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Bilateral Basal Ganglia Calcifications Manifesting as Psychosis With Manic Features: A Case Report on Fahr's Syndrome
Aa'Mani C Dennis1, Christian Nwabueze1, Fahima Banu1
1Psychiatry, Interfaith Medical Center, Brooklyn, USA.
Insights
Fahr's syndrome, a rare neurodegenerative disorder, involves basal ganglia calcifications and can present with psychiatric symptoms. Early diagnosis is crucial as it can be elusive, impacting cognitive and behavioral health.
Area of Science:
- Neurology
- Neurodegenerative Disorders
- Radiology
Background:
- Fahr's syndrome is a rare neurodegenerative disorder characterized by bilateral basal ganglia calcifications.
- It is predominantly hereditary but can occur sporadically.
- The condition presents with diverse neurological and psychiatric symptoms.
Observation:
- A 50-year-old woman with no prior history presented with altered mental status progressing to psychosis over three years.
- Initial workup revealed elevated liver enzymes and a positive antinuclear antibody panel, but no electrolyte abnormalities or movement issues.
- Diagnosis evolved from unspecified psychosis to Fahr's syndrome, confirmed by neuroimaging.
Findings:
- Basal ganglia calcifications are a hallmark of Fahr's syndrome.
- Psychiatric symptoms, including psychosis, are common, affecting up to 40% of patients.
- Neuroimaging is essential for confirming the diagnosis.
Implications:
- This case highlights the importance of thorough workup for cognitive and behavioral disturbances in middle-aged and elderly patients.
- Fahr's syndrome can mimic other psychiatric conditions, making early diagnosis challenging.
- Complete evaluation and follow-up are vital for managing this elusive disorder.
Abstract:
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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