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].