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PubMed1989

[Coma, hyperviscosity syndrome, hyperammonemia and myelofibrosis in a patient with IgG, lambda type multiple myeloma].

A 63-year-old man was admitted to our hospital with tremor and somnolence, followed soon by coma. Anemia and retinal bleeding were observed. The blood smear exhibited rouleaux formation and leukoerythroblastosis. A bone marrow aspiration resulted in dry tap. The biopsy specimens revealed remarkable infiltration of myeloma cells with fibrosis. The M-component of IgG-lambda type and hyper-ammonemia were detected in the serum. Liver and renal functions, however, were within normal range. His consciousness recovered after plasmapheresis. Two courses of VMCP (vincristine, melphalan, carboquone and prednisolone) did not affect the paraproteinemia. Five courses of VAD (vincristine, adriamycin and dexamethasone) could lower the level of IgG. He died of pneumonia. The plasma of some patients with multiple myeloma may contain unidentified factors which increase the plasma ammonia.

Ammonia↗
PubMed1988

[Cerebral edema with hyperammonemia in valpromide poisoning. Manifestation in an adult, of a partial deficit in type I carbamylphosphate synthetase].

A case of cerebral oedema developed during an apparently common attempted suicide with valpromide is reported. The most conspicuous biochemical abnormality was hyperammonaemia. The oedema proved refractory to the standard medical treatment of intracranial hypertension, and decompressive craniectomy was performed with only minor sequelae. The cerebral oedema cum hyperammonaemia syndrome led to the discovery, in this hitherto asymptomatic adult subject, of a 50 per cent deficiency in type a carbamyl phosphate synthetase liver activity. By completing such a deficiency, valproate may produce an extremely serious syndrome resembling the neonatal encephalopathy due to complete enzyme deficiencies in the urea cycle. All valpromide or valproate intoxications probably are cerebral oedemas with hyperammonaemia akin ti Reye's syndrome. All accidents of this type occurring during treatment or poisoning with valproate should be investigated for urea cycle enzyme abnormalities.

Adult↗