Widespread cranial nerve palsies while on cyclophosphamide therapy: a very rare manifestation of Wegener's granulomatosis (granulomatosis with polyangitis)

Wegener's granulomatosis (granulomatosis with polyangitis) is a rare autoimmune disease causing necrotising vasculitis of small blood vessels, mainly affecting the upper (90%) and lower (60%) respiratory tract tissues and kidneys (75%). Central nervous system (CNS) involvement is limited to 2–8% of cases while cranial nerve involvement is seen in only 1% of cases. Most cases are due to the granulomatous involvement of meninges (pachymeningitis), resulting in cranial nerve palsies. Very rarely, CNS lesions are caused by vasculitis of small vessels. We report a case of a man who, while being treated with cyclophosphamide, presented with an acute flare of the disease affecting all the cranial nerves except the olfactory, trigeminal and facial nerves. Extensive investigations failed to show any meningeal involvement, supporting the view of extensive small vessel vasculitis. It is extremely rare to have such an extensive cranial nerve involvement with no evidence of meningeal involvement. Immunosuppressive therapy with cyclophosphamide is considered to be the mainstay of the treatment (along with steroids) with complete remission seen in a large majority of cases. However, in this case, he was refractory to cyclophosphamide and also failed to respond to alternative therapy with rituximab (a monoclonal antibody that binds to CD20). After being on a high-dose steroid therapy for nearly a year, he successfully responded to subcutaneous methotrexate therapy, thus enabling the steroid dose to be reduced significantly. A 59-year-old Caucasian man with no significant medical history was diagnosed with Wegener's granulomatosis (granulomatosis with polyangitis) when he presented with deafness, epistaxis, haemoptysis, cavitating lung lesion on chest X-ray, high inflammatory markers, a very positive …