A 67-year-old woman with polymyalgia rheumatica and left hemispatial neglect

A 67-year-old woman with a diagnosis of polymyalgia rheumatica presented initially with periods of confusion and incontinence. A CT scan of the brain was normal and she was treated with tapering doses of corticosteroids and clinical improvement. After a brief period off steroids, the patient presented with a progressive dementia, left-sided clumsiness, gait disturbances and left hemispatial neglect. An MRI at this time demonstrated a large area of edema over the right parietal lobe and intense cortical enhancement. A chest CT demonstrated multiple nodules. Biopsies of the lung and brain failed to identify any infectious organisms or malignant tissue. The leptomeningeal biopsy revealed multiple granulomatous areas with central necrosis and hystiocytic cells consistent with idiopathic hypertrophic pachy-meningitis.