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)

Nine of 48 adult patients who underwent orthotopic liver transplantation developed significant clinical neurological abnormalities recognized shortly after operation. Decrease in consciousness occurred with resultant coma, focal and generalized seizures and the occasional appearance of a state of akinetic mutism. Neuropathological abnormalities consisted of multifocal areas of infarction in cerebral cortex and basal ganglia in five patients, central pontine myelinolysis in five (often more extensive than usually reported), Wernicke's encephalopathy in three, glial nodules in two, and fungal abscesses in one. Alzheimer II astrocytosis was found in all brains available for retrospective study. There was direct evidence in two of the patients that air embolization from the homografts had occurred. Correlation of this with the brain infarcts in these and other cases seems reasonable. The ease with which air passed to the systemic circulation is explicable by the right to left venous--arterial shunts that are common in chronic liver disease. With the delination of this cause for the neurologic complications, measures to prevent it in future cases have been described.
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PMID:Acute neurological complications after liver transplantation with particular reference to intraoperative cerebral air embolus. 34 84

Neuropathological considerations were performed on a case, who went into Lennox syndrome after an acute encephalopathy at the infantile period, and moreover who fell into an akinetic-mute state derived from brain damage by herniation caused by a head injury and subsequent status epilepticus. Neuropahtological background in the present case of Lennox syndrome is thought to be based on the widespread unilateral cerebral lesions and the basal ganglional, especially thalamic, degenerations derived secondarily from the diffuse cerebral damage. The patient revealed akinetic mutism with the disappearance of the epileptic seizures and the desynchronization of the EEG's, when the brain lesions formed at the adult period spread over the opposite hemispheric limbic system and the brain stem tectum.
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PMID:Neuropathology of secondary generalized epilepsy--(Lennox-Gastaut syndrome)--a case report. 65 45

A new type of non-specific X linked mental retardation is described in a three generation family. The three affected males had severe mental retardation (IQ 20 to 30), mutism, growth failure, frequent infections, seizures, and the following minor anomalies: brachycephaly, frontal hair whorl, square face, large mouth, thick lips, and prognathism. There was not a characteristic facies. Normal laboratory studies on the proband included a karyotype with fragile X screening, skeletal survey, blood amino acid, urine organic acid, and HGPRT levels. Linkage analysis was performed with 10 X chromosome DNA probes of which probe DXS255 at chromosomal region Xp11.22 gave a maximal two point lod score of 2.10 if phase was inferred and 1.20 if it was not. Crossovers were shown with probes mapping to regions Xp22, Xp21, and Xq28. Comparison of these patients with 80 X linked causes of mental retardation, including 41 which might be classified as 'non-specific', showed no other disorders compatible with the phenotypic and linkage data.
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PMID:Non-specific X linked mental retardation with aphasia exhibiting genetic linkage to chromosomal region Xp11. 847 12

An infant is described who developed operculum syndrome during an acute encephalitic illness. Presenting symptoms were cortical pseudobulbar palsy and focal seizures of facial origin. Persistent mutism--with normal language comprehension and orofacial motor disturbance--were the main neurological sequelae. Similarities between this case and other permanent or transient causes of cortical pseudobulbar palsy are discussed, as well as the possible relationship with certain types of childhood language disorders.
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PMID:Operculum syndrome in childhood: a rare cause of persistent speech disturbance. 1087 32

Five children with Landau-Kleffner syndrome (epilepsy, acquired aphasia, and continuous spike-wave discharges during sleep), were treated with antiepileptic drugs (AEDs), sleep-modifying drugs, and corticosteroids. The pharmacologic profiles differed from those observed in focal epilepsies, resembling instead those of certain generalized epilepsies, such as West or Lennox-Gastaut syndromes. Phenobarbital (PB), carbamazepine (CBZ), and phenytoin (PHT) were ineffective or worsened the EEG and neuropsychological symptoms, whereas valproate (VPA), ethosuximide (ESM), and benzodiazepines were partially or transiently efficacious. Dextroamphetamine produced a dramatic but transient improvement in waking and sleep EEG in one of two children; aphasia did not change. Corticosteroid treatment resulted in improved speech, suppression of seizures, and normalization of the EEG in three of three children. Our own experience and data from the literature suggest that corticosteroids should be given in high doses as soon as the diagnosis is firmly established and should be continued in maintenance dose for several months or years to avoid escape. Early diagnosis, before mutism or global deterioration develops, appears to be essential for effective therapy with minimal neuropsychological sequelae.
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PMID:Landau-Kleffner syndrome: a pharmacologic study of five cases. 170 Sep 53

Ifosfamide-associated central nervous system toxicity has been reported in 5% to 30% of patients treated with ifosfamide. Its pattern is characterized by metabolic encephalopathy with confusion, blurred vision, mutism, auditory or visual paranoid hallucinations, seizures, and rarely coma. The biochemical cause of the neurotoxicity is not understood completely, but it is thought to result from an accumulation of drug metabolites with direct central nervous system effects. A case of ifosfamide neurotoxicity is reported that had unusual extrapyramidal features in a patient treated with a 5-day course of infused ifosfamide. Although usually spontaneously reversible with cessation of drug administration, ifosfamide neurotoxicity occasionally has been associated with prolonged psychopathologic sequelae. Death from irreversible encephalopathy has also been reported rarely. The authors believe that classic extrapyramidal symptoms should be considered to be a part of the neurotoxic profile of ifosfamide.
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PMID:Ifosfamide extrapyramidal neurotoxicity. 142 8

The syndrome of acquired verbal auditory agnosia in childhood with mutism and epileptic discharges has been described in over 100 cases. An encephalitic etiology has often been postulated but never proved. We report two patients with this syndrome who were treated surgically. Despite careful search, no pathologic evidence of encephalitis was found. One patient, with the typical course, had no seizures but striking positive correlation between epileptic discharge and language disorder; the second, after classic onset, developed intractable temporal lobe epilepsy, a previously unreported outcome of this syndrome. EEG discharges are generalized, bilateral, multifocal, or with shifting predominance but mainly temporal in 85% of reported cases, and unilateral, also predominantly temporal, in 15%. Language areas are preferentially involved. This syndrome has certain biologic features that resemble the benign epilepsies of childhood and may be the result of the unusual localization of the epileptic abnormality.
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PMID:The Landau-Kleffner syndrome of acquired epileptic aphasia: unusual clinical outcome, surgical experience, and absence of encephalitis. 244 19

The clinical features, investigative profiles and outcome of 46 patients with biopsy or autopsy-proven herpes simplex encephalitis admitted to the Institute of Neurological Sciences, Glasgow between 1962 and 1985 were analysed retrospectively. The protean presenting symptoms and signs included a history of a prodromal influenza-like illness (48 per cent), rapid onset of headache, clouding of consciousness and confusion (52 per cent), meningism (65 per cent), raised intracranial pressure (33 per cent), deep coma (35 per cent), mutism or aphasia (46 per cent), focal neurological signs (89 per cent), and seizures (61 per cent). When seizures occurred they were almost always focal. The electroencephalogram was the most useful diagnostic test being abnormal in all cases, the majority showing focal changes in one or other hemisphere. Of the neuroradiological procedures employed, computerized tomographic and isotope brain scanning most frequently demonstrated localizing abnormalities in one or both temporal and/or frontal lobes. Midline shift was seen in half the cases. The cerebrospinal fluid was abnormal in every case but was not diagnostic. Cerebral biopsy of one temporal lobe was performed in 40 cases and a positive diagnosis of acute necrotizing encephalitis was made in 37 of these. Herpes simplex virus was isolated from the brains of 29 of the 40 cases in which the procedure was attempted, but immunofluorescence assays for antigens to herpes simplex virus were only positive in 11 out of 25 cases. Serological assays showed a greater than four-fold rise in the anti-herpes simplex virus antibody titre in 13 out of 22 patients tested.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:A retrospective analysis of forty-six cases of herpes simplex encephalitis seen in Glasgow between 1962 and 1985. 325 5

Locked-in syndrome is a rare syndrome characterized by quadriplegia, intact consciousness, mutism, and communication using vertical eye movements. We report the case history of a patient with transient locked-in syndrome following repeated shunt procedures for idiopathic aqueductal stenosis. Communication was with lateral eye movements. Although she eventually recovered and regained functional independence, high doses of phenobarbital, given to prevent seizures, sedated her and obscured her mental status during her rehabilitation. We urge the careful monitoring of all centrally acting agents in patients with locked-in syndrome.
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PMID:Transient locked-in syndrome and phenobarbital. 363 27

76 children are studied--66 from the literature and 10 from a personal series--who developed epileptic seizures, paroxysmal EEG abnormalities and language deterioration after a period of normal development of language. The clinical features of the fits, the age at which the seizures appear and disappear, the age of the EEG normalization and the normal clinical and radiological examinations were reminiscent of partial benign epilepsies of childhood. Shortly after the language deteriorates, the awake EEG exhibits slow spikes that are diffuse or predominate in the temporal areas, sometimes asymmetrically. During sleep, the paroxysmal abnormalities are clearly bilateral and continuous or subcontinuous. Spontaneously or under medications, the intensity of the paroxysmal abnormalities decreases before clinical improvement occurs. In comparison with the mutism resulting from acquired hearing loss, it is suggested that the language disturbances in these children result from a functional disorganization of the language centers due to the important intercritical EEG abnormalities. In some children, these functional disturbances could result from a benign epilepsy. This view leads to discuss the prognosis and the therapeutic possibilities.
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PMID:[Electroclinical and developmental aspects of epilepsy in the aphasia-epilepsy syndrome]. 688 17


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