Fahr syndrome

Fahr syndrome

A rare autosomal dominant movement disorder caused by the abnormal accumulation of calcium in the basal ganglia and the cerebral cortex.
Synonym: familial idiopathic basal ganglia calcification
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Fahr Syndrome is associated with various metabolic disorders, mainly parathyroid disorders.
Keywords: Fahr syndrome, Calcification, Intensive care.
1 In this presented case, we share our intensive care approach to a patient with Fahr Syndrome who was admitted with aspiration pneumonia and urosepsis.
The case was evaluated as Fahr Syndrome associated with hypoparathyroidism in the light of the laboratory, clinical and radiological findings (Table).
1 No individuals with similar neuropsychiatric symptoms or who had been diagnosed with Fahr Syndrome were encountered in the family history of our patient.
2 It has been reported that calcium accumulation in Fahr Syndrome starts approximately 3 decades before clinical symptoms appear.
Most Fahr syndrome cases progress symptomatically and they must be followed even when they are asymptomatic.
3 Together with the regulation of the calcium metabolism, various agents were tried for the treatment of Fahr syndrome, including Nimodipine which did not give satisfactory results.
In conclusion, although Fahr syndrome is not a newly identified disorder, it is often not brought to mind, or overlooked because it is so rare.
A confusional syndrome revealing a Fahr syndrome with hyperparathyroidism.
The Fahr syndrome and the chronic lymphocytic thyroiditis.
Differential diagnosis included Physiological intracranial calcification and Pathological basal ganglia calcification is due to various causes as hypoparathyroidism (Post-thyroidectomy), pseudohypoparathyroidism, secondary hyperparathyroidism (in renal disease), Fahr syndrome.