Basal Ganglia Calcification. Aetiopathogenesis, Diagnostics, Clinical Manifestations

Abstract

Fahr disease is a rare hereditary or sporadic neurological condition characterized by bilateral calcium deposition in the basal ganglia, dentate nuclei of cerebellum, and subcortical white matter. We can also distinguish Farh syndrome when its etiology is associated with the disorder of calcium metabolism, mitochondriopathies, cerebrum neoplasms, infections, inflammatory diseases of the nervous system, and injuries. The most common manifestations in patients with calcification of the basal ganglia of cerebrum are neurological and/or psychiatric disorders of varying severity. The clinical manifestation of the disease can occur at different ages, but mainly in young and middle-aged adults. However, some patients remain asymptomatic throughout their lives. The main clinical manifestations of the disease are extrapyramidal and movement disorders, emotional and cognitive impairments. At the same time, the correspondence of the form and severity of neurological conditions and the nature of calcification of the basal ganglia is rare. Currently, the treatment strategy for Fahr disease is based on symptomatic therapy and correction of etiological factors in Fahr syndrome. There is information about the reversibility of the calcification process and the complete restoration of mental functions in the early diagnosis and treatment of Fahr syndrome.