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Fernando Cendes

Publications and source records attributed to Fernando Cendes.

At least 19 recordsLinked to original sources
PubMed2006

Longitudinal analysis of gray and white matter loss in patients with systemic lupus erythematosus.

Cerebral atrophy has been described to occur in systemic lupus erythematosus (SLE) with variable frequency. The aim of this study was to determine white and gray matter abnormalities in brain magnetic resonance imaging (MRI) of patients with SLE and to determine if these abnormalities progress over a one-year period. Seventy-five patients with SLE and 44 healthy age and sex-matched controls were enrolled in this study. T1-weighted volumetric images were used for voxel based morphometry (VBM) analyses. SLE patients exhibited a significant reduction in white matter and gray matter volume compared to controls (p=0.001). Follow-up images, after an average interval of 19 months, revealed a progressive white matter and gray matter atrophy (p=0.001). Reduced white and gray matter volume was associated with disease duration and the presence of antiphospholipid antibodies. Patients with severe cognitive impairment had a more pronounced white and gray matter reduction than patients with moderate cognitive impairment. Total corticosteroid dose was associated with gray matter reduction and not with white matter loss in SLE patients. We concluded that brain tissue loss associated with SLE is significant and progresses over a relatively short period of time. Disease duration, the presence of antiphospholipid antibodies and cognitive impairment were associated with white and gray matter loss. Corticosteroid was associated only with gray matter atrophy.

Adolescent↗
PubMed2006

Intrathecal immunoglobulin G synthesis and brain injury by quantitative MRI in multiple sclerosis.

OBJECTIVES: It was the aim of this study to evaluate if the quantitative intrathecal immunoglobulin G (IgG) synthesis correlates with the brain atrophy and the total lesion volume (TLV) in brain magnetic resonance imaging (MRI) of multiple sclerosis (MS) patients. METHODS: A total of 50 patients with relapsing-remitting MS were included in this study. MRIs were performed and cerebrospinal fluid samples were collected during the diagnostic determination when patients were in remission without treatment. RESULTS: At study baseline, IgG index values were elevated in 36 patients (72%), and oligoclonal IgG bands were positive in 42 of 50 patients (84%). Brain MRI was abnormal in 94% of patients, and, compared with healthy controls, brain atrophy was observed in MS patients. A positive correlation among IgG index, cerebrospinal fluid leukocyte count and TLV was observed; the Expanded Disability Status Scale correlated positively with TLV and the number of lesions, although a significant relationship between disability and brain atrophy was not demonstrated. CONCLUSIONS: Although new parameters will be necessary in longitudinal studies to characterize the axonal injury in various stages of the disease, the data suggest that the high intrathecal IgG synthesis may predict a greater brain lesion burden.

Adolescent↗
PubMed2006

Focal transient lesion in the splenium of the corpus callosum in three non-epileptic patients.

INTRODUCTION: We analyzed the imaging features of transient focal lesions in the splenium of the corpus callosum (SCC) in non-epileptic patients receiving antiepileptic drugs (AEDs). METHODS: We identified signal abnormalities in the SCC in three non-epileptic patients, all of them receiving AEDs. We examined two of these patients with multiplanar magnetic resonance (MR) imaging using 1.0-T equipment including fluid-attenuated inversion recovery (FLAIR), T2-weighted (TSE) and T1-weighted (SE) sequences before and after injection of contrast agent. The third patient was studied using 1.5-T equipment with the same sequences. Additionally, a T1 SE sequence with a magnetization transfer contrast pulse off resonance (T1 SE/MTC), diffusion-weighted imaging (EPI-DWI) and apparent diffusion coefficient (ADC) maps were obtained. RESULTS: We observed an identical pattern of imaging abnormalities in all patients characterized by round lesions, hyperintense on FLAIR and hypointense on T1 SE images, located in the central portion of the SCC. One lesion showed homogeneous gadolinium enhancement and perilesional vasogenic edema. This particular lesion showed restricted diffusion confirmed on the ADC map. This pattern was considered consistent with focal demyelination. Follow-up MR examinations showed complete disappearance or a clear reduction in lesion size. All patients had been treated with AEDs, but they did not show any clinical signs of toxicity, interhemispheric symptoms, or abnormal neurological findings (including seizures). CONCLUSION: We believe that our MR findings might be interpreted as transient lesions related to AED toxicity. They presumably resulted from focal demyelination in the central portion of the SCC.

Adult↗
PubMed2006

THE SCN2A gene is not a likely candidate for familial mesial temporal lobe epilepsy.

A transgenic mouse model carrying a mutation in the Scn2a gene showed chronic focal seizures associated with extensive cell loss and gliosis in the hippocampus, a similar phenotype found in familial mesial temporal lobe epilepsy (FMTLE). Our objective was to test whether the human homolog of the Scn2a gene is responsible for hippocampal abnormalities in FMTLE by linkage analysis. We conclusively ruled out the SCN2A gene as candidate in FMTLE.

Epilepsy, Temporal Lobe↗
PubMed2006

Gray matter atrophy associated with duration of temporal lobe epilepsy.

Hippocampal sclerosis is the most common abnormality associated with medial temporal lobe epilepsy (MTLE). Converging evidence supports that hippocampal sclerosis progresses with time. However, it is unclear whether extrahippocampal atrophy in patients with MTLE, similarly to hippocampal sclerosis, is an unremitting progressive process. In this article, we investigate the relationship between duration of epilepsy and gray matter concentration reduction in patients with MTLE within and outside the hippocampus. We employed a voxel-based morphometry study of MRI of the entire brain of 36 patients with drug refractory MTLE and 49 neurologically healthy age-matched controls. We performed a voxel-based parametric and nonparametric investigation of the association between gray matter concentration, age and duration of epilepsy. We complemented the investigation by extracting the gray matter concentration of regions of interest (ROIs) within the limbic system, and we investigated the association between the gray matter concentration on the ROIs and duration of epilepsy. Patients with MTLE exhibited gray matter concentration reduction that is negatively correlated with the duration of epilepsy within the ipsilateral hippocampus, temporal lobes as well as extratemporal limbic structures that are closely connected with the hippocampus. In conclusion, longer duration of refractory epilepsy was associated with a more intense hippocampal and extrahippocampal atrophy in patients with MTLE. The mechanism of progressive neuronal damage in MTLE may be related to active seizure activity within a limbic network, and early seizure control may prevent further brain atrophy in patients with refractory MTLE.

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

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↗
PubMed2006

Clinical presentation of juvenile Huntington disease.

OBJECTIVE: To describe the clinical presentation a group of patients with juvenile onset of Huntington disease. METHOD: All patients were interviewed following a structured clinical questionnaire. Patients were genotyped for the trinucleotide cytosine-adenine-guanine (CAG) repeat in the Huntington Disease gene. High resolution brain MRI was performed in all patients. RESULTS: We identified 4 patients with juvenile onset of disease among 50 patients with Huntington disease followed prospectively in our Neurogenetics clinic. Age at onset varied from 3 to 13 years, there were 2 boys, and 3 patients had a paternal inheritance of the disease. Expanded Huntington disease allele sizes varied from 41 to 69 trinucleotide repeats. The early onset patients presented with rigidity, bradykinesia, dystonia, dysarthria, seizures and ataxia. MRI showed severe volume loss of caudate and putamen nuclei (p=0.001) and reduced cerebral and cerebellum volumes (p=0.01). CONCLUSION: 8% of Huntington disease patients seen in our clinic had juvenile onset of the disease. They did not present with typical chorea as seen in adult onset Huntington disease. There was a predominance of rigidity and bradykinesia. Two other important clinical features were seizures and ataxia, which related with the imaging findings of early cortical atrophy and cerebellum volume loss.

Adolescent↗
PubMed2006

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↗
PubMed2006

Memory and language impairments and their relationships to hippocampal and perirhinal cortex damage in patients with medial temporal lobe epilepsy.

Chronic medial temporal lobe epilepsy (MTLE) is associated with memory loss due to damage in the hippocampal system. To investigate the relationship between volume of medial temporal lobe structures and performance on neuropsychological tests, we studied 39 consecutive patients with MTLE and unilateral hippocampal atrophy (HA) determined by volumetric magnetic resonance imaging (MRI). Structures of interest comprised hippocampus, amygdala, and entorhinal, perirhinal, parahippocampal, and temporopolar cortices. The findings indicated that (1) performance was significantly worse in the group with left HA as compared with the group with right HA on general memory, verbal memory, delayed recall, and verbal fluency tests and the Boston Naming Test (BNT), and (2) the volume of the left hippocampus and also the degree of asymmetry of perirhinal cortex volume were significant and independent predictors of performance on general memory, verbal memory, and verbal fluency tests and the BNT in patients with MTLE.

Adolescent↗
PubMed2006

Focal transient lesions of the corpus callosum in systemic lupus erythematosus.

Focal lesions limited to the splenium of the corpus callosum are rare and little is known about their etiology. We describe three patients with systemic lupus erythematosus (SLE) that presented transient lesions of the corpus callosum. We reviewed three patients with SLE whose magnetic resonance imaging (MRI) results revealed focal lesions in the splenum of corpus callosum. The medical records, including clinical, serological, and treatment features, were reviewed to determine the etiology of these lesions. Of 115 patients who had MRI for research purposes, three patients with focal nonhemorrhagic lesions of the corpus callosum were identified. All patients had active SLE at the time of MRI. One patient had other findings on MRI, including cerebral venous thrombosis. On follow-up MRI, patients had an inactive disease and the corpus callosum lesions disappeared. A transient lesion in the splenium of corpus callosum seems to be a nonspecific endpoint of different disease processes leading to vasogenic edema. The complete and rapid reversibility in all cases with disease control is emphasized and any invasive diagnostic or therapeutic approach is discouraged.

Adolescent↗
PubMed2006

Neurolupus.

Explore the source record for details and available documents.

Brain Diseases↗
PubMed2006

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↗
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

Amygdalae calcifications associated with disease duration in lipoid proteinosis.

OBJECTIVES: To describe imaging findings of central nervous system involvement in patients with lipoid proteinosis. METHODS: Patients with lipoid proteinosis followed in the dermatology and neurology departments of the State University of Campinas between 2002 and 2004 were evaluated using high-resolution MRI and computerized tomography (CT). RESULTS: We reviewed the charts of three patients (two women) with lipoid proteinosis with, ages ranging from 5 to 44 years. Symptomatic disease duration ranged from 2 to 39 years. MRI scans showed hypointense signal in FLAIR and T2 images in the amygdalae in two of three patients. Brain CT scans from two patients showed that MRI findings were calcifications. The calcification was more evident in patients with longer disease duration. Epilepsy was identified in one patient with longer disease duration. No other epileptogenic lesion was identified on MRI in this patient. CONCLUSION: Lipoid proteinosis is associated with bilateral amygdalae calcification. These findings were more evident in patients with longer disease duration. Epilepsy, when present, may be related to these calcifications. Patients with lipoid proteinosis should be followed with MRI/CT in order to identify these abnormalities.

Adult↗
PubMed2006

T2-weighted and T2 relaxometry images in patients with medial temporal lobe epilepsy.

PURPOSE: Quantification of increased T2-weighted MRI signal that is associated with hippocampal sclerosis (HS) can be performed through (1) mean of hippocampal signal in single-echo T2 MRI and (2) hippocampal T2 relaxometry. It is not clear whether these two techniques are equivalent. In this study, we compare the hippocampal signal, detected by single-echo T2 quantification and by T2 relaxometry, in patients with medial temporal lobe epilepsy (MTLE). METHODS: We studied magnetic resonance images from 50 MTLE patients and 15 healthy subjects. We compared the quantification of a T2 signal from single echo images to T2 relaxometry, both obtained from a manually traced region of interest (ROI) in coronal slices involving the whole hippocampus. Repeated measures ANOVA was used to evaluate the differences in the distribution of the Z-scores from single-echo T2 quantification and T2 relaxometry within subjects. RESULTS: We observed a significant difference between the measurements obtained from single-echo T2 quantification and T2 relaxometry (P < .001). Measurements from head, body, and tail of the hippocampus were different (P=.04), with a significant interaction between anatomic location and type of measurement used (P= .008). Post hoc paired comparisons revealed that T2 relaxometry yielded greater Z-scores for the body (P= .002) and tail (P < .0001). CONCLUSIONS: For each subject with MTLE, T2 relaxometry was able to detect a higher signal in the body and tail of the hippocampus compared to single-echo T2. This is a possible indicator that T2 relaxometry is more sensitive in detecting T2 abnormalities within the body and tail of the hippocampus in patients with MTLE.

Analysis of Variance↗
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↗