Idiopathic hypertrophic cranial pachymeningitis: Report of 2 cases & Review of literatures

ABSTRACT

Idiopathic hypertrophic cranial pachymeningitis(IHCP) is a rare chronic pro gressive fibrosing inflammation of pachymeninges of unknown origin. Since the spreading of CT and MRI, there has been a few cases of IHCP have been reported. We describe two patients of IHCP with brain parenchymal involvement presented as epilepsia partialis continua which has not been described as a symptom of IHCP and review the previous reported literatures. IHCP commonly presents headache, multiple cranial nerve palsy, ataxia, and sometimes seizure. In many cases, the CSF finding is noninfectious inflammation, and the erythrocyte sedimentation rate is elevated. The brain MRI is the best noninvasive tool for diagnosis of IHCP and shows diffuse thickening and enhancing dura, especially posterior fossa. IHCP responds steroid initially but recurs frequently and progresses chronically.