[Treatment for paroxysmal sympathetic hyperactivity in amyotrophic lateral sclerosis patient]

We report a case of an 80-year-old man who contracted amyotrophic lateral sclerosis (ALS) 15 years ago, was put on a ventilator 8 years ago, and became locked in 3 years ago. Two years ago, he began to suffer from sudden symptoms of paroxysmal sympathetic hyperactivity (PSH) attacks (hot flushes, abnormal sweating, tachycardia, and increased blood pressure). One day, he developed multiple-organ failure. This failure healed in a few days, but PSH attacks remained. His catecholamine levels were abnormal: adrenaline, 215 pg/ml; noradrenaline, 5,960 pg/ml; and dopamine, 606 pg/ml. Diazepam was administered, which decreased both the number of PSH attacks and the catecholamine levels. When the dose was increased to 3 mg, the attacks stopped, whereas when the dose was reduced to 2 mg, the attacks relapsed. When the dose of 3 mg was continued, there was no relapse of the attacks and no re-rise in the catecholamine levels. These results show that diazepam alone has an effect on PSH attacks in ALS.