Neurological Disorders

Bilateral Basal Ganglia Calcification Secondary to FAHRs Syndrome: A Rare Entity

Abstract

Anmol Nagaraj, Rajesh Parameshwaran Nair, Sibhi Ganapathy, Lakshman IK

Background and importance: Fahr’s syndrome is also known as idiopathic calcification of the basal ganglia. It is described as a rare, degenerative, neuropsychiatric disorder characterized by seizures, extrapyramidal, and neuropsychiatric symptoms as a result of symmetric and bilateral calcifications within the basal ganglia. Involvement of the nucleus pallidus, the putamen, the dentate nucleus of the cerebellum, and the hemispheric white matter at the base of the skull, are common radiological hallmarks of this syndrome. The calcification probably occurs due to lipid deposition and demyelinization.
Clinical presentation: We present the case of a post thyroidectomy patient with hypoparathyroidism (HPT) with incidentally detected basal ganglia calcification (BGC). Retrospectively the patient was found to have hypocalcemia, secondary to a total thyroidectomy performed on her, 2 years ago. The second case was that of a 35 year old patient who presented with sudden onset of “worst headache of his life” since the previous night associated with multiple episodes of vomiting. Here we discuss our clinical dilemma and treatment strategy.
Conclusion: In presence of BGC, HPT should be investigated, especially in patients who have undergone thyroidectomy, since in the early stage, the recovery could be expedited with a precise diagnosis and prompt treatment. This case report illustrated the benefits of calcium supplementation and calcitriol, even with the patient being in advanced stage of disease.