Journal of Neurology

, Volume 256, Issue 3, pp 299-304

First online:

Cranial nerve palsy in Wegener’s granulomatosis – Lessons from clinical cases

  • R. NowackAffiliated withNephrology/Dialysis centre Lindau Email author 
  • , P. WachtlerAffiliated withNephrology/Dialysis centre Lindau
  • , J. KunzAffiliated withNeurologische Gemeinschaftspraxis
  • , N. RasmussenAffiliated withEar, Nose & Throat Department

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The problem of diagnosing vasculitic neuropathy is discussed based on case reports of two patients with Wegener’s granulomatosis. One patient developed de novo 6th nerve palsy as an isolated relapse manifestation and the second patient a sequence of multiple cranial nerve palsies. Brain imaging with CT and MRI and the laboratory provided no clues suggesting active vasculitis. However, in both patients the neuropathies fully recovered in response to standard induction protocols of vasculitis. In the absence of organspecific proof of vasculitis, these treatment decisions were guided by the overall clinical presentations. Cranial neuropathy may be the first obvious vasculitic manifestation preceding other organ disease, and since single reliable tests for its diagnosis are lacking, a multidisciplinary approach is advocated here to detect vasculitic manifestations in other organs.

Key words

ANCA systemic vasculitis abducens nerve palsy vagal nerve palsy Wegener’s granulomatosis cranial neuropathy