Bilateral symmetrical calcifications of the basal ganglia and
cerebellar nuclei are denominated as Fahr’s syndrome
following the publication of Fahr in 1930. Thereafter, the
classical triads of this sydrome consisting of Bilateral
Striopallidodentate Calcinosis (BPSDC), Hypoparathyroidism
neurological manifestations are defined
10-12.
Basal ganglia calcifications can originate from more than
30 medical conditions including some infectious, metabolic
and genetic conditions (Table 1). Although most of these
conditions are systemic diseases, the reason of the focal
accumulation of calcium in basal ganglia is not known well
currently 13-16.
About half of the patients with basal ganglia
calcifications exhibit neurological manifestations and deficits.
The most common neurological signs are headache, vertigo,
movement disorders, syncope and seizures. Other specific
neurological deficits consist of paresis, spasticity, gait
disturbance, speech disorders, coma, dementia, Parkinsonism,
chorea, tremor, dystonia, myoclonia and orthostatic
hypotension 13,14.
Manyam et al. reported movement disorders in 56% and
seizures in 22% of cases in a review of 213 patients with this
disorder 13. Seizure disorder was prominent in our first case
whereas coordination system deficits in the second. Our first
patient referred to our clinics with medically intractable seizure
disorder. Furthermore his seizures increased in number despite
the constitution of the anticonvulsant therapy. There is no
evidence that anticonvulsant drugs may give rise to
calcifications but its coincidence with intracranial
calcifications is well known 3,11,15.
Forty percent of patients with basal ganglia calcifications
present with psychiatric symptoms at the beginning of the
disease. Among these; cognitive and psychotic disorders are
most prominent 7,9,14. Our first case also manifested severe
irritability lasting for a long time with the addition of amnesia at the latter terms of his disease. Therefore his mini mental
status examination revealed a low score (21/30).
According to the laboratory findings, first of our cases
was thought to be secondary to hypoparathyroidism and the
second as idiopathic Fahr’s syndrome. Calcifications,
originating from primary hypoparathyroidism are more diffuse
than other situations resulting from various etiologies such as
post-thyroidectomy hypoparathyroidism. Our first case that we
thought about primary hypoparathyroidism as the causative
etiological factor also showed diffuse parenchyma
calcifications in his CT scan of the brain.
Early treatment of hypoparathyroidism with
supplementation of Calcium and vitamin D can prevent
calcifications and probable neurophysiological disorders. Our case also demonstrated major benefits with supplementation of
calcium and alpha-calcidiol although he was in the late stage of
the disease. He exhibited no seizure during his follow-up
period and his laboratory findings were in normal limits. We
did not institute any treatment for our idiopathic Fahr’s
syndrome case and just kept him under clinical follow-up.
As a conclusion, we presented 2 cases of intracranial
calcifications resulting from hypoparathyroidism and
idiopathic, in this study. We want to emphasize that
hypoparathyroidism cases which show great clinical
improvement from medical treatment must be kept in mind as
a secondary causative factor of intracranial calcifications and
must be investigated with advanced laboratory tests.
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