Quantitative MR Markers and Psychiatric Symptoms in a Patient with Fahr Disease

Am J Case Rep. 2015 Jun 21:16:382-5. doi: 10.12659/AJCR.893163.

Abstract

Background: Fahr's disease (FD), or primitive idiopathic calcification of the basal ganglia, is a rare neurodegenerative syndrome characterized by the presence of idiopathic bilateral and symmetrical cerebral calcifications.

Case report: We describe the case of 43-year-old woman presenting with psychiatric symptoms, disorganized behavior, and migraine. Magnetic resonance imaging (MRI) examination showed basal ganglia calcifications. In addition, we analyzed the cortical brain volume and noted cortical atrophy. Extensive etiological clinico-biological assessment allowed us to exclude known causes of brain calcifications and to diagnose Fahr disease (FD). Neurological symptoms associated with psychiatric manifestations are not uncommon in FD.

Conclusions: Purely psychiatric presentations are possible, as demonstrated by the present case, although there have been very few cases reported. To date, no studies related to the brain atrophy in FD have been reported.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Basal Ganglia / pathology*
  • Basal Ganglia Diseases / complications
  • Basal Ganglia Diseases / diagnosis*
  • Calcinosis / complications
  • Calcinosis / diagnosis*
  • Cognition / physiology*
  • Female
  • Humans
  • Magnetic Resonance Imaging / methods*
  • Mental Disorders / complications*
  • Mental Disorders / diagnosis
  • Mental Disorders / psychology
  • Neurodegenerative Diseases / complications
  • Neurodegenerative Diseases / diagnosis*
  • Tomography, X-Ray Computed

Supplementary concepts

  • Fahr's disease