Fahrs Syndrome due to Hypoparathyroidism Following Thyroidectomy

Abstract

Fahrs syndrome is a rare, degenerative, and neuropsychiatric disorder characterized by seizures, extrapyramidal and neuropsychiatric symptoms as a result of symmetric and bilateral calcifications of the nucleus pallidus, the putamen, the dentate nucleus of the cerebellum, and the hemispheric white matter at the base of the skull. The female patients, who has 53-year-old, was admitted in the intensive care unit with status epilepticus for 24 hours associated to acute respiratory failure due to aspiration pneumonia. She had undergone a thyroidectomy 22 years ago, when she started involuntary lasting muscular contractions of the upper extremities, which progressed to convulsions throughout the years. There were Chvostek and Trousseau signs. Laboratory tests showed ionic calcium of 0.55 mmol/L. Computed tomography scan of the brain showed intracranial symmetrical calcifications of the basal nuclei, semioval center, occipital, and cerebellar cortex. According to the clinical presentation, examination findings, and subsequent investigation exams, our case was diagnosed as Fahrs syndrome due to post-thyroidectomy hypoparathyroidism. Fahrs syndrome should be considered in patients with neuropsychiatric disturbances and seizure disorder, especially following thyroidectomy.




J Med Cases. 2013;4(6):380-384
doi: https://doi.org/10.4021/jmc1252e