Fahr’s syndrome: Clinical Case

Abstract

Fahr’s syndrome is a rare degenerative neurological disorder, with a prevalence of 1 in 1,000,000. The main characteristic of Fahr’s syndrome is the abnormal deposition of calcium in the brain, resulting in the formation of bilateral and symmetrical calcifications, primarily in the areas that control movement, such as the basal ganglia, the thalamus and the cerebellum.

The most common radiological feature of Fahr’s syndrome is the presence of bilateral and symmetrical intracranial calcifications that are generally confined to the globus pallidus, but may also affect the putamen, caudate nucleus, thalamus, dentate nucleus and the white matter of the cerebral hemispheres.

In this clinical case, the CT findings revealed a pattern of calcifications consistent with that described in the literature, both in terms of symmetry and anatomical distribution; and, as observed by other authors, there were no signs of oedema or mass effect, which reinforces the non-expansive nature of these lesions.

Fahr’s syndrome is a rare clinical entity characterised by abnormal calcium deposition in the brain, with a bilateral and symmetrical distribution predominantly in the basal ganglia and associated structures. The case presented illustrates the importance of computed tomography as the imaging method of choice for detecting these calcifications, enabling the identification of morphological patterns that are suggestive, and often diagnostic, of this condition.