Wegener's granulomatosis in an adolescent presenting with pachymeningitis, mastoid effusion and Horner's syndrome

Wegener's granulomatosis (WG) classically affects the upper and lower respiratory tracts as well as the renal system, while central nervous system (CNS) involvement is rare. We report a 17-year-old patient with WG who presented with a 2-month history of meningeal signs, left Homer's syndrome, multiple cranial neuropathies and a left middle ear effusion. MRI demonstrated left middle ear and mastoid effusions, pachymeningitis with diffuse left temporal dural enhancement, and right sinus destructive changes. In this report, we will review diagnostic and therapeutic approaches to Wegener's granulomatosis, with a particular emphasis on CNS manifestations and the adolescent population. (C) 2010 Elsevier Ireland Ltd. All rights reserved.