Fahr’s Syndrome in the Setting of Abnormal Calcium-Phosphate Metabolism and Lupus Nephritis

Fahr’s syndrome is a rare neurodegenerative disorder characterized by bilateral calcifications of the basal ganglia and cerebral cortex. These deposits are made of calcium and phosphorus and are thought to be due to abnormalities in calcium-phosphate homeostasis. The clinical manifestation includes extrapyramidal symptoms (e.g., spastic paralysis), generalized or partial seizures, cognitive impairment, and neuropsychiatric symptoms. Here, we discuss a case of a young female with a medical history of systemic lupus erythematosus (SLE) and lupus nephritis who developed generalized seizures and was found to have extensive bilateral calcifications in the basal ganglia and cerebral cortex. Her laboratory workup showed disorganized calcium-phosphorus homeostasis.