Rigon G, Baratti M, Quaini F, Calzetti S
Divisione Neurologica, Università di Parma.
Minerva Med. 1987 Sep 15;78(17):1325-9.
The authors report a 69-year-old man affected by polycythaemia rubra vera since the age of sixty, who presented persistent left hemichorea of acute onset, occurring ten months before the exacerbation of the haematologic abnormalities. Previously he had been suffering from a transient episode of chorea concomitant with relapsing of polycythaemia. CT scan of the brain showed diffuse cortico-subcortical atrophy, whereas Doppler velocimetry of carotid arteries demonstrated a rise in cerebral blood flow resistance and a stenosis of the right carotid artery in its first tract. Pathogenetic mechanisms of chorea complicating polycythaemia vera are discussed and a multifactorial origin of the syndrome is suggested.
作者报告了一名69岁男性,自60岁起患有真性红细胞增多症,出现急性起病的持续性左侧偏身舞蹈症,发生在血液学异常加重前10个月。此前他曾有过一次与真性红细胞增多症复发同时出现的短暂舞蹈症发作。脑部CT扫描显示弥漫性皮质-皮质下萎缩,而颈动脉多普勒测速显示脑血流阻力增加以及右侧颈动脉第一段狭窄。文中讨论了真性红细胞增多症并发舞蹈症的发病机制,并提出该综合征有多种因素起源。