「Lindau病」—2家系の症例報告および文献的考察

"LINDAU'S DISEASE":Report of two pedigrees and review of the literature

　I．はじめに　同一家系に多発する脳腫瘍は，稀れであるが，heman gioblastomaは，しばしば家族性に発症するので有名である。われわれは最近，続いて，独立の2つのheman gioblastomaの家系を経験したが，その1家系には，きわめて濃厚な遺伝関係が考えられ，優生学的見地からも興味深いもので，ここにその2家系の症例について報告する。なお，従来「Lindau病」と呼ばれる疾患があるが，その意味する内容については，必ずしも諸家の意見が一致せず，混乱をきたしているので，この「Lindau病」という定義について，われわれの見解を述べ，あわせて，文献的考察を加えたいと思う。

Among brain tumors, hemangioblastomas are some-times found in the same family. Among 28 cases of hemangioblastomas of the brain we have experienced, we reported here three patients of two pedigrees who suffered from the hemangioblastoma of the posterior fossa with or without angiomatosis retinae. In one pedigree the Mendelian dominant factor was sugges-tive.

In two cases, the location of the hemangioblastoma was interesting : one at the area postrema with syrin-gomyelia and the one at the medulla oblongata with angioma at the area postrema.

In two cases, erythrocytosis was present and in one case we measured the activity of erythropoietin in the plasma, CSF and tumor tissue. The activity in the plasma was elevated.

Many authors have different meanings for 'Lindau's disease'. With regard to the nomenclature of 'Lindau's disease', we have the opinion that it may be used preferably for the autopsied cases, and clinically the hemangioblastoma of the brain (infratentorial, spinal cord and supratentorial) and/or angiomatosis retinae should be included under the name of 'Hemangio-blastoma of the central nervous system' in a wider sense and if used clinically, 'Lindau's disease ' should be applied for the case of the hemangioblastoma of the brain with angiomatosis retinae.