Almarzooqi Ali, Mairajuddin Samar Iltaf, Ahmad Dar Javeed A, Shaffi Al Madani Abubaker Abdul Rahman
Neurology, Rashid Hospital, Dubai, ARE.
Cureus. 2025 Apr 28;17(4):e83114. doi: 10.7759/cureus.83114. eCollection 2025 Apr.
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.
Fahr综合征是一种罕见的神经系统疾病,其特征为双侧基底节钙化,通常与钙磷失衡或甲状旁腺功能障碍等代谢紊乱有关。虽然通常为偶然发现或亚临床状态,但它可能会出现一系列神经精神症状或与癫痫相关的症状。癫痫持续状态作为初始表现并不常见,报道也很少。我们报告一例42岁男性,既往无癫痫病史,在一次长途国际航班飞行期间发生了全身性癫痫持续状态。最初的脑部影像学检查显示广泛的双侧基底节钙化。实验室检查显示轻度离子钙降低(0.99 mmol/L)、继发性甲状旁腺功能亢进和维生素D缺乏,肾功能正常。患者承认登机前吸食了大量大麻(13支),这在潜在代谢易感性的情况下可能引发了癫痫发作。根据临床、影像学和生化检查结果诊断为继发性Fahr综合征。该病例突出了Fahr综合征一种罕见但严重的表现形式,即飞行中全身性癫痫持续状态。