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Background: Idiopathic hypoparathyroidism is a rare endocrine disorder caused by the deficiency of parathyroid hormone. It typically has a progressive course and is characterized by accumulations of calcium deposits in the basal ganglia bilaterally. In untreated patients, the intracranial calcification may also affect the thalamus, dentate nuclei, cerebral cortex, grey-white junctions and the cerebellum. Different locations can mimic multiple neurological diseases making the diagnosis of that rare disease a challenge. Purpose: To present a clinical case with untreated idiopathic hypoparathyroidism, extensive intracranial calcifications and neurological and ophthalmological complications. Material and methods: We present a 50-year-old man with untreated idiopathic hypoparathyroidism who was diagnosed in 2015 with massive intracranial calcifications located in the basal ganglia and outside the extrapyramidal structures. The neurological examination showed involuntary choreoathetotic movements of the right arm, progressive severe cognitive decline, generalized tonic-clonic seizures, gait imbalance and visual disorders. Abnormalities in the calcium-phosphorus metabolism and renal function tests were found. CT scans demonstrated extensive brain calcifications. The ophthalmological examination showed diminished visual acuity and mature cataract. The histopathological result did not demonstrate ragged red fibres. Some differential diagnostic opportunities like Fahr’s disease or Kearn Sayre’s syndrome were also considered. Results: The patient was diagnosed with untreated childhood idiopathic hypoparathyroidism with extensive intracranial calcifications and neurological and ophthalmological complications – a rare clinical case. This was confirmed by his medical history, general, neurological and ophthalmological examinations, laboratory, histopathological and neuroimaging investigations. Conclusion: The CT findings demonstrating extensive intracranial calcifications in the basal ganglia and the extrapyramidal structures make the presented clinical case a diagnostic challenge.

journal of imab

EXTENSIVE INTRACRANIAL CALCIFICATION WITH NEUROLOGICAL AND OPHTHALMOLOGICAL COMPLICATIONS IN A PATIENT WITH IDIOPATHIC HYPOPARATHYROIDISM: A CASE REPORT