デスフェリオキサミンが神経症状の改善に有効であった透析脳症の１剖検例

We describe a patient with advanced dialysis encephalopathy whose neurologic symptoms responded to chelation therapy with desferrioxamine (DFO).<br>The patient, a 35-year-old man with chronic glomerulonephritis, had been on hemodialysis 3 days a week since January, 1980. His hyperphosphatemia required treatment with oral aluminium hydroxide, 3 to 6 gram per day, for about 6 years. Stuttering was first noted in July, 1986. His condition subsequently deteriorated rapidly with the development of seizures, myoclonus and mental disorders. His electroencephalogram (EEG) revealed generalized paroxymal slowing with frequent spike and slow-wave discharges, and his serum aluminium (AI) level was elevated (26.8μg/dl). Cerebrospinal fluid analysis and brain CT scan, however, revealed no abnormalities. We, therefore, diagnosed this case as AI-associated dialysis encephalopathy. The exogenous source of AI in our patient was most likely ingestion of aluminium hydroxide, since the AI content of the dialysis water was less than 1.0, μg/dl.<br>The patient was given 2 grams of DFO during hemodialysis twice a week beginning in December, 1986, after aluminium hydroxide was discontinued. After two months of DFO therapy, his neurologic symptoms gradually improved, despite unchanged serum AI levels. The EEG performed after four months of therapy also revealed marked improvement with disappearance of the slow wave abnormality. There was little day-to-day change over the three months prior to death. The patient died as a result of brain hemorrhage in September, 1987 after the development of pneumonia. At autopsy there were no marked changes in the brain, however, AI deposits were observed in the macrophages found in the liver, spleen and lymph nodes.<br>This case demonstrates the usefulness of DFO in improving the neurologic status of dialysis encephalopathy patients, however, the prognosis of this disease seemed to be uninfluenced by chelation of aluminium alone.