A CLINICAL CASE OF SECONDARY CALCIFICATION OF THE BASAL GANGLIA (FAHR’S SYNDROME)

Sylenko H. Ya., Delva M. Yu., Pinchuk V. A., Kryvchun A. M., Purdenko T. Y.

CLINICAL AND EXPERIMENTAL MEDICINE

Scentific article

Abstract. The article presents an example from the authors’ clinical observation of a case of secondary calcification of the basal ganglia (Fahr’s syndrome) – a disease associated with the deposition of calcium and iron salts in the walls of small arteries and arterioles, as well as in the brain. The paper discusses the clinical and diagnostic criteria of this pathology. The disease has an autosomal dominant type of inheritance, it is rare, and its prevalence is less than 1/1.000,000. About 200 cases of the disease have been described in the scientific literature. The etiology of this syndrome does not allow us to identify a specific agent, but associations with a number of conditions have been noted; the most common of these are endocrine disorders, mitochondrial myopathies, dermatological abnormalities, and infectious diseases. The main pathogenetic mechanism is a disruption of calcium-phosphorus metabolism; the main cause is considered to be primary (autoimmune) or postoperative endocrine adenomatosis of the thyroid or parathyroid glands. There are primary and secondary forms of calcification of the basal ganglia. It is assumed that the primary (idiopathic) calcification of the basal ganglia, or Fahr’s disease, is genetically determined and associated with damage to the 14 q chromosome. The causes of secondary calcification of the basal ganglia are numerous. Among them, the pathology of the thyroid gland occupies the first place. The diagnosis is based on the clinical presentation and data obtained by computed tomography and magnetic resonance imaging of the brain. The study found an advantage of CT of the brain, which is considered more sensitive than magnetic resonance imaging to detect calcified deposits in the basal ganglia. Computed tomography detects calcifications in various regions of the brain. Currently, there are no effective treatment methods for Fahr’s disease, therefore, symptomatic medications are applied. Treatment strategies are mainly focused on symptomatic relief and are strictly related to clinical features.n Presented data and research results may be of interest to neurologists, neurosurgeons, radiologists, and physicians of other specialties.

Fahr’s disease, calcification of the basal ganglia, hypoparathyroidism.

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«Bulletin of problems biology and medicine» Issue 3 (161), 2021 year, 137-142 pages, index UDK 616.834.1-003.84-07-022.214

10.29254/2077-4214-2021-3-161-137-142