Endocrine Abstracts

Introduction

Fahr’s syndrome (FS) is a rare neurodegenerative disorder. It’s characterized by abnormal calcifications in basal ganglia and cerebral cortex inducing neuropsychiatric disorders and cognitive impairment. The aim of our study was to assess the prevalence of FS, its characteristics, and its associated factors in patients with permanent hypoparathyroidism.

Methods

We conducted a cross-sectional study including 38 patients with chronic hypoparathyroidism. A brain computed tomography scan was performed to all participants. Clinical and biological data were collected. The adherence to treatment was evaluated using Girerd adherence scale.

Results

There were 30 (82%) women and 8 (20%) men with a mean age of 53 ± 15.9 years. Neck surgery was the most frequent etiology of chronic hypoparathyroidism (66%). FS was diagnosed in 40% of cases (n = 15) with a sex-ratio of 2.75. Clinical manifestations included amnestic disorders, depressive mood, psychotic symptoms, tetany crisis and seizures in 80, 47, 27, 27, and 20% of cases, respectively. Brain CT-scan showed bilateral intracerebral calcifications located in the central gray nuclei (67%) or diffuse symmetric calcifications (33%). The prevalence of FS increased with the duration of hypoparathyroidism (P = 0.048) and decreased with the patient age at the diagnosis (P = 0.028). Amnestic disorders (Odds Ratio = 5.143, P = 0.043), poor adherence to treatment (Odds Ratio = 6, P < 10^–3), and hypomagnesemia (Odds Ratio = 98, P < 10^–3) were positively associated with FS. However, calcium level, PTH level, and coexisting vitamin D deficiency were not associated with FS.

Conclusion

Patients with chronic hypoparathyroidism are most likely to develop intracranial calcifications because of phosphocalcic metabolism disorders and blood-brain barrier alteration, leading to various neuropsychiatric manifestations. This study highlights the importance of adequate treatment and medication adherence to prevent Fahr’s syndrome.