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Brain and Nerve No to Shinkei Volume 36, Issue 2 （February 1984）

Brain and Nerve 脳と神経 36巻2号（1984年2月発行）

Japanese English

原著

Chorea-acanthocytosisの1剖検例—特に基底核の組織病理学的および生化学的所見について AN AUTOPSY CASE OF CHOREA-ACANTHOCYTOSIS:SPECIAL REFERENCE TO THE HISTOPATHOLOGICAL AND BIOCHEMICAL FINDINGS OF BASAL GANGLIA 佐藤雄二 ¹ , 大西晃生 ¹ , 立石潤 ¹ , 鬼塚雄三 ³ , 石本進士 ² , 岩下宏 ² , 黒岩義五郎 ² , 金澤一郎 ⁴ Yuji Sato ¹ , Akio Ohnishi ¹ , Jun Tateishi ¹ , Yuzo Onizuka ³ , Shinji Ishimoto ² , Hiroshi Iwashita ² , Yoshigoro Kuroiwa ² , Ichiro Kanazawa ⁴ ¹九州大学医学部脳研神経病理 ²九州大学医学部脳研紳経内科 ³田主丸中央病院 ⁴筑波大学臨床医学系神経内科 ¹Departments of Neuropathology, Neurological Institute, Faculty of Medicine, Kyushu University ²Departments of Neurology, Neurological Institute, Faculty of Medicine, Kyushu University ³Tanushimaru Chuoh Hospital ⁴Department of Neurology, Institute of Clinical Medicine, University of Tsukuba pp.105-111

発行日 1984年2月1日 Published Date 1984/2/1

DOI https://doi.org/10.11477/mf.1406205259

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Abstract 文献概要
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　抄録　Chorea-acanthocytosis例の剖検報告はこれまでに2例しかなく，系統的な病理学的，生化学的検索が乏しく不十分である。われわれは10年の臨床経過を示し39歳で死亡した本症の1剖検例を経験したので，特に基底核の病理学的および生化学的所見と，末梢神経・筋の病理学的所見を中心に詳細な検討を加えた。尾状核は高度の，被殻は軽度の萎縮を示し，組織定量的検索により尾状核，被殻ともに小型神経細胞の高度の減少（特に尾状核で顕著）と大型神経細胞の軽度の減少，尾状核大型神経細胞の小径化が認められた。生化学的にcholine acetyltransferaseと　glutamic acid decarboxylase　（GAD）活性値の低下は明らかでなかったが，substance P （SP）値の低下が認められた。また組織学的に明らかな変化が認められない黒質でGAD活性とSP値の低下がみられた。末梢神経，筋にはそれぞれ軸索変性と骨格筋の神経原性萎縮が認められた。Chorea-acanthocytosisでは多系統が遺伝的に規定された変性を示すと推測された。

Chorea-acanthocytosis has been separated as a clinical entity different from Huntington's chorea, mainly based on the clinical findings, but the his-topathological and biochemical features of chorea-acanthocytosis, especially of basal ganglia, have not been well established, because only two such autopsy cases have been reported. The case pre-sented here was a 39-year-old man at autopsy, with 10 years duration of typical symptoms and signs of chorea-acanthocytosis.

At autopsy, the abnormal histopathological find-ings in the central nervous system were mainly confined to the striatum, where the caudate nuc-leus and putamen showed severe and moderate atrophy, respectively.

Morphometric evaluation of the numbers of small and large neurons in the striatum with the adjustment for the shrinkage produced in the dis-ease processes was performed. The numbers of small neurons in the caudate nucleus and putamen were 1% and 20% of each control, respectively. On the other hand, the large neurons in the cau-date nucleus showed a reduction of diameters with-out a decrease in number and those in the puts-men showed a mild decrease in number.

In the biochemical studies, marked decrease of substance P (SP) level without definite decrease of choline acetyltransferase and glutamic acid de-carboxylase (GAD) activities in both caudate nuc-leus and putamen was found. Substantia nigra, where no evident histopathological abnormalities were found, showed definite decrease of GAD activity and SP level.

In the peripheral nervous system, the lateral branch of deep peroneal nerve showed mild deg-ree of axonal degeneration. Neurogenic muscular atrophy with severe and mild degrees was found in extensor digitorum brevis and quadriceps fe-moris muscles, respectively.

In chorea-acanthocytosis degeneration of the striatum and the distal peripheral nerve with neu-rogenic muscular atrophy seems to be important to understand the clinical symptoms and signs.