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[Multiple cranial nerve involvement associated with tentorial pachymeningitis of granulomatous type]
Abstract:
Otitis media in a 43 year-old male patient was followed by signs of chronic meningitis with persistent headaches, moderate pleiomorphic lymphocytosis of CSF without hypoglycorrhachia and unilateral impairment of cranial nerves XI to II. These signs were accompanied by widespread inflammation with general deterioration of the patient's condition. After a time the tentorium of cerebellum showed thickening and significant contrast uptake in conjunction with necrotizing granulomatous inflammation, indicating tentorial pachymeningitis. Swift and complete remission was obtained with a corticoid-cyclophosphamide combination. After reviewing the other possible causes of chronic meningitis, the authors proposed a diagnosis of Wegener's disease of auricular origin in view of the compatibility of all the data. A few cases of cranial pachymeningitis of indeterminate origin are described in the literature. Only this patient was treated with a corticoid-cyclophosphamide combination. The remarkable efficacy of this double therapy confirms the diagnostic hypothesis of Wegener's granulomatosis.
Insights
This case study highlights Wegener's granulomatosis presenting as chronic meningitis and tentorial pachymeningitis following otitis media. Treatment with corticosteroids and cyclophosphamide led to complete remission, confirming the diagnosis.
Area of Science:
- Neurology
- Otolaryngology
- Rheumatology
Background:
- Chronic meningitis can present with diverse neurological symptoms.
- Wegener's granulomatosis (granulomatosis with polyangiitis) is a rare autoimmune vasculitis that can affect multiple organ systems, including the central nervous system.
- Cranial pachymeningitis, inflammation of the dura mater, is an uncommon manifestation of systemic diseases.