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

Two patients with Sotos syndrome showed very intractable and prolonged status epilepticus, resulting in poor outcomes. Clinical seizures and EEG abnormalities in patients with Sotos syndrome are sometimes noted, but they are usually mild. These two patients showed hypoplasia of corpus callosum on MRI. We considered the mechanism of intractable seizures, and emphasized the importance of careful management for their seizures and EEG abnormalities.
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PMID:[Status epilepticus in two patients with Sotos syndrome]. 787 48

We reported here a 5-year-old girl with Sotos syndrome who developed acute shock encephalopathy syndrome (ASES), and differentiated ASES from Reye syndrome (RS). Abrupt onset of shock and status epilepticus developed and these were followed by disseminated intervascular coagulation (DIC) and liver damage. Gradual elevation of hepatic enzymes, high serum bilirubin value, and normal serum ammonia value in acute phase were incompatible with typical RS. Liver histology showed severe, diffuse necrosis of hepatocytes consisting of granular and vacuolar degeneration, which were quite different from those of RS. Thus, the disease process of ASES was shown to be different from those of RS.
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PMID:[Acute shock encephalopathy syndrome in a girl with Sotos syndrome--with special reference to differentiation from Reye syndrome]. 1042 86