Fahr’s Disease Presenting as Primary Progressive Aphasia

Case Report

A 66 years-old-man presented with progressive word-finding difficulties and impulsive behavior. Examination showed nonfluent aphasia, anomia, impulse control disorder, executive dysfunction and ataxia. Neuropsychological testing was consistent with primary progressive aphasia. Labs were unremarkable, including parathyroid hormone, calcium, and phosphorus. CT head showed symmetric calcifications in basal ganglia, thalamus and cerebellum (Figure 1 A, B, C). Brain PET was normal. Fahr’s disease also known as an idiopathic basal ganglia calcification may have genetic, infectious, metabolic etiology and typically presents with movement disorders. Dysarthria is common, but aphasia has not been reported. 1,2 This unusual clinical presentation is likely secondary to the involvement of frontal projections [1,2].

Figure 1: A, B, C. Axial head CT without contrast shows extensive, but symmetric brain calcifications.

Study funding

No targeted funding reported.

Disclosures

The authors report no disclosures relevant to the manuscript.

Acknowledgements

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Conflict of Interests

None.

References

• Donzuso G, Mostile G, Nicoletti A, Zappia M (2019) Basal ganglia calcification (Fahr’s syndrome): related conditions and clinical features. Neurol Sci 40: 2251-2263.
• Saleem S, Aslam HM, Anwar M, Saleem M, Saleem A, et al. (2013) Fahr’s syndrome: literature review of current evidence. Orphanet J Rare Dis 8: 156.