Fronto-temporal lobar degeneration - agrammatic variant primary progressive aphasia
Presentation
Patient with progressive difficulties of speaking, making errors in speech sounds and struggling to understand sentences.
Patient Data
MRI Brain
Significant encephalomalacia and volume loss is identified involving the left hemisphere. The atrophy affects the frontal lobe and temporal lobe more than the parietal and occipital lobe, however, all lobes are involved.
At the inferior aspect of the left temporal lobe there is FLAIR hyperintensity in keeping with gliosis.
The hippocampal formation on the left is significantly atrophied but not to any further degree than the surrounding temporal lobe changes. The left sylvian fissure is widened.
SPECT
RADIOPHARMACEUTICAL: 99mTc CERETEC 740 MBq
Case Discussion
This case illustrates a patient with frontotemporal lobar degeneration, as spotted on the MRI and SPECT images, with a clear progressive loss of specific language functions: dysfluent speech, with frequent hesitations, and errors in the production of speech sounds; however, relatively preserving the other cognitive domains. The neuropsychiatry team defined this case as agrammatic variant primary progressive aphasia.