Frontotemporal dementia - language variant

Case contributed by Assoc Prof Frank Gaillard


Progressive language deficit

Patient Data

Age: 50 years
Gender: Female

Striking volume loss is present in the left temporal lobe, particularly anteriorly with 'knife-like' gyri. Minor left frontal and parietal volume loss also noted. No significant right sided volume loss. Only minor chronic small vessel ischemic change. 


Nuclear medicine

SPECT studies (99mTc HMPAO : 795 MBq) of the brain were performed following technetium HMPAO infusion with mental stimulation.

The studies show moderate diffuse hypoperfusion of the left superior temporal cortex extending form the anterior pole of the posterior temporal region is also mild left superior frontal and parietal cortical hypoperfusion. Perfusion of the right cerebral cortex appears normal. In the cerebellar and is mildly reduced right cerebellar cortical perfusion consistent with diaschisis from the left cerebral cortex.

Basal ganglia are in brainstem perfusion is normal.


Case Discussion

The presentation and imaging is consistent with a language variant of frontotemporal degeneration (primary progressive aphasia) which is further divided into:

Although there is considerable overlap in presentation and imaging, this case is most consistent with semantic dementia, with prominent anterior temporal lobe involvement. 

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