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.

Cureus
|March 7, 2023
PubMed

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.

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