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Eur J Paediatr Neurol. 2008 Mar;12(2):144-7. Epub 2007 Sep 18.

Idiopathic hypertrophic cranial pachymeningitis causing progressive polyneuropathies in a child.

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Department of Pediatrics and Child Health, Faculty of Health Sciences, Stellenbosch University, Tygerberg Hospital, Tygerberg, Western Cape, South Africa.


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.

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