Presentation
Three to four-year history of disinhibition, personality change and executive dysfunction. Increasing rigidity and obsessional thoughts and social isolation.
Patient Data
There is widening of the sulci of the frontal lobes bilaterally, out of keeping with the patient's age and the rest of the brain. This is associated with enlargement of the frontal horns of the lateral ventricles.
There is marked widening of the sulci of the frontal lobes bilaterally, out of keeping with the patient's age and the rest of the brain, even though the entire brain is somewhat volume depleted. This is associated with enlargement of the frontal horns of the lateral ventricles. The mesial temporal lobes are relatively spared. Features are suggestive of a frontal dominant frontotemporal dementia (e.g. behavioral variant).
SPECT studies of the brain were performed.
There is bilateral reduction in perfusion to the mesial frontal and mesial temporal lobes as well as insular cortex bilaterally. Perfusion to the posterior cingulate gyrus is preserved.
IMPRESSION: The findings are suggestive of a frontotemporal degenerative process
Limited sequences obtained. The participant terminated the study early. On review of the motion degraded T1-weighted volumetric sequence, there is evidence of a frontal predominant atrophy which also extends to involve the temporal lobes bilaterally. This has progressed since the study form one year prior.
Case Discussion
This patient clinically and on neuropsychological testing has features consistent with behavioral variant frontotemporal dementia. There is a strong family history of similar dementia, however, despite extensive testing, no definitive genetic cause has been identified.