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Bilateral Basal Ganglia Calcifications Manifesting as Psychosis With Manic Features: A Case Report on Fahr’s Syndrome

Fahr’s syndrome is a rare neurodegenerative disorder characterized by symmetric bilateral calcifications in the basal ganglia. While this is largely a hereditary disease with autosomal dominant inheritance, a small percentage is sporadic in nature with no metabolic or other underlying causes identif...

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Detalles Bibliográficos
Autores principales: Dennis, Aa'Mani C, Nwabueze, Christian, Banu, Fahima, Nisenoff, Carolina D, Olupona, Tolu
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9985407/
https://www.ncbi.nlm.nih.gov/pubmed/36879722
http://dx.doi.org/10.7759/cureus.34547
Descripción
Sumario:Fahr’s syndrome is a rare neurodegenerative disorder characterized by symmetric bilateral calcifications in the basal ganglia. While this is largely a hereditary disease with autosomal dominant inheritance, a small percentage is sporadic in nature with no metabolic or other underlying causes identified. Fahr’s syndrome has both neurological and psychiatric manifestations that include movement abnormalities, seizures, psychosis, and depression. Approximately 40% of patients with basal ganglia calcification present with psychiatric symptoms including mania, apathy, or psychosis. We present a case of a 50-year-old woman with no previous medical or psychiatric history who presented with an altered mental status that progressed to psychosis over three years. On one admission, the patient was found to have elevated liver enzymes and a positive antinuclear antibody panel but was without electrolyte abnormalities or movement disturbances. The patient was subsequently diagnosed with unspecified psychosis in the emergency department, which was later revised to Fahr’s syndrome confirmed by neuroimaging. This report discusses her presentation, clinical symptoms, and management of Fahr’s syndrome. Above all, it underscores the importance of complete workup and adequate follow-up of middle-aged and elderly patients with cognitive and behavioral disturbances, as Fahr's syndrome can be elusive in the early stages.