Secondary Normal-Pressure Hydrocephalus in Rheumatoid Meningitis

Abstract

Normal-pressure hydrocephalus (NPH) is a common cause of gait apraxia, cognitive impairment, and urinary incontinence in the elderly. It is usually a primary idiopathic disorder but can be secondary. We present a case of secondary NPH due to biopsy-confirmed rheumatoid meningitis initially refractory to intravenous (IV) immunotherapy. Our patient reported an excellent response right after shunting. Her gait remains normal one and a half years later. We searched PubMed for similar cases of rheumatoid meningitis with gait abnormality for additional clinicopathologic discussion. The patient’s movement disorder initially improved with steroid taper. However, she developed progressive symptoms, later on, refractory to IV solumedrol and rituximab. She underwent ventriculoperitoneal shunting (VPS) and reported an outstanding outcome. This is the first reported biopsy-confirmed case of rheumatoid meningitis causing NPH to undergo shunting for immediate improvement. Previous cases of rheumatoid meningitis-associated Parkinsonism have improved with steroid induction. Although our patient’s rheumatoid arthritis is now controlled, her case illustrates that NPH in autoinflammatory conditions may not recover with immune suppression alone. VPS is an option for a faster response in secondary NPH due to rheumatoid meningitis or other inflammatory disorders with progressive symptoms despite standard induction therapy.