Huang Meng, Steele William J, Baskin David S
Department of Neurosurgery, Kenneth R. Peak Brain and Pituitary Center, Houston Methodist Neurological Institute, Houston, TX, USA.
Surg Neurol Int. 2015 Sep 28;6(Suppl 16):S407-13. doi: 10.4103/2152-7806.166176. eCollection 2015.
Primary central nervous system (CNS) vasculitis is an idiopathic inflammatory process that selectively affects CNS vasculature without a systemic inflammatory response, and causes luminal obstruction with resultant ischemia of recipient tissue. Its varying clinical symptoms and signs depend on the caliber of vessels involved and distribution and location of the affected structures. Granulomatous hypophysitis (GH) is an autoimmune inflammatory process typically affecting women, and usually presents with hypopituitarism, and at times, diabetes insipidus, and/or visual loss. Both entities are rare CNS diseases, which, to our knowledge, have never been previously reported in the same patient.
We present a unique case of chronic progressive primary CNS vasculitis causing limbic encephalopathy in a 30-year-old male with only a history of medication-controlled hypertension. He initially presented 4 months prior with nonspecific neurological complaints and was found to have a homogenously enhancing and enlarged pituitary, which was biopsy proven to be GH.
This rather unique presentation highlights the need to maintain a high index of suspicion for underlying PCNS vasculitis in a patient who does not fit the typical demographic for isolated GH.
原发性中枢神经系统（CNS）血管炎是一种特发性炎症过程，可选择性地影响CNS血管系统而无全身炎症反应，并导致管腔阻塞，进而引起受影响组织的缺血。其不同的临床症状和体征取决于受累血管的管径以及受影响结构的分布和位置。肉芽肿性垂体炎（GH）是一种通常影响女性的自身免疫性炎症过程，通常表现为垂体功能减退，有时还伴有尿崩症和/或视力丧失。这两种疾病均为罕见的CNS疾病，据我们所知，此前从未有过同一患者同时患有这两种疾病的报道。
我们报告了一例独特的慢性进行性原发性CNS血管炎病例，该病例导致一名30岁男性出现边缘性脑炎，该男性仅有药物控制的高血压病史。他最初在4个月前出现非特异性神经症状，检查发现垂体均匀强化且增大，活检证实为GH。
这种相当独特的表现突出了对于不符合孤立性GH典型人群特征的患者，需要高度怀疑其潜在的PCNS血管炎。
