The Fahr syndrome and the chronic lymphocytic thyroiditis.

Rom J Morphol Embryol

Discipline of Anatomy and Embryology, Morphofunctional Department, "Grigore T. Popa" University of Medicine and Pharmacy, Iassy, Romania.

Published: May 2014

AI Article Synopsis

  • Fahr syndrome (FS) is characterized by basal ganglia calcification and is linked to various neurological and psychiatric issues, often emerging as a secondary condition.
  • A patient in Romania presented with seizures and mood disorders; CT scans revealed bilateral calcifications in key brain regions, leading to hospitalization and subsequent autopsy after the patient succumbed to complications.
  • Autopsy findings showed distinct calcium deposits in multiple brain areas, suggesting that the calcifications were caused by hypoparathyroidism due to a longstanding autoimmune parathyroiditis, supported by the presence of autoimmune thyroiditis in histological examinations.

Article Abstract

Fahr syndrome (FS) refers to basal ganglia calcification that is associated with many neurological and psychiatric abnormalities and appears as secondary to other diseases. We described a case of FS patient who was admitted in the Department of Neurology of "Prof. Dr. Nicolae Oblu" Clinical Emergency Hospital, Iassy, Romania, with seizure and mood disorders. On CT, the cause of seizure was found to be the bilateral calcifications of cerebellum, basal ganglia, thalamus and internal capsule. As the patient died after 15 days of hospitalization due to new seizures and gastrointestinal infection, an autopsy was made. Grossly, there were bilateral symmetrically gritty yellow areas in basal ganglia, thalami, internal capsule, cerebral cortex, cerebellar folia, dentate nucleus, and brain stem. A detailed histopathological examination revealed five types of calcium deposits within the walls of capillaries, small and medium-sized arteries from the intracerebral affected areas, chronic lymphocytic thyroiditis and fibro-adipose tissue instead of parathyroids. We consider that intracerebral symmetrical calcifications were the results of the hypoparathyroidism determined by an ancient autoimmune parathyroiditis that evolved to fibrosis as at microscopy we found an autoimmune thyroiditis.

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