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J Neurol Neurosurg Psychiatry 75:1163-1165 doi:10.1136/jnnp.2003.019547
  • Short report

Subacute dementia and imaging correlates in a case of Fahr’s disease

  1. T Benke1,
  2. E Karner1,
  3. K Seppi1,
  4. M Delazer1,
  5. J Marksteiner2,
  6. E Donnemiller3
  1. 1University Clinic of Neurology, Innsbruck, Austria
  2. 2University Clinics of Psychiatry and Nuclear Medicine, Innsbruck, Austria
  3. 3University Clinic of Nuclear Medicine, Innsbruck, Austria
  1. Correspondence to:
 Dr T Benke
 Klinik für Neurologie Innsbruck, Anichstr. 35, A-6002 Innsbruck, Austria; thomas.benkeuibk.ac.at
  • Received 26 May 2003
  • Accepted 16 September 2003
  • Revised 19 August 2003

Abstract

We report a case of idiopathic bilateral basal ganglia calcinosis, or Fahr’s disease (FD) in a 50 year old patient who developed rapidly progressive behavioural abnormalities and severe neuropsychological impairments, but no movement disorder. Neuropsychological deficits included a severe dysexecutive syndrome, anterograde amnesia, and attentional impairment. Neuropsychiatric features comprised apathy with intermittent disinhibition, anxiety, irritability, frequent mood changes, ritualistic and antisocial behaviour, and psychosis. Fluorodeoxyglucose positron emission tomography showed a massive reduction of glucose metabolism in the basal ganglia and the frontal brain. The observed abnormalities possibly result from a disruption of frontostriatal circuits, presumably at the basal ganglia level. This case indicates that FD may cause exclusively behavioural alterations and that the associated hypometabolism in certain frontal areas is closely related to the clinical picture.

Footnotes

  • Competing interests: none declared

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