Headache as Initial Presentation of Human Chronic Necrotizing Granulomatous Meningoencephalitis

Abstract

Objective Chronic necrotizing and granulomatous meningoencephalitis is an idiopathic inflammatory disease with possible autoimmune mediated delayed type hypersensitivity response. It commonly affects the central nervous system of dogs and cats. However, this inflammatory disease has rarely been described in humans. Background A 69-year-old right-handed woman presented with worsening subacute headaches that were intermittent, sharp and holocephalic in nature which worsened with coughing and/or laughing. Her initial neurological examination was unremarkable. MRI of the brain revealed multifocal enhancing lesions and edema as well as multifocal signal abnormality throughout the supratentorial and infratentorial parenchyma. Her cerebrospinal fluid showed elevated opening pressure, elevated nucleated cells, elevated protein and normal glucose. CSF cytology, gram stains and cultures were unremarkable. Possibility of sarcoidosis, mycobacterium and fungal etiologies were ruled out. Other work up included but not limited to MRA head, MRV head, diagnostic cerebral angiogram, extensive serum and CSF autoimmune labs were also unremarkable. She underwent a brain biopsy which showed necrotizing granulomatous inflammation with associated dystrophic calcification. Patient was started on high dose methylprednisolone followed by a 6-week prednisone taper. There was a complete resolution of her symptoms, as well as improvement in her follow up MRI brain over a six month period. Design/Methods N/A. Results N/A. Conclusions We describe what we believe would be the third reported case of necrotizing granulomatous meningitis of an unknown cause in humans. This case demonstrates the response of necrotizing granulomatous meningoencephalitis in humans to steroid therapy. Yet, further studies are necessary to determine symptomatology and pathogenesis, as well as the treatment in humans.