Université catholique de Louvain, Cliniques universitaires Saint-Luc, avenue Hippocrate 10, B-1200 Bruxelles, Belgium.
Eur J Paediatr Neurol. 2012 Mar;16(2):196-9. doi: 10.1016/j.ejpn.2011.06.006. Epub 2011 Jul 23.
Primary angiitis of the central nervous system (PACNS) is an idiopathic, usually recurrent vasculitis confined to the brain. PACNS has been reported rarely in children, although the disease is probably underdiagnosed.
: We report the clinical history of a 3-year-old girl who presented subacute neurological deterioration characterised by headache, speech regression, and altered level of consciousness. Brain MRI revealed severe inflammatory lesions involving both grey and white matters. All blood and cerebrospinal fluid (CSF) tests for inflammatory or infectious processes were negative. Over the next 10 years, the patient relapsed eight times. Brain biopsy confirmed lesions suggestive of cerebral vasculitis. Based on histopathological features and due to the absence of systemic vasculitis, the patient was considered to have PACNS. She developed partial epilepsy, and clinical stabilisation was finally achieved via continuous oral corticosteroids and immunosuppressive agents.
PACNS may be the cause of subacute and relapsing inflammatory encephalopathy in children after excluding other diagnoses, such as multiple sclerosis, sarcoidosis, recurrent acute disseminated encephalomyelitis (ADEM), and primary central nervous system lymphoma. Brain biopsy is necessary to confirm the diagnosis of PACNS and exclude diseases with similar symptoms. Neurological outcome remains poor.
原发性中枢神经系统血管炎（PACNS）是一种特发性、通常为复发性血管炎，局限于大脑。PACNS 在儿童中很少见，尽管该病可能被漏诊。
我们报告了一名 3 岁女孩的临床病史，她表现为亚急性神经功能恶化，伴有头痛、言语退化和意识水平改变。脑 MRI 显示严重的炎症性病变累及灰质和白质。所有血液和脑脊液（CSF）检查均未发现炎症或感染过程。在接下来的 10 年中，该患者复发了 8 次。脑活检证实了提示脑血管炎的病变。基于组织病理学特征，且由于不存在系统性血管炎，该患者被认为患有 PACNS。她发展为部分性癫痫，通过持续口服皮质类固醇和免疫抑制剂最终实现了临床稳定。
在排除多发性硬化症、结节病、复发性急性播散性脑脊髓炎（ADEM）和原发性中枢神经系统淋巴瘤等其他诊断后，PACNS 可能是儿童亚急性和复发性炎症性脑病的病因。为了确诊 PACNS 并排除具有相似症状的疾病，有必要进行脑活检。神经预后仍然较差。
