Clinical and imaging characteristics of idiopathic hypertrophic pachymeningitis / 中华神经医学杂志

ABSTRACT

Objective To analyze clinical data of 6 cases of idiopathic hypertrophic pachymeningitis and review related literatures before, so as to offer a guidance for its clinical diagnosis and therapy. Methods We analyzed the clinical and imaging features and data of follow_up in the patients who were treated in our hospital 2005-2006. Results Idiopathic hypertrophic pachymeningitis was always accompanied by immune fimction abnormality to different degrees. The main clinical manifestation was headache which was found in every case. In these cases, headache was the onset and recur symptom. In addition to headache, various symptoms of nervous system dysfunction especially the cranial nerves could be found in some cases. In these cases, a localized or diffuse thickening of tentorium of cerebellum and/or falciform process of cerebrum was found in MRI, and intensification was presented in enhancement scanning. Corticosteroid was effective to all of the cases. Conclusions Idiopathic hypertrophic pachymeningitis may be an autoimmune disorder, of which the symptoms are various, and need to be discriminated from other diseases. MR/of cranium is important criteria for diagnosis.