al-Hakim M, Katirji B, Osorio I, Weisman R
Department of Neurology, Case Western Reserve University, Cleveland, OH.
Neurology. 1993 Apr;43(4):742-6. doi: 10.1212/wnl.43.4.742.
We report the clinical, radiologic, and postmortem findings in two patients with paroxysmal nocturnal hemoglobinuria (PNH) who developed cerebral venous thromboses (CVTs). In contrast with those in most published cases, our patients did not have focal neurologic signs. Antemortem diagnosis of CVT had been made by MR cerebral venograms. We conclude that (1) PNH should be considered in any patient with stroke associated with iron deficiency anemia, hemolysis, hemoglobinuria, or hemosiderinuria; (2) PNH should be in the differential diagnosis of CVT; (3) the latter could present without focal neurologic signs; and (4) MR cerebral venography may be a reliable diagnostic alternative to cerebral angiography when CVT is suspected.
我们报告了两例阵发性夜间血红蛋白尿（PNH）并发脑静脉血栓形成（CVT）患者的临床、影像学及尸检结果。与大多数已发表病例不同的是，我们的患者没有局灶性神经体征。CVT的生前诊断通过磁共振脑静脉造影做出。我们得出结论：（1）对于任何伴有缺铁性贫血、溶血、血红蛋白尿或含铁血黄素尿的中风患者，都应考虑PNH；（2）PNH应列入CVT的鉴别诊断；（3）CVT可能不伴有局灶性神经体征；（4）当怀疑CVT时，磁共振脑静脉造影可能是一种可靠的替代脑血管造影的诊断方法。
