Abstract
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.
Highlights
Background and importanceFahr’s syndrome is known as idiopathic calcification of the basal ganglia
Clinical presentation: We present the case of a post thyroidectomy patient with hypoparathyroidism (HPT) with incidentally detected basal ganglia calcification (BGC)
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
Summary
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. The clinical examination and laboratory values and subsequent radiological investigations drove us to a diagnosis of Fahr’s syndrome secondary to endocrinological dysfunction due to post-thyroidectomy HPT She was managed conservatively in the ICU with intravenous calcium gluconate, and ampicilin/sulbactam for respiratory infection and started on supportive oxygen therapy via 60% venturi. An immediate plain CT brain was ordered which showed diffuse subarachnoid haemorrhage, especially in the supra-chiasmatic cistern and was highly suggestive of an aneurysmal bleed Another peculiarity noted in the scan was the presence of significant calcification in the basal ganglia region (Figure 3). He was evaluated for the same and his serum calcium and PTH levels were well below normal values. The patient had a sudden deterioration in his glascow coma scale (GCS), the following day, and was discharged against medical advice and discharged from the hospital
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