Hirayama K, Tomonaga M, Kitano K, Yamada T, Kojima S, Arai K
J Neurol Neurosurg Psychiatry. 1987 Mar;50(3):285-90. doi: 10.1136/jnnp.50.3.285.
A new clinical entity under the name of "juvenile muscular atrophy of unilateral upper extremity" was first described in 1959. Although about 150 cases in Japan, and several additional cases in other countries, have been clinically reported in the literature, the pathology has remained unknown because of the benign course of the disease. The first necropsy findings are reported, obtained from a patient with this disease, who died of lung cancer at the age of 38, 23 years after the onset of the disease. The lesions existed only in the anterior horns of the spinal cord at C5 approximately T1, particularly marked at C7 and C8, showing shrinkage and necrosis, degeneration of various degrees of large and small nerve cells, and mild gliosis. The pathological findings differ from those of reported cases of spinal vascular disorders, but some circulatory insufficiency in the territory of the spinal cord would seem to be suggested, although the underlying aetiology remains unknown.
1959年首次描述了一种名为“青少年单侧上肢肌肉萎缩”的新临床实体。尽管日本文献中临床报道了约150例，其他国家也有几例，但由于该疾病病程良性，其病理学仍不清楚。本文报告了首例尸检结果，患者患有此病，在发病23年后，38岁死于肺癌。病变仅存在于脊髓C5至T1节段的前角，以C7和C8节段尤为明显，表现为萎缩、坏死，大小神经细胞不同程度的变性，以及轻度胶质细胞增生。病理结果与报道的脊髓血管疾病病例不同，但尽管潜在病因不明，但似乎提示脊髓区域存在某种循环功能不全。
