Idiopathic striatopallidodentate calcification. Description of the clinical case

Abstract

Idiopathic striatopallidodentate calcification (Fahr's disease/syndrome, idiopathic non-atherosclerotic calcification of intracerebral vessels, idiopathic calcification of the walls of cerebral vessels with progressive dementia) is a chronic neurodegenerative disease with an exchange component belonging to the orphan group. This nosology is based on the deposition of calcium and iron salts in the walls of small cerebral arteries, as well as directly in the structures of the central nervous system: in the cerebral hemispheres, basal ganglia, and cerebellum. Given the multiple lesions of various parts of the brain, the clinical picture of this disease is polymorphic and manifests itself with various symptoms (asthenia, coordination and speech disorders, extrapyramidal syndrome), which, together with a rare occurrence, leads to significant difficulties in diagnosis and, as a result, to a later onset. treatment. This article presents literature data and describes a clinical case of Fahr's disease in a young patient. The debut of the disease was accompanied by focal cervical dystonia with further gradual involvement of the axial muscles and muscles of the upper extremities over the next few years.