Clinical,imaging and pathologic features of hypertrophic cranial pachymeningitis / 临床神经病学杂志

ABSTRACT

Objective To explore the clinical,imaging and pathologic features of hypertrophic cranial pachymeningitis (HCP).Methods The clinical data of one HCP patient who treated in our hospital and other 77 HCP patients from literatures were analyzed retrospectively.Results The clinical manifestation of all the 78 HCP patients had chronic headache,multiple cranial nerves impairment.The secondary was psychiatric disorder(10.3%),ataxia(9.0%) and seizure disorder(6.4%).Hemiplegia,menorrhea and galactosis were found few.Headache was the first onset symptom in the 74 cases (94.9%).HCP was often misdiagnosed subarachnoid hemorrhage,hypotensive cranial pressure headache and cerebral venous sinus thrombosis in early stage.MRI demonstrated local or diffused thickened dura,especially in cerebral falx and/or tentorium of cerebellum,which could be enhanced through reinforced scanning.Pathological evidence indicated an obvious proliferation of dura fiber tissue accompanied with inflammatory cells infiltration.Corticosteroid was effective to all the cases.Conclusions The clinical manifestation of HCP was multiplicity,but it mainly was chronic headache and multiple cranial nerves impairment.MRI demonstrates local or diffused thickened dura especially in cerebral falx and/or tentorium of cerebellum.MRI has an important significance for diagnosis.