Mixed Connective Tissue Disorder Associated Rare Case of Hypertrophic Pachymeningitis

Dr. Kinjal Patel, Dr. Kaushik Rana, Dr. Jay Patel

Abstract: Hypertrophic pachymeningitis is a inflammatory disease of the dura matter characterised by localized or diffuse thickening of the dura mater with fibrosis. It is rare disorder of diverse etiology like secondary to infections, carcinomatosis, or inflammatory diseases or part of mixed connective tissue disorder. It may also be idiopathic. We report a case of pachymeningitis which was a 45 year-old female presented with left-extremity focal seizure and paraesthesia of both lower limbs and blurring of vision. Magnetic resonance imaging (MRI) revealed a diffuse thickening and enhancement of the right convexity dura matter and falx with focal nodularity. She was diagnosed to have mixed connective tissue disorder. The disease may have a remitting and relapsing course. She responded to steroids. She had relapse of disease after one month and recovered with immunosuppresants.

Keywords: Hypertrophic pachymeningitis, Mixed connective tissue disorder, focal seizure