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)

It is well known that intraventricular tumors are occasionally seen in patients with tuberous sclerosis. We have experienced two cases of tuberous sclerosis with intraventricular tumor. Case 1: an 8-year-old girl was admitted to our clinic because of headache and vomiting of one month's duration. She had adenoma sebaceum, mental retardation and seizures clinically, and a large tumor was found in the right lateral ventricle by pneumoventriculography. Partial removal of the tumor was performed by the right frontal transcortical approach, but she later died of pneumonia. Necropsy revealed hamartomatous disease characterized by multiple focal tumor-like malformations in various organs including the brain, kidneys, heart, lungs and liver. The histological diagnosis of the intraventricular tumor was subependymal giant cell astrocytoma. Case 2: a 6-year-old boy was admitted to our clinic because of headache of one year's duration. He had also suffered from seizures since 6 months of age. Adenoma sebaceum was noted in the cheeks. CT revealed a medium sized tumor at the right foramen of Monro and the moderately dilated right lateral ventricle and several calcified deposits in the lateral ventricle wall. The tumor was subtotally removed by the right frontal transcortical approach. The pathological examination showed subependymal giant cell astrocytoma. Now 6 years after the operation he is enjoying a normal school life. In our cases, intraventricular tumors associated with tuberous sclerosis were of a typically benign histological appearance, that is subependymal giant cell astrocytoma.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Tuberous sclerosis with intraventricular tumor: report of 2 cases]. 380 99

Primary varicella-zoster virus infection (chickenpox) in immunocompromised children is frequently associated with visceral dissemination and attendant high mortality. Eight children with malignant neoplasms and chickenpox with visceral involvement (seven with hepatitis, three with pneumonitis, two with encephalitis, and two with coagulopathy) were initially treated with intravenously (IV) administered vidarabine but demonstrated progressive visceral involvement. After three days of vidarabine treatment (two days for two patients), seven had rising serum SGPT levels, all eight had pneumonitis, seven had deteriorating mental status and/or seizure activity, and six had worsening coagulopathy. Vidarabine was replaced by IV administered acyclovir, with subsequent improvement in all but the most severely ill patient who died. Seven of eight patients recovered completely; no side effects of acyclovir were observed. This clinical experience suggests that acyclovir may be more effective than vidarabine in disseminated varicella infection; however, controlled clinical trials will be necessary to establish this.
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PMID:Acyclovir treatment of disseminated varicella in childhood malignant neoplasms. 385 82

Twenty-five adults who harbored malignant gliomas received 72 courses of intraarterial 1,3-bis(2-chloroethyl)-1-nitrosourea (BCNU) (100 mg/m2) and 67 courses of systemic vincristine (1.0 mg/m2) and procarbazine (100 mg/m2) as induction therapy (BVP) followed by 106 courses of systemic 1-(2-chloroethyl)-3-(4-methylcyclohexyl)-1-nitrosourea (methyl-CCNU) (130 mg/m2), vincristine, and procarbazine as maintenance therapy (MVP). With a 6-week interval between each treatment, the median and range for the number of courses of BVP were 3 and 1 to 4 and those for MVP were 3 and 0 to 14, respectively. Fifteen patients (60%) responded to both BVP and MVP, and 10 (40%) did not. The overall median survival time was 12.7 months (range, 1.8 to 48.5+ months). Two of 3 patients who had recurrent gliomas responded and survived for 37+ to 45+ months. Seven of 10 who had nonirradiated glioblastomas responded and survived for 9 to 22 months. Four who had nonirradiated anaplastic astrocytomas all responded and survived for 38+ to 48.5+ months. Two who also received radiotherapy (1 glioblastoma and 1 primitive neuroectodermal tumor) benefited and survived for 16.9 and 28.5+ months. All who did not respond favorably died within 8 months. During the infusion of BCNU, complications included transient orbital and head pain, periorbital and scleral erythema in all patients, and a focal seizure in 1 (4%). During the 6-month induction periods, leukopenia and thrombocytopenia occurred in 1 (4%), deep vein thrombosis occurred in 9 (36%), pulmonary emboli occurred in 8 (32%), upper respiratory infections occurred in 6 (24%), pneumonia occurred in 9 (36%), and herpes zoster occurred in 1 (4%).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Intraarterial 1,3-bis(2-chloroethyl)-1-nitrosourea (BCNU) and systemic chemotherapy for malignant gliomas: a follow-up study. 631 73

Foreign body in the tracheobronchial tree is a relatively infrequent, but, potentially fatal event, requiring rapid and expert intervention. The symptoms and signs may be mistaken for asthma and pneumonia, - or, as in the case described, with grand mal epileptiform seizures. This emphasises the need to take a thorough case history, and to have a high index of suspicion in a case presenting with convulsion associated with cough, wheezing or respiratory distress.
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PMID:Pulmonary aspiration presenting with generalised convulsions. 641 86

A boy of Finnish descent developed nerve deafness at six years of age, action myoclonus two years later, generalized myoclonic seizures when 16 years old and muscular atrophy at the age of 17 years. Bulbar palsy caused his death from inhalational pneumonia when he was 19 years old. Autopsy disclosed no significant changes in the cerebral cortex, thalamus, striatum, Purkinje cells or dentate nucleus. The most striking histological finding was degeneration of motor neurones in cranial nerves and anterior horns of the spinal cord, with neuroaxonal dystrophy of nucleus gracilis and cuneatus. While nerve deafness and spinal muscular atrophy have been recorded (each in different families) in association with progressive myoclonic epilepsy, the combination of these features has not previously been reported. Reasons are put forward for regarding all the system degenerations found in PME, including Unverricht-Lundborg disease (Baltic myoclonus) and the Ramsay Hunt syndrome, as variations of the same disorder.
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PMID:Progressive myoclonic epilepsy, nerve deafness and spinal muscular atrophy. 643 45

We describe a family in which two males and seven females have brown pigmentation of the skin. In the females, the type and distribution of the pigmentation mimicked incontinentia pigmenti; in the males, the pattern was reticulate. The histological appearance was the same in both sexes with amyloid deposits in the papillary dermis, melanin in the basal layer, and slight hyperkeratosis. The females were otherwise normal. Both males had thrived poorly as infants but had survived. One had severe gastroenteritis with blood in the stools starting at the age of three weeks followed by seizures, hemiplegia, and developmental delay; the other had recurrent pneumonia throughout life, a urethral stricture, inguinal herniae, and near-blindness from amyloid deposition in the cornea. Five other males in the family had had severe illnesses. Two died of pneumonia by three months. One died at three months from colitis. Both remaining boys had colitis as infants, failed to thrive, and developed recurrent pneumonia from which one died at three years. We think all of these relatives had the same disease carried by a single gene with pleiotropic effects. The most likely form of inheritance is X-linked.
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PMID:Familial cutaneous amyloidosis with systemic manifestations in males. 679 69

An 8-month-old girl with acute mercury vapor intoxication experienced pneumonitis with respiratory failure, bilateral pneumothorax, pneumomediastinum, acute renal failure, hepatocellular dysfunction, and seizures. Treatment centered on intensive supportive care; her survival is exceptional among infants with severe mercury intoxication.
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PMID:Survival after acute mercury vapor poisoning. 686 32

The clinical courses of 63 survivors of cardiopulmonary arrest were reviewed to determine the incidence and temporal occurrence of noncardiac complications and their relationships to mortality. Complications were grouped as occurring within 48 hours or less, within 48 to 96 hours, or more than 96 hours after cardiopulmonary arrest. Pneumonia, electrolyte level disturbances, and gastrointestinal tract hemorrhage each occurred in more than 28 (45%) of the 63 patients. Resuscitation-related injuries, seizures, and liver function test abnormalities each occurred in at least 18 (28%) of the 63 patients. Pneumonia and liver function test abnormalities were each significantly correlated with increased mortality. Septicemia, acute renal failure, and adult respiratory distress syndrome each occurred in three (5%) to four (7%) of the 63 patients and were always associated with mortality.
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PMID:Medical complications of cardiopulmonary arrest. 697 15

A patient with chronic bronchitis and acute respiratory failure due to mycoplasma pneumonia received excess parenteral fluid and developed hyponatremia associated with generalized seizures. The low serum osmolarity and elevated urine osmolarity were consistent with SIADH. Treatment with erythromycin and water restriction resulted in the complete recovery of her acute respiratory condition and return of the serum sodium and osmolarity values to normal.
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PMID:Inappropriate secretion of antidiuretic hormone and mycoplasma pneumonia infection. 703 12

At a follow-up study of 385 patients with epilepsy beginning under age 15, 22 (5.7%) had died during the first 10 years after the onset of epilepsy and another 11 (2.9%) between 11 and 24 years. Mortality was significantly high in cases with the following clinical features: (1) epilepsy with onset before the first birthday (mortality being 25.5%), (2) symptomatic epilepsy in etiology (17.2%), (3) infantile spasms (40.7%), tonic epilepsy (33.3%) or myoclonic epilepsy (33.3%) as compared with grand mal (5.9%) in seizure type and (4) developmental retardation at the first visit (25.5%). Seizures were not controlled in 31 out of 33 patients at the time of death. The causes of death were status epilepticus or convulsion in 10, pneumonia in 5, severe emaciation in 3, "cerebral palsy" in 5, and drowning, suffocation, traffic accidents or acute lymphocytic leukemia, in one each, and unknown in 6. Most of the patients died at home.
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PMID:Mortality and clinical features in cases of death among epileptic children. 713 11


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