Zimelewicz Oberman Dan, Cuello Oderiz Carolina, Baccanelli Matteo, Christiansen Silvia, Zurrú María Cristina
Instituto Universitario Hospital Italiano de Buenos Aires, Argentina. E-mail:
Servicio de Neurología, Hospital Italiano de Buenos Aires, Argentina.
Medicina (B Aires). 2017;77(3):242-244.
Hypertrophic pachymeningitis is an infrequent disorder. It can be idiopathic or secondary to infectious, autoimmune or neoplastic disease. The recently described 〈IgG4-related disease〉 could be the origin of many cases considered cryptogenic. We present the case of a 60-year-old man, with a history of headache and episcleritis in both eyes, with partial response to corticoid therapy. The brain MR study with gadolinium showed enhancement and thickening of the dura mater, extending from lateral wall of left temporal and occipital lobes to ipsilateral tentorium. Meningeal biopsy showed fibrosis and lymphoplasmacytic infiltrate, with more than 10 IgG4+ plasma cells per high power field. After treatment with rituximab there was clinical improvement accompanied by the virtual disappearance of the alterations detected in neuroimaging. Hypertrophic pachymeningitis as a manifestation of IgG4-related disease can be based on MRI findings if plasma IgG4 are elevated.
肥厚性硬脑膜炎是一种罕见的疾病。它可以是特发性的，也可以继发于感染性、自身免疫性或肿瘤性疾病。最近描述的〈IgG4相关性疾病〉可能是许多被认为是隐源性病例的病因。我们报告一例60岁男性患者，有头痛和双眼巩膜炎病史，对皮质类固醇治疗有部分反应。钆增强脑磁共振成像显示硬脑膜强化和增厚，从左侧颞叶和枕叶侧壁延伸至同侧小脑幕。脑膜活检显示纤维化和淋巴浆细胞浸润，每高倍视野有超过10个IgG4+浆细胞。使用利妥昔单抗治疗后，临床症状改善，神经影像学检测到的改变几乎消失。如果血浆IgG4升高，肥厚性硬脑膜炎作为IgG4相关性疾病的一种表现可基于MRI表现。
