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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report clinical, EEG and neuroimaging findings of three patients in two Italian families with merosin-negative congenital muscular dystrophy (CMD), drug-resistant occipital epilepsy, diffuse persistent cerebral white matter changes and focal cortical dysplasia. Clinical and epilepsy histories, EEG and neuroimaging findings were very similar in all patients. Seizures started in childhood and mainly consisted of periodic spasms, a particular type of partial seizure characterized by clusters of epileptic spasms. The motor expression of the spasms was very mild so that they had been frequently missed or misinterpreted as non-convulsive generalized absence seizures. Interictal EEG showed occipital spike-waves and bilateral synchronous slow spike-wave discharges. Ictal EEG showed prolonged periodic sequences of slow waves with associated fast rhythm complexes, characteristic of periodic spasms. Two patients had normal intelligence, one patient presented moderate mental retardation. Focal cortical dysplasia in the posterior areas of the brain, in addition to marked diffuse white matter alterations, was detected in the magnetic resonance images of all patients. Findings in these patients indicate that in merosin-negative CMD brain involvement can include cortical dysplasia, in addition to white matter changes. In such cases the brain damage can lead to a childhood-onset localization-related symptomatic occipital epilepsy. Epileptic seizures and cortical dysplasia can be, however, difficult to detect in CMD. The clinical semiology of epileptic seizures may in fact be modified because of muscular weakness. This implies that epilepsy may be misdiagnosed or even missed and EEG-polymyographic recordings may be necessary to identify it. Similarly, cortical dysplasia may be very localized and visible by neuroimaging only if it is carefully investigated on the basis of epileptological and EEG-polymyographic findings.
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PMID:Merosin-negative congenital muscular dystrophy, occipital epilepsy with periodic spasms and focal cortical dysplasia. Report of three Italian cases in two families. 887 53

We present the morphology and the laminar distribution of the serotonin (5-hydroxytryptamine, 5-HT) innervation of the cerebral cortex of patients who underwent cortical resection for partial seizures. The limits of the resections were established by stereoelectroencephalography. The 5-HT innervation was mapped by using an antiserum anti-5-HT. Two patients had cryptogenic epilepsies and two others had seizures related to focal cortical dysplasia. 5-HT immunoreactive axons were morphologically heterogeneous and projected diffusely to the cerebral cortex with regional-specific densities. Two types of terminal axon were demonstrated. Type I had large and spherical (intensely immunoreactive) varicosities and was distributed sparsely with a characteristic predominance in the molecular layer. Type II had fine and pleiomorphic varicosities (granular or fusiform) and was distributed through all cortical layers. The distribution of the 5-HT innervation varied according to the different architectonic areas investigated. The granular cortical areas characterized by a highly developed layer IV (primary somatosensory, primary visual and prefrontal cortices) had the highest density of 5-HT-ir fibers distributed from layer I to layer V. The agranular primary motor cortex had the lowest density with fibers preferentially seen in layers I, IIIa and V-VI. The orbital cortex with a poorly defined layer IV had an intermediate density with a laminar repartition predominant in the supragranular layers. In patients with cryptogenic epilepsies, the brain epileptogenic tissue was histologically normal as well as the serotonergic innervation. In contrast, in patients with focal cortical dysplasia, the dysplastic epileptogenic tissue was characterized by a serotonergic hyperinnervation. In agreement with previous data in primates, we give morphological evidence for two morphologically distinct serotonergic subsystems and for regional specific densities in the human cerebral cortex. Moreover, we previously reported an altered pattern of the catecholaminergic innervation in the same dysplasia areas. All these results provide evidence that this development epileptogenic lesion involves several sets of neurons which may contribute to epileptogenic activity.
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PMID:The serotonergic innervation of the cerebral cortex in man and its changes in focal cortical dysplasia. 888 67

It is currently believed that there are two subsets of septo-optic dysplasia (de Morsier's syndrome), one with schizencephaly and the other without schizencephaly. Also, some authors consider septo-optic dysplasia as a mild form of holo-prosencephaly. This article describes a third form of the anomaly which is associated with total callosal absence. Two patients similar to this one have previously been reported. None of these three patients had interhemispheric fusion in any form, excluding holoprosencephaly. They had normal facies. All of them presented with seizures, and no hormonal abnormality was found. This new clinicoradiological type of the anomaly is suggested to be labelled as calloso-septo-optic or calloso-optic dysplasia.
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PMID:Septo-optic dysplasia (de Morsier's syndrome) associated with total callosal absence. A new type of the anomaly. 894 59

Despite the increasing number of patients affected by neuronal migration disorders (NMDs) recently diagnosed in vivo by means of magnetic resonance imaging (MRI), few detailed data on the correlation between the neuroradiological and the anatomical features in the single NMD case are available. The present paper reports a combined cytoarchitectural and immunocytochemical analysis, by means of antisera recognizing specific neuronal and glial markers, of three MRI diagnosed NMD patients surgically treated for the relief of intractable seizures. The first case was a giant subcortical nodular heterotopia of morphologically normal neurons lacking any type of cortical lamination. The second case was a layered polymicrogyria with an abnormal amount of ectopic neurons in the underlying white matter. The third case was a focal cortical dysplasia characterized by a dramatic disruption of the normal cortical layering associated with marked cytological abnormalities. The present data demonstrate that the macroscopical and microscopical brain abnormalities can be markedly different in different NMD subtypes, and suggest that different anatomical substrates can underlie the intrinsic hyperexcitability of these brain malformations. The relevance of further prospective clinico-morphological studies for a better understanding of the mechanisms determining the development of these brain malformations is underlined.
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PMID:Neuronal migration disorders and epilepsy: a morphological analysis of three surgically treated patients. 898 86

Clinical and electroencephalographic (EEG) studies were performed in nine patients with cerebral cortical dysplasia (CD). Interictal single photon emission computed tomography (SPECT) using 99mTc-hexamethyl propylene amine oxime were studied in four patients. A patient with hemimegalencephaly and agyria had poor outcome in both developmental and epileptic aspects. The prognoses of clinical seizures were generally related to the severity, location and size of CD. The size of the lesion was not always correlated with the clinical seizure outcome. Four patients had focal pachygyria. Clinical pictures were diverse in these patients. One patient underwent callosotomy for the control of intractable seizures. The others had no clinical seizures despite of the appearance of paradoxical discharges in the area of pachygyria. The distribution of CD detected by MRI did not always correlate with that of paradoxical discharges in EEG and/or hypoperfusional areas seen in SPECT. These findings suggest that a detailed neuroimaging study is useful to elucidate the epileptogenesis in patients with CD, and that all the cortical abnormalities in patients with intractable epilepsy are not detected by MRI.
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PMID:[Cerebral cortical dysplasia associated with epilepsy: MRI and clinical aspects]. 898 96

The dysembryoplastic neuroepithelial tumor (DNT) is a rare, recently recognized neoplasm, characterized by a mixed glial-neuronal cell proliferation. We studied 11 cases of DNT including 6 males and 5 females (age range 2.1-39.4 years, mean 13.8 years). All patients presented with chronic epilepsy of 4.8 years mean duration. Nine tumors were located in the temporal lobe and 2 in the frontal lobe. Four tumors were located on the right side and 7 on the left. All tumors were characterized by a predominant oligodendrocyte-like cell component with intermixed normal appearing neurons and astrocytes. All were focally microcystic and 7 of 9 evaluable tumors were multinodular. Other observed histologic features included a focal arcuate vascular pattern in 7 tumors, calcification in 4 tumors, and a rare mitotic figure in 2 tumors. Necrosis and vascular hyperplasia were not observed in any of the tumors. Cortical architectural disorganization (cortical dysplasia) was observed in 9 of 10 evaluable cases. MIB1 (a marker of cellular proliferation) immunostaining was performed in all 11 tumors and MIB1 indices (number of positive tumor cells/100 tumor cells counted) ranged from 0-0.6 (mean 0.2). Four patients required at least 1 additional surgical procedure for tumor recurrence, related to an incomplete initial excision 2.1-4.4 years after their initial operation. All patients are seizure-free at their last follow-up. DNTs are low grade neoplasms which typically present with chronic epilepsy, are most frequently located in the temporal lobe, and occur most frequently in pediatric patients. Their appearance and association with cortical dysplasia suggest a maldevelopmental origin. The generally slow growth and relatively benign nature of DNT is reflected by their low MIB1 index.
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PMID:Dysembryoplastic neuroepithelial tumor: a clinicopathologic and immunohistochemical study of 11 tumors including MIB1 immunoreactivity. 899 57

The clinical characteristics of seizures in adults and children with localization-related epilepsy have been clearly described and classified, but few data are available based on video EEG studies of postneonatal infants under 2 years of age. We analyzed 125 videotaped seizures from 23 infants aged 2 to 24 months with localization-related epilepsy defined by localized ictal EEG or localized lesion on neuroimaging with seizure-free surgical outcome. Seizure symptomatology was classified based on observable behavioral and motor manifestations and then correlated with location of the epileptogenic zone. Seizures characterized by decrease in behavioral motor activity with indeterminate level of consciousness and minimal or no automatisms ("hypomotor" seizures) arose from temporal, temporoparietal, or parieto-occipital regions (7 patients). Seizures with localized or bilateral clonic, tonic, or atonic motor phenomena arose predominantly from frontal, frontocentral, central, or frontoparietal areas (12 patients). One patient had versive seizures arising from the contralateral occipital lobe, 2 patients had infantile spasms (one with a frontal tumor, one with temporo-parieto-occipital dysplasia), and one patient had unclassifiable seizures. Disruption of temporal or temporoparietal function resulted primarily in diminution of behavioral activity, whereas ictal activation of motor areas during frontal or central onset seizures resulted mainly in localized or generalized motor phenomena. Infantile spasms occurred because of lesions in either location. Using an approach based on easily observable behavioral and motor phenomena, it was possible to classify the seizures in all but one infant.
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PMID:Seizure symptomatology in infants with localization-related epilepsy. 900 17

Hamartomas are a poorly defined group of lesions and a rare cause of chronic epilepsy. We studied 13 patients, nine males and four females, whose cause of seizures was attributed to a hamartoma. The patients ranged in age from 6 to 33 years (mean, 21 years). Seizure duration before surgery ranged from 1.5 to 22 years (mean, 10 years). Seven hamartomas were located on the right side and six on the left. Six were located in the frontal lobe, five in the temporal lobe, and two in the occipital lobe. Twelve patients underwent gross total resection of the lesion and one a partial resection. All consisted of a circumscribed, disorganized collection of glial cells, primarily astrocytes. Rarely a neuronal component was admixed. One lesion contained an increased number of small blood vessels. Eight (62%) hamartomas contained eosinophilic granular bodies, and focal microcalcification was observed in three lesions (23%). Adjacent cortical architectural abnormalities (cortical dysplasia) were identified in eight (62%) resection specimens. Necrosis, mitoses, and prominent cytological atypia were absent in all lesions. Differential diagnostic considerations include low grade astrocytoma, ganglioglioma, dysembryoplastic neuroepithelial tumor, and cortical dysplasia. Postoperatively, 10 patients (77%) had complete resolution or greater than 90% reduction of seizure frequency. Two patients (16%) developed recurrent seizures 8 and 13 months postoperatively. One patient who underwent a partial resection showed no decrease in seizure frequency. No lesion recurrence on imaging studies has been observed in the 12 patients who underwent gross total resection of their hamartoma during 1 to 51 months (mean, 14 months) follow-up. We conclude that hamartomas seen in the setting of chronic epilepsy are generally low-grade lesions that respond well to gross total resection. Circumscription and lack of significant cytological atypia help distinguish these lesions from other neoplastic causes of epilepsy. Hamartomas that arise in the setting of chronic epilepsy appear to be associated with increased incidence of cortical architectural abnormalities (cortical dysplasia) and represent maldevelopmental lesions.
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PMID:Hamartomas in the setting of chronic epilepsy: a clinicopathologic study of 13 cases. 902 7

We retrospectively studied 32 patients who had cortical dysplasia and epilepsy. Cortical dysplasia was classified into diffuse cortical dysplasia (8 patients), bilateral localized cortical dysplasia (5), unilateral diffuse cortical dysplasia (2), and focal cortical dysplasia (17). The onset of epilepsy was younger in patients with more widespread lesions. At the onset, patients with bilateral lesions generally had symptomatic generalized epilepsy, while those with unilateral cortical dysplasia tended to have symptomatic localization-related epilepsy. In patients with focal cortical dysplasia, however, 4 patients had West syndrome, either at the onset of epilepsy or during the follow up period. Seizure outcome was poor in any type of cortical dysplasia. An evaluation of prognostic factors in patients with focal cortical dysplasia did not show any of statistical significance, including gender, age at onset of epilepsy, psychomotor delay and the presence of high intensity areas in T2-weighted MRI.
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PMID:[Neuronal migration disorders and epilepsy]. 907 Nov 90

We conducted corticectomy in twenty-five patients with intractable partial epilepsy due to focal cortical dysplasia (FCD). MRI could not detect FCDs in three patients, interictal SPECT, however, revealed hypoperfusion corresponding to FCDs in two of these patients, while the FCD in one remaining patient was histologically identified in a resected specimen. The location of FCDs was as follows: the frontal lobe in sixteen patients, the temporal in five, the occipital in two, fronto-parietal in one, and the temporo-parietal in one. Prior to the surgery, twenty-one patients underwent invasive long-term intracranial EEG/CCTV monitoring. Of the fourteen patients who were tracked for longer than 2 years following surgery, eleven belonged to Class I according to Engel's criteria, two to Class II, and one to Class III. We compared intracranial EEG findings (interictal and ictal) between these twenty-one patients and eight patients with frontal lobe epilepsy resulting from different lesions. The results of this comparison, together with the seizure outcome following surgery, indicated that FCD is intrinsically epileptogenic. The invasive long-term monitoring should, as a rule, be performed in all patients with FCDs prior to the corticectomy.
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PMID:[Focal cortical dysplasia and epilepsy surgery]. 907 Nov 91


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