Figure 2: Axial sections of SWI sequence of MRI brain showing susceptibility changes in bilateral caudate nucleus and globus pallidi (dotted circles, A), dentate nuclei (curved dotted arrows, B) and cerebellum (black, arrows, B)
Fahr’s syndrome usually follows secondary to underlying pathologies such as hypocalcemia, hypoparathyroidism, autoimmune conditions, infections, mitochondrial diseases, infections, toxic exposures, and can be associated with other conditions such as Cockayne syndrome, and Aicardi-Goutieres syndrome [9]. The primary form is a group of primary familial brain calcification (PFBC) and a heterogenic pathological variant has been identified in more than half of the cases. Moreover, studies have found the chance of inheritance to be as high as 50% from parents to the offsprings [9, 10].
Management should be primarily focused in the treatment of the underlying condition. Seizure, secondary to hypoparathyroidism could be the primary manifestation in patients with Fahr’s syndrome, yet clinical trials have shown antiepileptic therapies have not been beneficial in treatment in patients with such conditions [11]. These symptoms that are related to the Fahr’s syndrome, can be treated with vitamin D3 and steroids as well. Atypical antipsychotics are also proven beneficial [1]. Moreover, there are ongoing trials studying the effects of bisphosphonates on Fahr’s disease and syndrome [12].