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Case Reports
. 2021 Apr-Jun;18(4-6):11-14.

A Rare Case of Fahr's Syndrome in a Patient with Secondary Hypoparathyroidism and Neuropsychiatric Symptoms

Eirini Beneki  1   2   3 , Xenofon Papazarkadas  1   2   3 , Achillefs Karras  1   2   3 , Konstantinos Kalantzis  1   2   3 , Konstantinos Tsatiris  1   2   3
Affiliations
Case Reports

A Rare Case of Fahr's Syndrome in a Patient with Secondary Hypoparathyroidism and Neuropsychiatric Symptoms

Eirini Beneki et al. Innov Clin Neurosci. 2021 Apr-Jun.

Abstract

Background: Fahr's syndrome, also known as basal ganglia calcification, is a rare neurodegenerative disorder characterized by radiological findings of symmetrical and bilateral idiopathic abnormal deposits of calcium in areas of the brain that control motor activity, including the basal ganglia and the cerebral cortex. There is neither a specific cure, nor a standard treatment for Fahr's syndrome and treatment is primarily symptomatic. Brain imaging has gained widespread use in order to support clinicians in diagnosing intracranial calcifications.

Case presentation: We present a case of an 83-year-old female patient who presented with symptoms of confusion, fever, nausea, and vomiting. Clinical diagnosis of Fahr's syndrome secondary to hypopathyroidism was based on the neuropsychiatric signs and symptoms, laboratory evidence of hypoparathyroidism, and radiological signs of calcifications in the basal ganglia. The patient improved following replacement therapy with calcium gluconate, followed by oral supplemental calcitriol.

Conclusion: This case highlights the importance of considering organic causes when patients present with neuropsychiatric disturbances, especially following thyroidectomy.

Keywords: Fahr’s syndrome; basal ganglia calcification; bilateral intracranial calcification; hypoparathyroidism.

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Conflict of interest statement

DISCLOSURES: The authors report no conflicts of interest relevant to the content of this article.

Figures

FIGURE 1.
FIGURE 1.
Bilateral symmetric and extensive calcifications in the basal ganglia
FIGURE 2.
FIGURE 2.
Bilateral symmetric and extensive calcifications in the cerebellar hemispheres
FIGURE 3.
FIGURE 3.
Differential diagnostic process. CNS; central nervous system, CT; computed tomography, PTH; parathormone, PMR; polymyalgia rheumatica
See this image and copyright information in PMC

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