神经系统疾病

Seronegative Wegener’s Granulomatosis Presenting as a Multiple Cranial Neuropathy

Abstract

Sara Machado, Amélia Nogueira Pinto, Nuno Inácio, Filipe Paulas, Luísa Biscoito, João Paulo Farias, Marta Amaral and José Alves

Wegener’s granulomatosis (WG) is an immune-mediated systemic vasculitis of unknown etiology that can be seen in almost any system. It classically affects the upper and lower airways, lungs and kidneys. Despite its possibility of generalization, neurological involvement is hardly seen. When it occurs, WG tends to affect the peripheral nervous system, often as sensory-motor polyneuropathy or mononeuritis multiplex. Involvement of cranial nerves is much less frequent, and the most affected is the optic nerve. Involvement of the central nervous system is even rarer but there are reports including cerebrovascular events, seizures, cerebritis, diabetes insipidus and pachymeningitis. In 1990, four criteria have been identified for the clinical diagnosis of WG by the American College of Rheumatology, which are still in effect and include upper airways inflammation, pulmonary nodules and microhematuria. A patient who meets at least 2 of the criteria may be diagnosed as having classical WG with a sensitivity of 88% and a specificity of 92%. Usually there is positivity for antineutrophil cytoplasmatic antigene (ANCA) but its absence does not exclude this diagnosis, especially when the clinical picture is highly suggestive. We present a case of palsy of the lower cranial nerves as the initial presentation of a seronegative WG.