Primary Angiitis Of The Central Nervous System: One Case Report And Review Of Literature

[Background] Primary angiitis of the central nervous system (PACNS) is well confined to the central nervous system(CNS), mainly brain, less common spine cord, which is rare and difficult to diagnose. Its annual incidence rate was about 1-2.4 cases per 1,000,000 person-years. It is a serious disorder leading to 3-5% of cerebrovascular accidents in patients aged under 50 years. To date, there hasn't been any such statistical data in China. The etiology of PACNS is unclear. Cerebral angiography and brain biopsy are important approaches in making the diagnosis. PACNS is a diagnosis of exclusion.[objective] Here we report one biopsy-proven PACNS recently in Qilu Hospital of Shandong University, simultaneously, and review the literature. Based on the analysis of the Chinese patients'clinical features, imaging and histopathological characteristics, we aim to know more about PACNS, which is help to clinical diagnosis and treatment early.[Methods] In this article, we reported one case diagnosed PACNS with biopsy confirmation in Qilu Hospital of Shandong University, and retrospectively reviewed biopsy-proven cases in medical history from 1989 to 2010. All of them were analyzed in clinical features, laboratory tests, imaging findings, and pathological characteristic aspects.[Results] Case report:The 50-year-old male had a fluctuating disease course of more than 3 years. He presented visual disorders, seizure, cognitive impairment, hypersomnia, confusion, gait unsteady, aphasia, dysphasia, deglutition barrier, incontinence. Magnetic Resonance (MR) imaging showed multiple, supratentorial and infratentorial abnormal signals, with nodular and patch enhancement, while cerebrospinal fluid (CSF) analysis, MR angiography (MRA) and cerebral angiography test were normal. MR spectrum (MRS) showed decrease of N-acetyl-aspartate (NAA). Histopathological findings of leptomeningeal and brain biopsy revealed nongranulomatous lymphatic vasculities with reactive gliosis, cicatrization, demyelination and focal haemorrhages. He responsed little to glucocorticoid alone before definitely diagnosed. After receiving oral cyclophosphamide, his symptoms were alleviated slightly.Review of literature:There were 40 pathology confirmed cases of PACNS, with 21 males and 19 females. The median age was 40 years old, ranging from 11 to 70 years old. Most cases had been misdiagnosed as glioma, metastatic carcinoma, multiple sclerosis, etc. The clinical features revealed that headache was the most common symptom(65%), with focal neurologic deficits such as hemiplegia or quadriplegia (50%), epilepsy seizure(30%). Alternation of consciousness and confusion were less common. The erythrocyte sedimentation rate (ESR) was elevated in 3 for 11 patients. The abnormal of CSF with protein content elevated and/or pleocytosis counted for 41%. Brain CT and/or MR imaging showed abnormal lesions for 100% of the cases, located mostly in supratentorial, with the spinal cord involved in 2 cases. Eight cases underwent MRA, and 63% of them displayed segmental stenosis and ectasia, like 'beading'in multiple vessels, with all median cerebral arteries involved. Only 1 in 3 cases had abnormal findings in cerebral angiography. Histologically, there were lymphocytic, granulomatous (5/17), or necrotizing and mixed types in character. After definitely diagnosed,31 cases received corticosteroids and/or suppressive agent, and 28 of them responded well, while 2 died. A follow-up period varied from 4 months to 3 years in 23 cases and 3 cases relapsed.[Conclusions] PACNS is rare, with males slightly outnumbering females. The clinical features are protean, which can be presented as cerebrovescular incidents or encephalopathy. CSF tests usually show mild increase in mononuclear cell count or protein content, or both. Lesions on MR are high of diversity. Biopsy is the "gold standard" for PACNS. Multiple modities of cerebral angiography and biopsy, combined with medical history can enhance the diagnosis potential maximally. Corticosteroids and/or suppressive agent usually make PACNS remission. The response and relapse rate are not associated with pathological types.