静脈管開存症による後天性肝脳変性症と考えられた若年女性の1例

A case of acquired hepatocerebral degeneration caused by patent ductus venosus

後天性肝脳変性症（acquired hepatocerebral degeneration：AHD）は肝硬変または門脈体循環シャントを背景として発症する慢性神経症候群であり，肝硬変を背景とした中高年患者の症例が多く報告されているが，若年者の報告は非常にまれである。今回我々は，頭部MRI所見を契機に高マンガン血症および静脈管開存症が発見されAHDと考えられた若年女性の1例を経験したので，若干の文献的考察と併せて報告する。

This is an unusual case of a 15-year-old female with a bilateral hand tremor and headache that led to the discovery of a patent ductus venosus and possible acquired hepatocerebral degeneration. Porto-systemic shunts（PSS）are considered an important cause of elevated manganese in children and adults without liver disease. Acquired hepatocerebral degeneration is usually diagnosed based on the presence of PSS or chronic liver disease, bilateral symmetric hyperintensity in globus pallidus on T1-weighted MR imaging, and neurological symptoms. Brain MR studies play an important role in the diagnosis of acquired hepatocerebral degeneration in patients without liver disease, allowing for body screening to detect PSS.