Neurology Department, Hospital de Santo António, Centro Hospitalar do Porto, Porto, Portugal.
Neuroradiology Department, Hospital de Santo António, Centro Hospitalar do Porto, Porto, Portugal.
Neurol Sci. 2019 Mar;40(3):543-551. doi: 10.1007/s10072-018-3689-3. Epub 2018 Dec 26.
Hypertrophic pachymeningitis (HP) is characterized by cranial and/or spinal thickening of the dura mater with or without associated inflammation. Neuroimaging studies reveal dura mater thickening and focal or diffuse contrast enhancement. It is described in association with trauma, infections, tumors, autoimmune/inflammatory diseases, and cerebrospinal fluid hypotension syndrome, with some cases remaining idiopathic.
A retrospective study was conducted with patients' identification through a key terms search within MRI reports in the period of July 2008 to September 2015. Clinical files, MRI, laboratory, and pathology data were reviewed.
Fifty-three patients were identified and 20 were excluded because they did not meet the inclusion criteria. Of the 33 included, 19 were female, with a mean age at symptoms onset of 51.2 ± 17.6 years. The most common presenting symptoms were headache and cranial nerves palsy, followed by seizures, delirium, lumbar pain, cognitive decline, motor deficit, and language impairment. In 17 patients, a neoplastic etiology was identified; in eight, inflammatory/autoimmune; in six, infectious; and two were classified as idiopathic. Of the eight patients with inflammatory/autoimmune etiology, four had possible IgG4-related disease (IgG4-RD) and the remaining had granulomatosis with polyangiitis, sarcoidosis, rheumatoid arthritis, and Tolosa-Hunt syndrome. Treatment was directed according to the underlying etiology.
In the described series, a female predominance was identified, with symptoms' onset in the 5th decade. Although headache was the most common symptom, clinical presentation was varied, emphasizing the role of MRI in HP diagnosis. The underlying etiologies were diverse, with only a few cases remaining idiopathic, also reflecting the contribution of the recently described IgG4-RD.
肥厚性硬脑膜炎（HP）的特征是硬脑膜的颅部和/或脊柱增厚，伴有或不伴有相关炎症。神经影像学研究显示硬脑膜增厚和局灶性或弥漫性对比增强。它与创伤、感染、肿瘤、自身免疫/炎症性疾病和脑脊液低血压综合征有关，有些病例仍然是特发性的。
通过 2008 年 7 月至 2015 年 9 月 MRI 报告中的关键词搜索进行回顾性研究，对患者进行识别。对临床档案、MRI、实验室和病理数据进行了回顾。
共识别出 53 例患者，20 例因不符合纳入标准而被排除。在 33 例纳入的患者中，19 例为女性，症状发作时的平均年龄为 51.2±17.6 岁。最常见的首发症状是头痛和颅神经麻痹，其次是癫痫发作、意识障碍、腰痛、认知功能下降、运动障碍和语言障碍。在 17 例患者中，确定了肿瘤性病因；8 例为炎症/自身免疫性病因；6 例为感染性病因；2 例为特发性病因。在 8 例炎症/自身免疫性病因患者中，有 4 例可能为 IgG4 相关疾病（IgG4-RD），其余患者分别为肉芽肿性多血管炎、结节病、类风湿关节炎和托洛萨-亨特综合征。治疗是根据潜在病因进行的。
在所描述的系列中，女性占优势，症状发作于 50 岁。虽然头痛是最常见的症状，但临床表现多种多样，强调了 MRI 在 HP 诊断中的作用。潜在病因多种多样，只有少数病例仍然是特发性的，这也反映了最近描述的 IgG4-RD 的作用。
