Crow-深瀬症候群の末梢神経病理

Pathology of Crow-Fukase Syndrome

はじめに

　末梢神経系はCrow-Fukase症候群（CFS）の病変の主座の1つであり，病理学的所見についても，本症候群が一疾患単位として認知された頃から精力的に解析が進められてきた。1970～1980年代にかけては本邦からの報告が他を圧しているが，近年は欧米からも重要な知見が多数集積されてきている。本稿ではCFSの病理所見について今まで報告されてきた生検所見，剖検所見を概括し，近年明らかになった本症でのVEGF高値と病理所見（特に血管病変）との関連について述べることとする。

Abstract

　Although the exact mechanisms underlying peripheral neuropathy in Crow-Fukase syndrome (CFS), also known as POEMS syndrome, remain obscure, careful scrutiny of the pathological changes in the peripheral nervous system (PNS) and systemic organs by using biopsy and autopsy materials may provide useful information regarding the pathogenesis and future therapeutics of the syndrome.　In this review, previous biopsy/autopsy studies on CFS were systematically reviewed and the details of the pathological changes in the PNS and vascular system were noted.　Most biopsied nerves revealed the characteristics of axonal degeneration and demyelination together; however, the nerve roots obtained at autopsy showed massive demyelination with few axonal changes.　This morphological discrepancy can be interpreted as primary demyelination in the proximal PNS and secondary axonal degeneration in the distal PNS.　Another histological hallmark of the syndrome is edema in the endoneurial space.　Changes in the endoneurial and epineurial microvessels, including hyperplasia of endothelial cells, were occasionally observed.　Endoneurial edema and microvascular changes can be attributed to the high serum concentration of vascular endothelial growth factor in this disorder, and the derangement in endoneurial homeostasis due to impaired blood-nerve barrier can be considered a possible pathomechanism underlying peripheral neuropathy in the CFS.