Sepsis Unmasking Fahr's Disease

Fahr's disease is a sporadic or familial neurodegenerative disorder characterized by symmetrical calcification of cerebral structures, particularly the basal ganglia, cerebellar dentate nuclei and surrounding white matter, in the absence of metabolic causes of calcification. We report the case of a previously fit, high functioning 58-year-old man who developed catastrophic irreversible neuropsychiatric collapse after sepsis despite appropriate antimicrobial treatment. Cranial computed tomography revealed extensive diffuse calcifications located in unusual areas. Laboratory studies excluded the presence of other pathologic processes leading to secondary intracranial calcification and a multigene panel failed to confirm mutations in the genes currently known to be associated with the disorder, supporting a diagnosis of sporadic Fahr's disease or idiopathic brain calcification. Important diagnostic considerations in the septic patient who develops neurological complications, namely sepsis-associated encephalopathy and antibiotic-associated encephalopathy, are discussed. The patient remains severely handicapped 6 months after the acute event. Patients with clinically silent neurodegenerative/neuropsychiatric conditions, such as Fahr's disease, may present with florid and unpredicted neurological features in the context of systemic illness.