Dawoud Fakhry, Lucke-Wold Brandon, Trejo-Lopez Jorge, Yachnis Anthony, Rahman Maryam
Quillen College of Medicine, East Tennessee State University, United States.
Department of Neurosurgery, University of Florida, United States.
Neurol Sci Neurosurg. 2020;1(2). doi: 10.47275/2692-093x-109. Epub 2020 Oct 19.
Primary angiitis of the central nervous system (PACNS) is a rare form of vasculitis. It is a diagnosis of exclusion and often diagnosed post mortem on pathologic evaluation. Cerebral angiography can be suggestive, but biopsy is required. Symptoms can vary from headache to focal cranial nerve deficits. On the more severe spectrum, patients can present with ischemic and vary rarely hemorrhagic stroke. We present in this case report key clinical pearls regarding suspected diagnosis. Younger patients with cortical hemorrhages may have PACNS instead of the more common cerebral amyloid angiopathy. Early suspicion may aid in initiating effective treatment as we highlight in the discussion.
中枢神经系统原发性血管炎(PACNS)是一种罕见的血管炎形式。它是一种排除性诊断,常通过病理评估在尸检后确诊。脑血管造影可能具有提示作用,但活检是必需的。症状从头痛到局灶性颅神经功能缺损不等。在更严重的情况下,患者可能出现缺血性中风,很少出现出血性中风。我们在本病例报告中介绍了关于疑似诊断的关键临床要点。患有皮质出血的年轻患者可能患有PACNS,而非更常见的脑淀粉样血管病。如我们在讨论中所强调的,早期怀疑可能有助于启动有效的治疗。