Atypical Type of Idiopathic Hypertrophic Pachymeningitis with Predominant Leptomeningeal Enhancement
Sung-Woog Lee, MD, Seol-Hee Baek, MD, Jin-Man Jung, MD, Do-Young Kwon, MD, PhD, Moon Ho Park, MD, PhD
Department of Neurology, Korea University Ansan Hospital, Korea University College of Medicine, Ansan, Korea
Idiopathic hypertrophic pachymeningitis (IHP) is an uncommon chronic inflammatory disorder that
causes a localized or diffuse fibrous thickening of the dura matter. It presents as diverse neurological
symptoms attributed to meningeal involvement and increased intracranial pressure. IHP is diagnosed
by magnetic resonance image (MRI) with gadolinium-enhancement and brain biopsy, which
can reveal an inflammatory change and diffuse fibrosis of dura matter. It has been reported that IHP
might fairly well respond to corticosteroid treatment or immunosuppressant. However, we present
here on a case of atypical type of IHP with predominantly leptomeningeal enhancement and poor response
to steroid and immunosuppressive agent.
Journal of Multiple Sclerosis 4(2):57-60, 2013
