Frontotemporal lobar degeneration - logopaenic

Case contributed by Frank Gaillard
Diagnosis almost certain

Presentation

3-5 years of functional decline. Speech is empty at a conversational level, and she really struggles with direct confrontational testing.

Patient Data

Age: 65
Gender: Female

Again prominent cerebral volume loss is demonstrated particularly in the frontal and temporal lobes, particularly on the left. This is more pronounced than before, and is associated with ex vacuo dilatation of the ventricles and progressive widening of the left sylvian fissure. The hippocampi are bilaterally small, also on the left, particularly involving the hippocampal heads. No restricted diffusion, or cortical infarction. No intra or extra-axial collection/mass.

Conclusion:

Progressive volume loss in a pattern consistent with the diagnosis of logopaenic frontotemporal dementia.

Case Discussion

As is the case with most neurodegenerative conditions, a detailed history and a clinical differential diagnosis is often crucial in interpreting the findings. Although behavioral and language variants of FTLD do often have more pronounced frontal and temporal involvement respectively, this is not always the case.  

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