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

Abstract

The problem of diagnosing vasculitic neuropathy is discussed based on case reports of two patients with Wegener's granulomatosis. One patient developed de novo 6(th) 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 organ-specific 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.

Neurological involvement in Wegener's granulomatosis

“…Cranial nerve palsy may occur as single or multiple cranial nerve involvement and may precede the involvement of other organs [9,10 ]. Among the cranial nerves, the most frequent involvement has been described for the optic nerve which is usually compromised by granulomatous mass formation in the orbit [5,8,10 ].…”

Neurological manifestations in ANCA-associated vasculitis - assessment and treatment

“…can be involved because of pachymeningitis or other inflammatory process [14]. Clinical presentation involves visual impairment, olfactory impairment, facial nerve palsy, dysphagia and sensory disorders.…”

Imaging Features of Granulomatosis with Polyangiitis (Wegener’s)

“…Cranial nerve palsy occurs as palsy of single or multiple cranial nerves. It may precede the involvement of other organs (Novack et al 2009). Palsies of cranial nerves are due to a granulomatous mass formation in the orbit, a destructive granulomatous inflammation (third to 12th cranial nerves), or as a vasculitis manifestation (fifth, sixth, and seventh cranial nerves).…”

“…Peripheral cranial nerves, especially the third, fifth, seventh, and eighth, account for about 50% of pachymeningitis cases (Anderson et al 1975;Newman et al 1995;Novack et al 2009;Frith 2010).…”