Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 24 year old woman with a 15 year history of intractable seizures underwent resection of a small low grade astrocytoma of the right mesial frontal lobe. The tumor arose beneath a benign scalp nevus that had been treated in infancy with radium patches and focal x-irradiation. Neuropathological changes within the tumor were compatible with radiation injury. Meningiomas and sarcomas complicate cranial irradiation but the evidence that gliomas do so is less convincing. Our observations support an association between radiation exposure and the subsequent development of glial tumors.
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PMID:Astrocytoma following scalp radiotherapy in infancy. 651 32

A 32-year-old male, who had had temporal lobe seizure for the past 10 years, was admitted to the neurosurgical institute of Tenri Hospital on March 10, 1981. Physical examination on admission revealed some memory disturbance, neuroasthenic tendency and a purplish nevus in the left foot. Plain x-ray series of the skull showed several nodular calcified lesions in the medial aspect of the left temporal lobe. Electroencephalography showed sporadic negative spikes and irregular slow waves dominant in the left anterior quadrant of the head. CT scan showed a high-density area in the left hippocampal gyrus with slight enhancement by contrast medium. Under stereotactic consideration, a left temporal osteoplastic craniotomy and total removal of the mass were performed on March 20, 1981. The histological examination proved it to be calcified cavernous hemangioma or hemangioma calcificans after Penfield and Ward (1948). Postoperative course was uneventful; even 4 weeks after surgery, electroencephalogram became normalized, and memory and anxiety scores became better. At present, in spite of decreased anticonvulsant, the patient has had no more seizure since surgery, and has returned to his business without any complaint. The authors have emphasized the necessity of active operation for hemangioma calcificans even for the one located in the depth of the dominant cerebral hemisphere. Stereotactic consideration can make the surgical risk minimize. CT scan especially with its contour plot of the images is helpful precisely to locate the lesion. For the nevus in the left foot, the biopsy was refused by the patient, which might have connection with the intracerebral cavernous angioma.
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PMID:[A case of hemangioma calcificans (author's transl)]. 704 21

A 13-year-old boy had ocular malformations and a sebaceous nevus of Jadassohn. The salient features included left frontoparietal alopecia, a pigmented flat nevus anterior to the left tragus, and a left-sided fleshy corneoscleral limbal mass. Histopathologic analysis showed a complex choristoma of lacrimal tissue and cartilage, together with intrascleral cartilage and bone. Deformity of the left sphenoid bone, dilatation of the left lateral ventricle, and localized atrophy of the left cerebral hemisphere were evident, but the patient had no history of seizures, no neurologic deficit, and normal mentality. Patients with a sebaceous nevus of Jadassohn should be examined for ocular malformations and watched closely for the development of malignant nevoid tumors within the area of the nevus.
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PMID:Ocular malformation in association with ipsilateral facial nevus of Jadassohn. 729 93

A novel purine, (N6-2-(4-chlorophenyl)-bicyclo 2.2.2.-octyl-(3)-adenosine) EMD 28422 increases the binding of (3H) diazepam to benzodiazepine receptors in vivo within 10 min after intraperitoneal administration. This increase in (3H) diazepam binding is due to an increase in the number of benzodiazepine receptors (Bmax) rather than an altered affinity of the radioligand for receptor (Kd), EMD 28422 protects mice against pentylenetetrazole and caffeine-induced seizures and potentiates the anticonvulsant action of subeffective doses of diazepam in a dose-dependent fashion. Furthermore, EMD 28422 also produces a significant increase in punished responding in a conflict situation (rats), and a long-lasting, dose-dependent decrease in spontaneous motor activity (mice). In contrast, neither EMD 39011 nor adenosine (the two component molecules of EMD 28422) possess anticonvulsant properties at doses up to five mole-equivalents of EMD 28422. These data indicate that the purine EMD 28422 produces a spectrum of pharmacologic effects similar to the benzodiazepines, yet in contrast to the benzodiazepines (and other purines), increases benzodiazepine receptor number. Thus, EMD 28422 may represent the prototype of a class of synthetic purines exerting a unique neurochemical effect on benzodiazepine receptors and possessing several therapeutic actions of the benzodiazepines.
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PMID:Pharmacologic and behavioral effects of EMD 28422: a novel purine which enhances (3H) diazepam binding to brain benzodiazepine receptors. 739 62

Congenital and acquired cerebrovascular diseases in the very young age group are reviewed and discussed. Whilst saccular aneurysms are rare arteriovenous malformations and cavernous hemangiomas represent the most frequent types of all congenital anomalies. The aneurysm of the great vein of Galen manifests in the newborn period and mostly is mistaken for congenital heart disease. If the infant survives this angioma causes hydrocephalus. A particular type of phakomatosis combines intracerebral pure venous malformation with homolateral port-wine nevus on the front, often causing epileptic seizures. Spontaneous intracerebral hemorrhage occurs in children with small cavernous hemangiomas. Acquired arterial lesions may develop during fetal life by embolism, causing porencephaly or unilateral brain atrophy. The "Moyamoya" syndrome represents a frequent multi-arterial lesion causing characteristic ischemic episodes. Etiology is still obscure. We do not even know if the disease is acquired or congenital.
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PMID:Cerebrovascular diseases in the first three years of life. 743 79

The sebaceous nevus syndrome is sometimes associated with hemimegalencephaly and a group of related abnormalities including ipsilateral gyral malformation, mental retardation, seizures, especially infantile spasms, and facial hemihypertrophy. This combination has been described as the "neurological variant of epidermal nevus syndrome." Other brain malformations have been reported only rarely. We report on a child with a subtle sebaceous nevus associated with hemimegalencephaly who also had agenesis of the corpus callosum and Dandy-Walker malformation.
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PMID:Agenesis of the corpus callosum and Dandy-Walker malformation associated with hemimegalencephaly in the sebaceous nevus syndrome. 762 36

MK-801, a noncompetitive antagonist of the NMDA (N-methyl-D-aspartate) receptor, has protective effects against excitotoxicity and ethanol withdrawal seizures. We have determined membrane/buffer partition coefficients (Kp[mem]) of MK-801 and its rates of association with and dissociation from membranes. Kp[mem] (+/- SD) = 1137 (+/- 320) in DOPC membranes and 485 (+/- 99) in synaptoneurosomal (SNM) lipid membranes from rat cerebral cortex (unilamellar vesicles). In multilamellar vesicles, Kp[mem] was higher: 3374 (+/- 253) in DOPC and 6879 (+/- 947) in SNM. In cholesterol/DOPC membranes, Kp[mem] decreased as the cholesterol content increased. MK-801 associated with and dissociated from membranes rapidly. Addition of ethanol to SNM did not affect Kp[mem]. MK-801 decreased the cooperative unit size of DMPC membranes. The decrease was smaller than that caused by 1,4-dihydropyridine drugs, indicating a weaker interaction with the hydrocarbon core. Small angle x-ray diffraction, with multilayer autocorrelation difference function modeling, indicated that MK-801 in a cholesterol/DOPC membrane (mole ratio = 0.6) causes a perturbation at approximately 16.0 A from the bilayer center. In bilayers of cholesterol/DOPC = 0.15 (mole ratio) or pure DOPC, the perturbation caused by MK-801 was more complex. The physical chemical interactions of MK-801 with membranes in vitro are consistent with a fast onset and short duration of action in vivo.
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PMID:Interaction of the NMDA receptor noncompetitive antagonist MK-801 with model and native membranes. 769 77

Forty patients with Sturge-Weber syndrome were studied over a 26-year period. The nevus flammeus was unilateral in 27 patients (twice as often on left side) and bilateral in 13 patients. Only 3 of these 13 patients had bilateral cerebral lesions. Seizures, most of which were focal, were present in 32 patients (80%). The percentage of patients in whom the seizures subsequently became generalized was very high. Seizure presentation coincided with febrile episodes in 10 of 32 patients (31%). Total seizure control was obtained in 15 patients (47%). Abnormality of the cerebral parenchyma can be detected from birth in some patients and has a progressive character; at the same time, progressive atrophy and parenchymatous hyperdensity of the affected hemisphere is evident, as well as a decrease in arterial size, especially during the first decade of life. No relationship exists between the size of the facial nevus flammeus or its unilateral or bilateral location and clinical neurologic impairment. Conversely, a direct relationship exists between greater anatomic manifestations (i.e., atrophy, calcification) in the involved hemisphere when the lesion is unilateral as well as the presence of leptomeningeal angiomatosis in both cerebral hemispheres in patients with bilateral facial nevus flammeus and the severity of clinical disorders. Mental retardation was present in 60% of patients and was severe in 32.5% of all patients. Even though computed tomography and T1- and T2-weighted magnetic resonance imaging have great diagnostic value, magnetic resonance imaging enhanced with gadolinium-DTPA discloses the cerebral, leptomeningeal, and ocular lesions before the first evidence of neurologic abnormality.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Sturge-Weber syndrome: study of 40 patients. 821 40

I report 2 unusual cases of Proteus syndrome that support the concept of somatic mosaicism. In one patient, a huge connective tissue nevus covered the chest and abdomen and hyperostoses of the calvaria were observed. In the other patient, linear verrucous epidermal nevi, epibulbar dermoids, and hyperostoses were found. No enlargement of the limbs or digits occurred and the plantar surfaces of the feet were normal. Selective aspects of Proteus syndrome not previously reviewed are also presented including: uncommon neoplasms; pulmonary and renal abnormalities; brain malformations; facial phenotype associated with seizures and severe mental deficiency; and types of abnormal growth in the craniofacial skeleton.
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PMID:Proteus syndrome: clinical evidence for somatic mosaicism and selective review. 1044 40

"Epidermal nevus syndrome" ("ENS") is a neurocutaneous disorder in which epidermal nevi are associated with other abnormalities, most commonly of the skeletal and central nervous systems. We present two cases of epidermal nevus syndrome (ENS) with very different clinical findings. The first case is a newborn with multiple linear epidermal nevi of the trunk and limbs, and several other anomalies, including bony duplications of the lower limbs and hypoplastic left heart syndrome. The second patient, a 6-year-old boy, has a linear nevus sebaceous of the scalp with severe CNS involvement, including generalized seizures, moderate mental retardation, microcephaly, and a left hemiparesis. He also has genitourinary, cardiac, and skeletal defects. These two patients exhibit several abnormalities not previously recognized and illustrate the wide clinical spectrum of "epidermal nevus syndrome." We present a review of the clinical findings in 74 cases of "ENS." Correlation was noted between the presence of skin lesions located on the head and CNS involvement. The wide clinical spectrum of "ENS" as illustrated by these two patients suggests that "ENS" is a causally heterogeneous group of disorders.
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PMID:Further delineation of the epidermal nevus syndrome: two cases with new findings and literature review. 836 47


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