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

The migrational disorders are a rare group of congenital malformations of the brain. They consist of the following entities - lissencephaly (agyria - pachygyria), pachygyria, schizencephaly, heterotopia and polymicrogyria. We studied 40 children with migrational disorders radiologically with CT and MR. This article (Part II) deals with our patients with schizencephaly, heterotopia and polymicrogyria. These patients presented clinically with a variety of symptoms. The most common were seizures, delayed development, failure to thrive and hydrocephalus. CT and MR both demonstrated the characteristic findings in all of our patients except the polymicrogyria group. The gray matter and cleft abnormalities seen in these disorders were demonstrated with CT and MR. However, MR provided better delineation of these disorders than CT. Because some forms of migrational disorders can be inherited, it is extremely important for the radiologist to understand the characteristic findings for correct diagnosis which is essential for parental counseling.
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PMID:The CT and MR evaluation of migrational disorders of the brain. Part II. Schizencephaly, heterotopia and polymicrogyria. 274 27

Migration anomalies are congenital malformations caused by insults to migrating neuroblasts during the third to fifth gestational months. Included in this group are agyria, pachygyria, polymicrogyria, unilateral megalencephaly, schizencephaly, and gray matter heterotopias. Patients who have these conditions present clinically with developmental delay and seizures, and abnormal motor skills are noted in the more severely affected infants. To determine the utility of MR as a method for imaging in these patients, we used MR to evaluate 13 patients who had the full spectrum of migration anomalies. MR was more sensitive than CT in detecting these anomalies because of its better contrast between gray and white matter. We found that MR was particularly more sensitive in detecting schizencephaly, where recognizing the presence of gray matter lining the cleft is critical to distinguishing that disease from porencephaly, and in detecting polymicrogyria, where critical details of cortical architecture are obscured on CT by the overlying bone. Multiplanar capabilities were also found to be essential, since narrow clefts may not be detected when the imaging plane is parallel to the cleft. MR should be the primary imaging method for infants who have seizures or developmental delay.
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PMID:MR of neuronal migration anomalies. 325 18

MR imaging was used to evaluate six patients who had schizencephaly, a disorder of cell migration characterized by holohemispheric, gray-matter-lined clefts. Clinically, these patients presented with intractable seizures and variable developmental delay. Although three of these patients had previous CT scans, the diagnosis was made only by MR. MR was more sensitive than CT in detecting the clefts as well as the accompanying abnormalities, including areas of pachygyria, polymicrogyria, and heterotopic gray matter. The possible pathogenesis of schizencephaly is discussed. MR provides excellent demonstration of the anatomic changes in schizencephaly.
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PMID:MR imaging of schizencephaly. 325 84

In a patient with clinical manifestations suggestive of brain malformation, computer-assisted tomography (CT) showed lissencephaly: agyria, pachygyria, absent opercularization, and colpocephaly. The patient did not have seizures or a typical EEG of hypsarrhythmia. By magnetic resonance imaging (MRI), using a long inversion-recovery sequence, it was possible to verify the CT-findings and to demonstrate heterotopic grey matter and missing claustrum. By MRI it was much easier to estimate the altered ratio of grey and white matter. High grey-white matter contrast of inversion-recovery scans and the possibility of imaging the brain in sagittal, coronal and transverse planes make MRI the method of choice for the evaluation of lissencephaly and other brain malformations. In this case it helped to verify lissencephaly as one aspect of an unknown clinical entity of type-I-lissencephaly, defective structure of lymphatic nodes, a polyarthritis-like clinical picture, hypodontia, and flaring of the ribs.
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PMID:Magnetic resonance imaging in lissencephaly. 356 64

Ten children at the age of 1-16 with various types of cortical dysplasias are demonstrated by means of computerized tomography. Clinical features of focal subcortical mass of heterotopic gray matter (focal pachygyria) are contralateral partial seizures (n = 5). Pachygyria commonly is associated with severe psychomotor retardation and epilepsy (n = 3). In two cases nodular heterotopias protruding the ventricular walls are accompanied by epilepsy only.
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PMID:[Cortical abnormalities of the brain. Morphologic and clinical findings]. 378 35

Four unrelated patients who had the clinical appearance of Miller-Dieker syndrome, also called lissencephaly syndrome, were studied. All four had a typical clinical course with failure to thrive, severe psychomotor retardation, opisthotonos, seizures, and death early in life. None of these children had lissencephaly, the anticipated central feature of this disorder. One of the four had pachygyria, one had polymicrogyria, and two had both pachygyria and polymicrogyria. The brain weights were normal to decreased. The ventricles were dilated in all cases. The cerebral cortex was thickened in each, with decreased white matter and diminution or distortion of the cellular layers, and there were neuroglial heterotopias. The corpus callosum was partially absent in one and thinned in three. The neuropathy found in these children with Miller-Dieker syndrome suggests a spectrum of gyral anomalies resulting from a single type of embryonic error.
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PMID:A spectrum of gyral anomalies in Miller-Dieker (lissencephaly) syndrome. 683 90

The "band heterotopia" or "double cortex" is a brain anomaly that is presumed to result from a premature arrest of neuronal migration. Patients with this anomaly are reported to have a variable clinical course that has been, heretofore, unpredictable. The clinical records and magnetic resonance (MR) imaging studies of 27 patients with band heterotopia were retrospectively reviewed in an attempt to determine whether imaging findings are useful in predicting clinical outcome of affected patients. Statistical analyses revealed the following correlations: (1) severity of T2 prolongation in the brain with motor delay (p = 0.03); (2) degree of ventricular enlargement with the age of seizure onset (p = 0.04), and with development and intelligence (p = 0.04); (3) severity of pachygyria with the age of seizure onset (p = 0.01), seizure type (p = 0.03), and an abnormal neurologic examination (p = 0.002); (4) parietal involvement with delayed speech development (p = 0.05); (5) occipital involvement with age of seizure onset (p = 0.006); (6) age of seizure onset with development and intelligence (p = 0.03) and with an abnormal neurologic examination (p = 0.04); and (7) severity of the pachygyria and thickness of band with development of symptomatic generalized epilepsy (p = 0.002 and p = 0.02, respectively) and Lennox-Gastaut syndrome (p = 0.002 and p = 0.01, respectively).
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PMID:Band heterotopia: correlation of outcome with magnetic resonance imaging parameters. 752 38

MRI findings of bilateral central macrogyria allowed the diagnosis of a congenital variant of Foix-Chavany-Marie syndrome in four patients aged between 13 and 32 years, with facio-pharyngo-glosso-masticatory central diplegia, mental retardation and seizures.
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PMID:MRI of congenital Foix-Chavany-Marie syndrome. 756 51

Cerebral cortical dysgenesis (CD) is a heterogeneous disorder of cortical development and organization commonly associated with epilepsy, with a variety of subtypes. We reviewed the clinical, EEG and neuroimaging features in 100 adult patients with CD. There were 39 men and 61 women with a median age of 27 years (range 15-63 years). All patients were referred because of medically refractory epilepsy. Median age at seizure onset was 10 years (range 3 weeks to 39 years); in 30 patients, onset was in adulthood. The epilepsy was classified as generalized in 16 patients and localization-related in 84. Of the latter, the epileptic syndromes in decreasing frequency were frontal (32%), temporal (31%), parietal (14%) and occipital (7%). Only 15% of patients had a history of status epilepticus. Prenatal/perinatal problems were reported in 32 patients but these were severe in only four: exposure to drugs (three) and infection (one) during the first trimester. Delayed developmental milestones were seen in 10%, mental retardation in 9%, additional congenital abnormalities in 4% and neurological deficits in 14% of patients. Diagnosis of CD was based on neuroimaging in 70, pathology in four and both methods in the remaining 26. The following subcategories were identified: agyria/diffuse macrogyria (four patients), focal macrogyria (16), focal polymicrogyria (one), focal macrogyria/polymicrogyria associated with a cleft (11), minor gyral abnormalities (seven), subependymal grey matter heterotopia (20), bilateral subcortical laminar grey matter heterotopia (eight), tuberous sclerosis (five), focal cortical dysplasia/microdysgenesis (seven) and dysembryoplastic neuroepithelial tumours (DNT) (21). Sixty-eight percent of patients had normal CT and 19 out of 36 patients had normal previous conventional MRI. MRI-based hippocampal volume measurements in 47 patients revealed ratios (smaller: larger hippocampus) of < 0.90 in 16, 0.90-0.94 in 14 and > or = 0.95 in 17 patients. EEGs were normal in only five patients. Alpha rhythm was preserved in 78 patients, including one patient with bilateral posterior macrogyria. Localized polymorphic slow activity was present in 43 patients. Five of 68 patients with focal/unilateral CD had only bilateral independent/synchronous spiking and 14 out of 32 with diffuse/bilateral CD only focal/unilateral spiking. In 60 patients with nondiffuse CD or with abnormal gyration or DNT, the epileptiform abnormalities were less extensive than coextensive with the lesion in 28, more extensive than and overlapped the lesion in 18 and remote from the lesion in five; nine patients did not have epileptiform abnormalities. There was poor correlation between the epileptic syndromes and EEG abnormalities and the location/extent of CD as defined by MRI and pathology.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Abnormalities of gyration, heterotopias, tuberous sclerosis, focal cortical dysplasia, microdysgenesis, dysembryoplastic neuroepithelial tumour and dysgenesis of the archicortex in epilepsy. Clinical, EEG and neuroimaging features in 100 adult patients. 760 83

Hemimegalencephaly is a rare congenital anomaly of the brain characterized by unilateral hypertrophy, usually with an abnormal gyri pattern and derangement of the cortical architecture. We report 2 patients with hemimegalencephaly who presented with early-onset seizures, hemiparesis and developmental delay. An electroencephalogram showed hemispheric continuous spikes and spike-and-waves in one patient and repetitive spike-and-waves in the other. Magnetic resonance imaging showed left hemimegalencephaly in both cases and in case 1 pachygyria and heterotopia. A functional hemispherectomy was done on case 1 at the age of 33 months and on case 2 at 7 months due to difficulty in seizure control with antiepileptic drugs. The frequency of seizure dramatically decreased and there was an obvious improvement in neurologic development after surgical intervention. Pathology revealed disorganized lamination of the cortical layers with increased neuron size and bizarre-shaped neurons in both cases. Heterotopia of neurons and glia in the subarachnoid space was noted in case 1. Hemispherectomy should be performed as soon as possible when medical treatment fails to control seizures.
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PMID:Hemimegalencephaly treated by hemispherectomy: report of two cases. 763 3


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