Presentation
Progressive language deficit
Patient Data
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.
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:
- agrammatic variant primary progressive aphasia (also known as progressive nonfluent aphasia)
- semantic variant primary progressive aphasia (also known as semantic dementia)
- logopaenic variant primary progressive aphasia
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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