Frontotemporal dementia - behavioral variant

Case contributed by Frank Gaillard
Diagnosis almost certain

Presentation

Two-year history of altered behavior. Disinhibition.

Patient Data

Age: 70 years
Gender: Male

There is profound bilateral frontal volume loss more pronounced on the left with less marked reduction in left temporal lobe volume. Prominent ex vacuo dilatation of the lateral ventricles particularly anteriorly is noted. The parietal lobes and the hippocampi are relatively unremarkable without substantial volume loss. The posterior fossa is spared. No evidence of micro hemorrhages. No abnormal diffusion restriction.

Conclusion: Marked bifrontal volume loss, with less pronounced temporal lobe volume reduction, is asymmetric and more pronounced on the left. This pattern is not usually encountered in Alzheimer's disease, but rather favors frontotemporal dementia, most likely behavioral variant. 

Case Discussion

This patient was assessed by the neuropsychiatry unit and underwent neuropsychological testing. The features were consistent with a behavioral variant of frontotemporal dementia. The patient died two years later.

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