title: Idiopathic hypertrophic cranial pachymeningitis causing progressive polyneuropathies in a child.
abstract: We describe the clinical manifestations, radiological features and response to therapy of a 10-year-old child with idiopathic hypertrophic cranial pachymeningitis, a rare condition with potentially severe disabling neurological consequences. The child presented with progressive cranial polyneuropathies secondary to dural compression of the neural and vascular structures within the cavernous sinus. Corticosteroids and methotrexate proved effective in inducing near complete and sustained remission of the condition. To our knowledge, this is the first case reported of idiopathic hypertrophy cranial pachymeningitis in a child. The experience with oral methotrexate to treat this entity in a child has also never been reported. Idiopathic cranial hypertrophic pachymeningitis should be considered in children presenting with a progressive cranial polyneuropathies.
authors: Department of Pediatrics and Child Health, Faculty of Health Sciences, Stellenbosch University, Tygerberg Hospital, Tygerberg, Western Cape, South Africa. vtoorn@sun.ac.za
authors: Ronald
authors: R
authors: van Toorn
authors: Monika
authors: M
authors: Esser
authors: Derrick
authors: D
authors: Smit
authors: Savvas
authors: S
authors: Andronikou
mesh_terms: D020434:Abducens Nerve Diseases / Q000150:complications; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D020226:Cavernous Sinus Thrombosis / Q000473:pathology; D002648:Child; D004172:Diplopia / Q000209:etiology; D004948:Esotropia / Q000209:etiology; D005493:Folic Acid Antagonists / Q000627:therapeutic use; D006801:Humans; D006984:Hypertrophy; D008279:Magnetic Resonance Imaging; D008297:Male; D008581:Meningitis / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D008727:Methotrexate / Q000627:therapeutic use; D008775:Methylprednisolone / Q000627:therapeutic use; D015840:Oculomotor Nerve Diseases / Q000209:etiology; D011115:Polyneuropathies / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology
chemical_list: D000893:Anti-Inflammatory Agents; D005493:Folic Acid Antagonists; D008775:Methylprednisolone; D008727:Methotrexate