Endocrine Abstracts

Introduction: Fahr syndrome is a rare disease, characterized by bilateral and symmetrical intracerebral calcifications, localized in the central gray nucleus and by the classic association with hypoparathyroidism. We report a case of Fahr syndrome associated with postoperative hypoparathyroidism.

Case report: A 58 year old female patient with a history of total thyroidectomy 18 years ago. She complained of chronic paresthesia, muscle cramps complicated sometimes by attacks of tetany mistaken for hysterical crisis. The evolution was marked last year by the installation of a state of generalized convulsive crisis. The assessment carried out in the emergency department objectified a deep hypocalcemia at 70 mg/l, a hyperphosphoremia, associated with a low serum level of PTH on radioimmunoassay (PTH = 0.001 pg/ml). On the electrocardiogram the QT was lengthened. The cerebral scanner had shown bilateral and symmetrical calcifications of the central gray nucleus of the upper and sub tentorial stages. The diagnosis of Fahr syndrome secondary to hypoparathyroidism was accepted and the patient was substituted with calcium and vitamin D.

Discussion: Fahr syndrome can complicate untreated hypoparathyroidism at any age. The pathophysiological mechanisms of intracerebral calcifications are poorly understood. In front of phosphocalcic metabolism disorders, and specially in case of associated neuropsychiatric signs, intracerebral calcinosis should be sought. Treatment is based on the correction of disorders of phosphocalcic metabolism which often leads to a marked improvement. This observation illustrates the diagnostic difficulty with the clinical heterogeneity of this pathology and the advantage of systematic research of postoperative hypoparathyroidism in order to avoid its deleterious complications.

Conclusion: Fahr syndrome is a rare entity with contrast between a rich and severe symptomatology and a simple and effective treatment. The etiologies are dominated mainly by hypoparathyroidism.