肥厚性硬脑膜炎与自发性低颅压综合征的临床分析

Clinical analysis of hypertrophic cranial pachymeningitis and spontaneous intracranial hypotension

目的比较肥厚性硬脑膜炎(HCP)与自发性低颅压综合征(SIH)的临床表现、实验室检查、影像学特点,为鉴别诊断与治疗提供依据。方法分析8例HCP患者及10例SIH患者的临床、实验室检查、影像学资料并对比分析及根据上述资料进行个体化治疗。结果在临床表现为两组患者均有头痛症状、均可伴有脑神经受损症状,但SIH以直立性头痛为主要表现,HCP头痛与体位改变无关;SIH组脑脊液(CSF)压力均低于正常,而HCP组多为正常或偏高。实验室检查HCP组患者红细胞沉降率、血IgG4升高,部分患者p-ANCA阳性;SIH组上述指标基本正常。影像学检查HCP和SIH均可出现硬脑膜增厚、硬膜下积液、硬膜下出血、垂体增大等特征,但SIH组往往还有脑通过幕切迹向下移位。结论 HCP与SIH的影像学特异性不强,主要鉴别点在CSF压力及实验室检查,HCP组患者红细胞沉降率、血IgG4升高,部分患者p-ANCA阳性;SIH组上述指标基本正常。HCP组治疗亦是根据红细胞沉降率、血IgG4等结果调整糖皮质激素剂量。

Objective To analyze the clinical manifestation, examination and imaging features of hypertrophic cranial pachymeningitis（HCP） and spontaneous intracranial hypotension（SIH）, so as to improve the diagnosis and treatment ability. MethodThe clinical, examination and imaging data of eight patients with HCP and ten patients with SIH were analyzed retrospectively, and individualized treatment was performed according to the above data. Results In the clinical performance, two groups of patients had headache symptoms, all of them could be accompanied by cranial nerve injury symptoms, but SIH was mainly manifested by erect position headache. Group HCP headache had nothing to do with body position change. Group SIH cerebrospinal fluid pressure were all lower than normal, while Group HCP were mostly normal or high. In the laboratory examination, the erythrocyte sedimentation rate and blood IgG4 increased in the group HCP, and the p-ANCA was positive in some patients, and the above indexes in group SIH were basically normal. Dural thickening,subdural effusion, subdural hemorrhage and pituitary enlargemen can occur in both HCP and SIH for imaging examination. However, group SIH often has characteristics of down shift of tentorial notch. Conclusion The imaging specificity of HCP and SIH was not strong. The main identification points were cerebrospinal fluid（CSF） pressure and laboratory examination. The erythrocyte sedimentation rate and blood IgG4 increased in the group HCP, and the p-ANCA was positive in some patients, and the above indexes in group SIH were basically normal. The HCP group also adjusted glucocorticoid dose according to erythrocyte sedimentation rate and blood IgG4.