PET findings and neuropsychological deficits in a case of Fahr's disease

Psychiatry Res. 2001 Nov 30;108(2):133-40. doi: 10.1016/s0165-1781(01)00308-0.


In a case of Fahr's disease with frontal lobe type dementia and hyperkinetic-hypotone syndrome, functional changes were investigated using positron emission tomography (PET) with (18)F-fluorodeoxyglucose (FDG) as a tracer. Computed tomography showed bilateral calcifications in the putamen and globus pallidus consistent with the diagnosis of Fahr's disease and a frontally pronounced brain atrophy. In contrast, reduced glucose uptake in PET was not only confined to the areas mentioned above, but extended to the temporal and parietal cortices, bilaterally. These functional changes corresponded to the neuropsychological deficits observed, i.e. disturbed selective attention and cognitive flexibility, verbal perseverations, and declarative memory deficits. It is suggested that functional changes may precede cerebral atrophy in Fahr's disease and may reflect deficits in functional circuits, which involve both the basal ganglia and the frontal, parietal, and temporal lobes.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Atrophy
  • Basal Ganglia Diseases / diagnostic imaging*
  • Basal Ganglia Diseases / psychology
  • Brain Mapping
  • Calcinosis / diagnostic imaging*
  • Calcinosis / psychology
  • Cerebral Cortex / diagnostic imaging
  • Cerebral Cortex / pathology
  • Dementia / diagnostic imaging*
  • Dementia / psychology
  • Energy Metabolism / physiology*
  • Female
  • Humans
  • Neuropsychological Tests*
  • Syndrome
  • Tomography, Emission-Computed*