Nobuyuki Anzai

Cardiac Disorders and Autonomic Nervous System Involvement in Familial Amyloidosis

To clarify the pathogenesis of cardiac involvement in familial amyloidosis, the authors studied histological changes of the autonomic nervous system and conduction system of the heart in seven cases with this disease. Postmortem examination revealed that the autonomic innervation of the heart was impaired in both sympathetic and parasympathetic nervous system. The specialized conduction system was involved most severely and regularly in the sinus node, and to a lesser extent, in the atrioventricular node, His bundle and its periphery. Dual damage to the sinus node through autonomic denervation and degeneration of the specialized conduction system accounts for arrhythmias and conduction disturbances frequently associated with this disease.

Pacemaker Treatment in Familial Amyloid Polyneuropathy

The heart is also infiltrated by amyloid in Familial Amyloid Polyneuropathy (FAP). Conduction disturbances and abnormalities in impulse formation are major importance because of requiring pacemaker treatment. FAP is found in limitted localised asreas in Japan and also in the world. Most of the report are from Portugal, Sweden and Japan. Therefore, only limitted number of cases with pacemaker treatment in this disease have been reported.

Pathological Sutdies on Familial Amyloidosis

Although familial amyloidosis of Andrade type is mainly manifested by polyneuropathy, it is complicated with involvement of multiple visceral organs including the heart, kidney, intestine and so on (Andrade, 1952, 1965). We have reported some clinicopathological aspects of the disease. In this paper, through the analysis of clinical and pathological findings of autopsy cases, the factors leading to the cause of death are clarified.