Clinical Presentations And Imaging Features Of Hypertrophic Cranial Pachymengitis

Objective: To explore the clinical presentations and imaging features of hypertrophic cranial pachymengitis(HCP).Methods: The clinical data of one HCP patient who was finally diagnosed and cuerd in our hospital and other 153 patients from literatures were analyzed retrospectively.Results: It was chronic onset in 138(89.6%)patients of 154 patients. The first sympotom of HCP was headache in 136(88.3%) patients. It was multiple cranial nerves impairment subsequently. It was diplopia in 61(39.6%)patients, desent of auditory in 37(24.0%) patients, desent of visual acuity in 35(22.7%) patients, sensory disturbance of head and face in 28(18.2%) patients, facial paly in 27(17.5%)patients,dysphagia, dysarthria or hoarse voice in 17(11.0%) patients, atrophy of lingual muscle in 11(7.1%) patients, ataxia in 28(18.2%) patients and so on. Hemogram detection: it was the augment of white blood cell in 12 patients; it was the augument of C reactive protein in 25 patients; it was the augument of erythrosedimentation in 38 patients. CSF detection: it was the augment of white blood cell in 44 patients, amplitude:(8~2260)×10~6/L and the majority were less than 50×10~6/L; it was the augment of proteinum in 66 patients, amplitude:(8~2260)×10~6/L and the majority were less than 1000mg/L. MRI of head demonstrated local or diffused thickened in cerebral flax, entorium of cerebellum, and the dura of frontal region, temporalis, parietalis and occiput, which could be enhanced through reinforced scanning. 73(47.4%) patients were misdiagnosed early. The 128 patients were treated by corticosteroid hormone and 11 patients whose pathogenetic condition recured were treated by immunodepressant.Conclusion: The first sympotom of HCP was chronic headache. It was multiple cranial nerves impariment and cerebellar ataxia subsequently. laboratory examination is not especificity . mageology can demonstrate thickened dura which can be reinforced in enhancement scanning. The MRI of head has an important significance for diagnosis.The treatments are corticosteroid hormone and immunodepressant.