Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038220 (status epilepticus)
 7,272 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A new technique is described for the autoradiographic determination of regional brain glucose metabolism employing 14C labeled glucose as substrate and measurement principles previously described for whole brain. Regional glucose values correlate closely with those reported for the 14C-deoxyglucose technique. The method has the advantages of 1) a much shorter experimental period, 2) a relatively simple mathematical treatment, and 3) the utilization of the actual, fully metabolizable substance itself, glucose, as the label. In addition to normal rats, regional values are reported for 20 individual brain areas of rats in bicuculline induced status epilepticus, rats intoxicated with ammonium and rats anesthetized with pentobarbital sodium or ketamine.
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PMID:Measurement of regional brain glucose utilization in vivo using [2(-14)C] glucose. 52 10

There have been anecdotes of unexplained coma and death in patients after otherwise successful orthotopic lung transplantation. A patient with primary pulmonary hypertension who underwent a technically uncomplicated single orthotopic lung transplantation is described. The patient developed intractable status epilepticus 4 days after surgery in association with the presence of a markedly elevated plasma ammonium level. Despite multiple therapeutic interventions, the hyperammonemia ultimately resulted in the patient's death. Both metabolic and enzymatic studies showed that the unique physiological disturbance in this disorder results at least in part from defective in vivo conversion of waste nitrogen to urea and increased production of waste nitrogen. Although the rate of hepatic ureagenesis was therefore insufficient to prevent accumulation of ammonium, the cause was not severe liver disease. Liver histology showed widespread, microvesicular steatosis on light-microscopic examination, but only electron-microscopic examination showed severe microvesicular steatosis with severe mitochondrial injury. As in Reye's syndrome, it was unclear whether the hepatic mitochondrial injury played a role in development or if it was the result of hyperammonemia. We recommended that any patient with an unexplained alteration of mental status after solid organ transplantation be evaluated for hyperammonemia.
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PMID:Fatal hyperammonemia following orthotopic lung transplantation. 917 20

A 67-year-old man presented with cognitive deficits, status epilepticus, left hemiparesis, and severe lactic acidosis. Respiratory chain enzyme analysis of skeletal muscle revealed a defect in complex I activity, associated with a heteroplasmic C11777A mutation in the mitochondrial ND4 gene. This case is remarkable not only because of the late onset of symptoms, but because this mutation affects the identical ND4 codon as the G11778A mutation that causes Leber hereditary optic neuropathy.
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PMID:Late-onset encephalopathy associated with a C11777A mutation of mitochondrial DNA. 1270 44