Scintigraphic, neuroradiological and clinical comparison in two patients with primary sporadic and two with secondary Fahr’s disease

Abstract

Bilateral striopallidodentate calcification, usually termed Fahr's disease, can give rise to various clinical manifestations including hyperkinetic movement disorders or a hypokinetic Parkinsonian syndrome, behavioural and mood changes, cognitive deficits and even frank dementia. We describe four patients all of whom underwent a detailed scintigraphic, neuroradiological and clinical work-up: two had primary, sporadic Fahr's disease and two had Fahr's disease secondary to hypoparathyroidism. The neuroradiological and clinical studies disclosed similar anatomical and pathological changes in the four patients but variable and sometimes unexpected clinical manifestations. Both patients with primary forms had hypokinetic Parkinsonian syndrome, both patients with secondary forms had hyperkinetic movements. Dopamine autotransporter scan brain scintigraphy disclosed an unexpected unilateral putamen involvement despite substantially symmetric calcifications.

A Case of Idiopathic Basal Ganglia Calcification Associated with Membranoproliferative Glomerulonephritis

“…Patients with IBGC often show slow progression from pyramidal symptoms to extrapyramidal involvement, cerebellar ataxia, and cognitive impairment (3,7). Here we report a case of IBGC complicated by nephrotic syndrome that required dialysis.…”

I123‐FP‐CIT (DaTSCAN) SPECT beyond the Most Common Causes of Parkinsonism: A Systematic Review

“…Conversely, Kalampokini et al presented a case of Fahr disease due to hypoparathyroidism in which the patient presented parkinsonism with severe rigidity in the limbs, bilateral calcifications in the brain confirmed by both CT and MRI examination, and a normal DaTSCAN result 48 . However, a previous report of Fahr disease due to hypoparathyroidism had previously shown asymmetrical radiotracer uptake in the putamen, although the presence of calcifications was symmetric 49 . These contradictory results point to currently unknown factors, besides the presence of calcifications, affecting the dopaminergic activity in this rare disorder.…”

“…48 However, a previous report of Fahr disease due to hypoparathyroidism had previously shown asymmetrical radiotracer uptake in the putamen, although the presence of calcifications was symmetric. 49 These contradictory results point to currently unknown factors, besides the presence of calcifications, affecting the dopaminergic activity in this rare disorder.…”