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Scholars Journal of Medical Case Reports | Volume-14 | Issue-10
Fahr Syndrome Presenting in the Emergency Department: A Series of Eight Cases and a Review of the Literature
N. Smirko, N. Chouaib, S. Jidane, A. Belcouch, M. Lekhlit, T. Nebhani, S. Zidouh, L. Belyamani
Published: Oct. 24, 2026 |
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18
Pages: 2321-2324
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Abstract
Introduction: Fahr syndrome (FS) is characterised by bilateral, symmetrical, non-arteriosclerotic calcifications of the basal ganglia. It is a rare condition with heterogeneous clinical manifestations and is most commonly associated with parathyroid disorders. Patients and methods: We conducted a retrospective descriptive study of eight patients admitted to the medical and surgical emergency department of Mohammed V Military Teaching Hospital over a nine-year period, all of whom had basal ganglia calcifications on brain computed tomography (CT). Results: The mean age was 52 years (range, 39–60 years), and six patients were women. Clinical manifestations were non-specific and included seizures (n = 4), cognitive impairment (n = 3), altered consciousness (n = 3), psychiatric symptoms (n = 2), and signs of hypocalcaemia (n = 2). FS was detected incidentally in three patients during the evaluation of ischaemic stroke, haemorrhagic stroke, and shock, respectively. Abnormalities of calcium and phosphate metabolism were identified in seven patients, with hypoparathyroidism in four (including two following thyroidectomy) and pseudohypoparathyroidism in three. Electroencephalography, performed in four patients, showed background slowing or diffuse cerebral dysfunction in two, ictal activity in one, and normal findings in one. Calcium and vitamin D supplementation was initiated in seven patients, and six patients had a favourable clinical outcome. Two patients died of causes not directly related to FS. Conclusion: Bilateral, symmetrical basal ganglia calcifications should prompt a systematic evaluation for an underlying parathyroid disorder. Correction of the associated metabolic abnormalities usually results in a favourable clinical outcome.


