Frontotemporal dementia - behavioral variant

Case contributed by Frank Gaillard
Diagnosis almost certain

Presentation

Three to four-year history of disinhibition, personality change and executive dysfunction. Increasing rigidity and obsessional thoughts and social isolation.

Patient Data

Age: 35 years
Gender: Female
This study is a stack
Axial
non-contrast
This study is a stack
Sagittal
non-contrast
This study is a stack
Coronal
non-contrast
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Info

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. 

This study is a stack
Sagittal
T1
This study is a stack
Axial
T1
This study is a stack
Coronal
T1 IR
This study is a stack
Coronal
T2
This study is a stack
Axial
FLAIR
This study is a stack
This study is a stack
Axial
T2
This study is a stack
Axial
ADC
This study is a stack
Coronal
Gradient Echo
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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). 

Nuclear medicine
Neurostat
Sagittal
Axial
Coronal
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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

1 year later

mri
This study is a stack
Sagittal
T1
This study is a stack
Coronal
T1
This study is a stack
Axial
T1
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Info

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. 

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