A Case of Serum Amino Acid Disturbance with Hyperammonemia in Patient with Primary Amyloidosis

There have been reports that hyperammonemia and amino acid disturbance can cause loss of consciousness in patients with multiple myelomas and normal liver function. We experienced a case of a 71-years-old female patient with amyloidosis, who had shown disturbance of consciousness. At that time, the serum ammonia level was elevated; serum amino acids disturbance was also noted. In particular, the decrease in branched-chain amino acids and increase in aromatic amino acids results in a low Fisher ratio. The Fisher ratio, the ratio of branched-chain to aromatic amino acids, has been suggested as an important indicator of consciousness disturbance. We report, for the first time in Korea, a case of amyloidosis, with mental disturbance due to serum amino acid disturbance.

A Case of Serum Amino Acid Disturbance with Hyperammonemia in a Patient with Multiple Myeloma / 대한혈액학회지

Hyperviscosity and hypercalcemia are the common causes of disturbance of consciousness in patients with multiple myeloma (MM). However, there have been anecdotal reports of disturbance of consciousness in MM due to hyperammonemia and serum amino acid disturbance without possible causes such as liver failure. A 45-year-old female patient with MM was admitted due to general weakness. On 7th hospital day, she showed somnolence and disorientation. On the assumption of hyperviscosity syndrome, plasma exchange was performed immediately, but no effect. At that time, serum ammonia level was 431ng/dL, and serum glycine level was abnormally elevated. This type of serum amino acid disturbance was different from that usually found in chronic liver disease. After vincristine, adriamycin, and dexamethasone chemotherapy, serum ammonia level was restored to the normal range followed by improved mental status. We report, first in Korea, a case of MM with mental disturbance due to hyperammonemia and serum amino acid disturbance.