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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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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
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author Dennis, Aa'Mani C
Nwabueze, Christian
Banu, Fahima
Nisenoff, Carolina D
Olupona, Tolu
author_facet Dennis, Aa'Mani C
Nwabueze, Christian
Banu, Fahima
Nisenoff, Carolina D
Olupona, Tolu
author_sort Dennis, Aa'Mani C
collection PubMed
description 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.
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spelling pubmed-99854072023-03-05 Bilateral Basal Ganglia Calcifications Manifesting as Psychosis With Manic Features: A Case Report on Fahr’s Syndrome Dennis, Aa'Mani C Nwabueze, Christian Banu, Fahima Nisenoff, Carolina D Olupona, Tolu Cureus Internal Medicine 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. Cureus 2023-02-02 /pmc/articles/PMC9985407/ /pubmed/36879722 http://dx.doi.org/10.7759/cureus.34547 Text en Copyright © 2023, Dennis et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Internal Medicine
Dennis, Aa'Mani C
Nwabueze, Christian
Banu, Fahima
Nisenoff, Carolina D
Olupona, Tolu
Bilateral Basal Ganglia Calcifications Manifesting as Psychosis With Manic Features: A Case Report on Fahr’s Syndrome
title Bilateral Basal Ganglia Calcifications Manifesting as Psychosis With Manic Features: A Case Report on Fahr’s Syndrome
title_full Bilateral Basal Ganglia Calcifications Manifesting as Psychosis With Manic Features: A Case Report on Fahr’s Syndrome
title_fullStr Bilateral Basal Ganglia Calcifications Manifesting as Psychosis With Manic Features: A Case Report on Fahr’s Syndrome
title_full_unstemmed Bilateral Basal Ganglia Calcifications Manifesting as Psychosis With Manic Features: A Case Report on Fahr’s Syndrome
title_short Bilateral Basal Ganglia Calcifications Manifesting as Psychosis With Manic Features: A Case Report on Fahr’s Syndrome
title_sort bilateral basal ganglia calcifications manifesting as psychosis with manic features: a case report on fahr’s syndrome
topic Internal Medicine
url 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
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