Idiopathic Hypertrophic Pachymeningitis presenting with hydrocephalus in a child

Idiopathic hypertrophic pachymeningitis (IHP) is a rare but increasingly recognized disorder with localized or diffuse thickening of the dura mater of unknown etiology. The inflammation usually involves the cranial or spinal dura mater, with resultant neurological deficits. It has been primarily reported in adults, with focal involvement of the brain or spinal cord. We report a child who presented at the age of 3.5 years with IHP involving the whole neuraxis, with poor response to steroids,cyclophosphamide and intraventricular Cytarabine