We report a patient with multiple myeloma who suffered from hyperammonemia
after a second stem cell autograft, This syndrome is not well known but is
associated with a high mortality rate. Considering the possibility of this
diagnosis in patients developing confusion and neurological degradation wit
h respiratory alkalosis after intensive chemotherapy, could allow earlier t
reatment and perhaps improved survival. Possible mechanisms and potential t
herapies are discussed. With rapid recognition and treatment of the syndrom
e, the patient fully recovered. One and a half years later, she is still al
ive and well, on interferon for persisting myeloma.