Lewy Bodyを伴ったHallervorden—Spatz症候群の1剖検例

An Autopsy Case of Hallervorden, Spatz Syndrome with Lewy's Inclusion Body

I．緒言

　進行性の筋強剛と精神荒廃のみられる疾患群の中で，剖検上淡蒼球および黒質の鉄陽性色素顆粒の増加と全中枢神経系に広汎に分布するspheroidなどにより特徴づけられるHallervorden-Spatz症候群は，1922年HallervordenおよびSpatz^1）によつて最初の症例報告がなされたが，その後かなり稀な疾患と考えられながらも，漸次その報告例を増しつつある。この病変の主体が淡蒼球および黒質にあるにしても，症例によつては大脳皮質をはじめとし基底核，小脳および脊髄といつた広汎な領域に病変がおよぶものもあつて，診断の確定は生前にはしばしば困難であり，剖検をまたねばならぬことが多い。

　著者らの症例は19歳で発病し26歳で死亡した家婦で，その6年数カ月の間に，錐体外路症状，知能障害，精神症状，小脳症状，一過性の視力障害ならびに錐体路症状といつた多彩な症状を呈し，臨床的には全中枢神経系の変性疾患と診断するほかないようなものであつた。剖検によつて，この症例はこれまで報告されてきたHallervorden-Spatz症候群^2〜4）の病理組織学的特徴をすべてかねそなえているばかりでなく，無質の神経細胞に多数のLewy氏細胞質内封入体^5）がみられるという珍しい所見を伴つているので，ここに報告する。

　An interesting case of Hallervorden-Spatz syndrome with Lewy's bodies was clinically and histopathologically studied. A 19-year-old female, whose family history was noncontributory, fell ill with initial symptom of motor weakness of lower limbs. Gradually she presented varigated disorders, such as extrapyramidal, psychic, mental, cerebellar and pyramidal symptoms, transitory visual disturbance, and urinary incontinence. She expired in 6 years and several months, but the diagnosis was not established intra vitam.

　Important anatomic and histologic findings were as following: 1) Marked rust-brown discoloration of globus pallidus, oral striatum, and reticular zone of substantia nigra, where abundant siderophilic pigmentary granules were present histologically. 2) Marked symmetric atrophy (neuronal loss and glial fibrosis) of oral portions of striati. 3) Abundant presence of spheroid structures (axonal, dendritic or focal neuronal swellings) in almost whole gray matter of central nervous system (see photo 13). 4) Presence of Lewy's intracytoplasmic inclusion bodies in a large number of nerve cells of substantia nigra- a rarely reported finding in cases of Hallervorden-Spatz syndrome.5) Diffuse atrophy of cerebellar cortex. Loss or degeneration of Purkinje cells, associated with torpedoes, stellate bodies, and cactus-like expansions- a finding, which suggests origin of spheroids. Moderate depopulation of granule cells. 6) Moderate atrophy of cerebral cortex, especially marked of frontal and parietal lobes where some nerve cells showed swelling and chromatolysis. 7) Slight neurogenic atrophy of striated muscles. 8) Moderate hypoplasia of visceral organs (heart, aorta, lungs, liver and kidneys).