Wegener Granulomatosis (WG) is a multisystem autoimmune disorder characterized by necrotizing granulomatous vasculitis that most commonly involves the upper respiratory tract, lungs, and kidneys. The involvement of the central nervous system (CNS) is infrequent and can cause stroke, cranial nerve abnormalities, cerebrovascular events, seizures, and meningeal involvement. Meningeal involvement is rare and may occur due to local vasculitis, directly spread from adjacent disease in the skull base, paranasal or orbital region. We describe the case ofa 20-year-old Caucasian man who was diagnosed with sinonasal WG with frontal focal meningeal involvement. A literature review on diagnosis and treatment of meningeal involvement in course of WG was carried out. The importance of an early diagnosis and treatment of localized WG has been emphasized, in order to avoid the progression to a severe form of disease, especially in younger patients and in paucisymptomatic cases.
Case reportA twenty-year-old Caucasian man with history of parietaria allergy presented with nasal congestion, occasional epistaxis and purulent rhinorrhea that had started 1 year previously. He had been seen at another hospital and had received a diagnosis of allergic rhinosinusitis. Large doses of intranasal steroids and multiple courses ofantibiotic therapy did not result in an improvement. An X-ray of the paranasal sinuses carried out six months previously was negative. His familial history was unremarkable; his parents were both living and well; the patient was the only child, he was a medical student at University in his own city, he was single and lived with his parents. He had no allergies to medications, and he denied use of alcohol, illicit drugs and smoking.On admission to our Department, headache, neck stiffness, dyspnoea, cough, fever, night sweats, fatigue, myalgia or arthralgia were absent. On examination, the patient was alert, oriented, and cooperative. There was nasal congestion and copious discharge. The lungs were clear on the auscultation, the cardiac and abdominal examinations were normal. Neurologic examination was negative for focal motor or sensory deficits as well as examination of cranial nerves II through XII. A complete blood count revealed a white-cell count of 8,430 per cubic millimeter, with 46% polymorphonuclear cells. The hematocrit was 41%, and the platelet count 296,000 per cubic millimeter. The blood urea nitrogen level was 27 mg per deciliter, and the creatinine level 0.70 mg per deciliter. Erythrocyte sedimentationrate (ESR)