The clinical status and daily-life activity of 136 patients with amyotrophic lateral sclerosis (ALS) living in Nagano prefecture were evaluated. The mean age of the patients was 67.4 ± 10.4 years, and the mean age at onset was 61.0 ± 12.3 years. Thirty-nine patients (29.1%) developed the disease after 70 years of age. The mean duration of disease was 6.5 years (maximum 41 years), and 26 patients showed a duration of disease of over 10 years. Sixteen of these patients did not need artificial nutritional or respiratory support. Sixty-seven patients (49.3%) needed total support for dressing and 54 (39.7%) for walking/moving. Forty-nine (36.3%) were unable to speak, and 49 (36.0%) needed tube feeding. Sixty-six (48.5%) had respiratory difficulties, and 34 needed mechanical ventilation. Only 27 patients (19.9%) were almost independent. Out of 133 patients, 99 (74.4%) lived in their own home, with the remainder being hospitalized or institutionalized. Of the 34 patients requiring mechanical ventilation, 19 (55.9%) lived in their own home, 12 were hospitalized, and 3 were institutionalized.
A tendency of late-onset for ALS patients, which has been recently reported worldwide, was confirmed in Nagano. There were a significant number of ALS patients who had not developed bulbar palsy or respiratory failure regardless of duration of disease. As expected, artificial respiratory support was a major factor in the confinement of ALS patients to hospitals or nursing institutions.

View full abstract

We report a case of an adrenal pheochromocytoma that ruptured spontaneously and caused multiple organ failure (MOF). Spontaneous rupture of pheochromocytoma is extremely rare, but it can be lethal because of dramatic changes in the circulation.
A 44-year-old woman was hospitalized because of the sudden occurence of left loin and back pain with hypovolemic shock due to hypotension. Abdominal CT scan showed a hemorrhaging left adrenal mass surrounded by a massive hematoma. MOF developed and we decided to proceed conservatively because an emergency operation could be dangerous. The patient recovered from MOF and 122 days after the rupture, surgery was undertaken. The pathological diagnosis was adrenal pheochromocytoma.

View full abstract

[in Japanese]

View full abstract