Secondary Fahr Syndrome Presenting With In-Flight Generalized Status Epilepticus in a Middle-Aged Male

Abstract

Fahr syndrome is a rare neurological condition characterized by bilateral basal ganglia calcifications, typically associated with metabolic disturbances such as calcium-phosphorus imbalance or parathyroid dysfunction. Although often incidental or subclinical, it may present with a range of neuropsychiatric or seizure-related symptoms. Status epilepticus as an initial manifestation is uncommon and rarely reported. We report the case of a 42-year-old male with no prior seizure history who developed generalized status epilepticus during a long-haul international flight. Initial brain imaging revealed extensive bilateral basal ganglia calcifications. Laboratory investigations showed mild ionized hypocalcemia (0.99 mmol/L), secondary hyperparathyroidism, and vitamin D deficiency, with normal renal function. The patient admitted to smoking a significant amount of cannabis (13 joints) before boarding, which may have acted as a seizure trigger in the context of underlying metabolic vulnerability. Secondary Fahr syndrome was diagnosed based on clinical, radiological, and biochemical findings. This case highlights a rare but serious presentation of Fahr syndrome with in-flight generalized status epilepticus.

Keywords: fahr's syndrome; fahr’s disease or fahr’s syndrome; marijuana use; secondary fahr's syndrome; status epilepticus (se).

Figure 1. Non-contrast axial computed tomography (CT) of the brain showing bilateral symmetrical hyperdensities in the globus pallidus (arrows), consistent with calcium deposition.

Figure 2. Axial susceptibility-weighted imaging (SWI) MRI of the brain demonstrating marked hypointensities in the bilateral basal ganglia, consistent with mineral (calcium) deposition (arrows).