Lewy小体病の自律神経障害と病理

抄録

The histological hallmark of Lewy body disease (LBD), which encompasses Parkinson's disease, Parkinson's disease with dementia, dementia with Lewy bodies, and pure autonomic failure with Lewy bodies, is neuronal α–synuclein aggregates called Lewy bodies and Lewy neurites. Neuronal α–synuclein aggregates are distributed throughout the nervous system, including not only the central nervous system (CNS) including substantia nigra, locus ceruleus, dorsal motor nucleus of the vagus, intermediolateral nucleus of thoracic cord and hypothalamus, but also sympathetic ganglia, enteric nervous system, cardiac and pelvic plexuses, submandibular gland, adrenal medulla and skin. Involvement of central and peripheral autonomic nervous system is characteristic feature of LBD. Non–motor symptoms of LBD closely related to the pathological α–synuclein aggregates in central and peripheral autonomic nervous system. The long duration of pure autonomic failure with Lewy bodies may suggest that development of α–synuclein aggregates from peripheral autonomic nervous system to CNS is not always the same as those among LBD.