IDIOPATHIC HYPERTROPHIC PACHYMENINGITIS - A CASE REPORT

Idiopathic Hypertrophic Pachymeninghits is a rare pathological state, with still unclear aetiopathogenesis.. It presents with headache, cranial Neuropathies and ataxia occurring alone or in combinations. The disease was diagnosed with magnetic resonance imaging (MRI) and histopathological assesment of the pachymeningeal biopsy specimen.The disease may have remitting and relapsing course and usually response to steroids. We report a case of 40 year old man with cranial veriety of this desease. Our patient presented with headache, reccurent episodes of generalised tonic clonic seizures, behavioural disturbance and defective vision in right eye. MRI showed prominent pachymeningeal thickening on imaging. Dura mater biopsy revealed meningeal thickening and non specific chronic inflammation of the dura. Clinical improvement was noted in our patient. Early institution and long term maintenance of steroid theraphy prevents neurologic sequelae.