Annals of Neurology | 2021
Hypoparathyroidism Associated with Vascular Centrum Semiovale Calcifications
Abstract
We present an 86-year-old woman with a progressive cognitive deficit. The patient had a recent history of idiopathic hypoparathyroidism (parathyroid hormone level < 1ng/l, normal range = 15–88), discovered following decreased calcium blood levels (1.45mmol/l, normal range = 2.2–2.55). Computed tomography (Fig, axial [A] and coronal [B] 10mm-thick multiplanar reconstruction images), performed because of persistent cognitive deficit despite correction of hypocalcemia, showed bilateral (peri)vascular centrum semiovale calcifications, and dentate nuclei calcifications (Fig, C, arrowheads) in the absence of basal ganglia involvement (Fig, D) on standard axial views. Centrum semiovale perivascular spaces were not dilated on magnetic resonance imaging. We attributed the (peri)vascular spaces and dentate nuclei calcifications to the associated hypoparathyroidism (often referred to as Fahr syndrome or disease). Delacour described, almost a century before Fahr’s original report, an 81-year-old demented patient with bilateral cerebral calcifications most prominent in the striatum at postmortem, corresponding to what would now probably be called striopallidal or striopallidodentate calcifications (often further categorized as idiopathic vs symptomatic, and sporadic vs familial). This latter condition is often erroneously called Fahr syndrome or disease. Actually, the original report of Theodor Fahr in 1930 described a postmortem analysis of a patient (possibly suffering from hypoparathyroidism) with diffuse idiopathic arterial and venous calcification predominant in the white matter (and nearly absent in the basal ganglia), as in our patient. Basal ganglia calcification, and not white matter calcification, has been reported to be associated with hypoparathyroidism. Fahr’s original report and our patient probably represent rare cases of hypoparathyroidism with