CR(t)), NAA/choline-containing metabolites (Cho(t)) and NAA/Cr(t) + Cho(t)), were all significantly reduced throughout the spectroscopic image as compared with that of the controls. Furthermore, reduction of the NAA ratios was greater in the epileptogenic region as compared with the nonepileptogenic regions, on EEG investigation. CONCLUSIONS: In vivo proton MRSI of patients with nonlesional partial extratemporal epilepsy detected evidence of widespread neuronal damage or dysfunction that was greatest in the region of seizure focus.

Adult↗