Rinsho Shinkeigaku (Clinical Neurology)

Case Report

An autopsy case of amyotrophic lateral sclerosis with ampulla cardiomyopathy

Yumi Matsuyama M.D.1)6), Naokazu Sasagasako M.D.1), Akihiro Koike M.D.2), Masashiro Matsuura M.D.3), Takaomi Koga M.D.3), Masakazu Kawajiri M.D.4), Yasumasa Ohyagi M.D.4), Toru Iwaki M.D.4) and Jun-ichi Kira M.D.4)

1)Department of Neurology, Fukuoka Higashi Medical Center
2)Department of Cardiology, Fukuoka Higashi Medical Center
3)Department of Pathology, Graduate School of Medical Sciences, Kyushu University
4)Department of Neurology, Graduate School of Medical Sciences, Kyushu University
5)Department of Neuropathology, Graduate School of Medical Sciences, Kyushu University
6)Department of Neurology, Hamanomachi hospital

We herein report an autopsy case of a 63-year-old man with amyotrophic lateral sclerosis (ALS) who developed "ampulla cardiomyopathy." At the age of 56, he noticed a progressive weakness in his right upper extremity. One year later, a progressive weakness of the left upper extremity began. Dropped head and progressive weakness of the lower extremities emerged at the ages of 61 and 62, respectively. Intravenous immunoglobulin and plasma-exchange therapies did not improve his weakness. At the age of 63, one month before his death, he was hospitalized due to aspiration pneumonia. A tracheostomy was performed to secure his airway. Four days after the operation, an ST elevation of his electrocardiogram was incidentally found on the ECG monitor. An echocardiogram revealed diffuse hypokinesia of the wall of the left ventricle except in the basal portion, which is the typical finding of "ampulla cardiomyopathy." Wall motion of the left ventricle improved and his circulatory condition was stabilized after treatment, but his condition thereafter worsened again and he died 3 weeks later. An autopsy examination revealed diffuse fibrosis and degeneration of the cardiomyofibers. Neuropathological examination revealed neuronal cell loss, Bunina bodies and skein-like inclusions in the hippoglossal nuclei. In the thoracic spinal cord, degenarated anterior horn cells were seen and macrophage permeation in the corticospinal tract were shown by CD68 immunostaining. Therefore, the final neuropathological diagnosis was ALS. This report is the first autopsy case of ALS complicated with "ampulla cardiomyopathy."
Full Text of this Article in Japanese PDF (1113K)

(CLINICA NEUROL, 48: 249－254, 2008)
key words: amyotrophic lateral sclerosis, ampulla cardiomyopathy, autopsy

(Received: 31-Oct-06)