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Frontotemporal dementia

Case contributed by Frank Gaillard
Diagnosis almost certain

Presentation

12-month rapid progression of inappropriate behavior, hyperorality, stereotyped behavior, executive impairment, apathy, lack of empathy, poor personal care and reduced verbal output, with UMNL (hyperreflexia, clonus), LMNL (muscle atrophy, reduced gag reflex) and positive glabellar tap.

Patient Data

Age: 45 years
mri

Prominent bilateral frontal lobe atrophy with preservation of the rest of the brain. No microhemorrhages or abnormal diffusion. 

Nuclear medicine

RADIOPHARMACEUTICAL: 99mTc ECD, 512 MBq

TECHNICAL PROCEDURE AND RESULTS SPECT

Studies of the brain were performed. There is severe hypoperfusion of the frontal lobes with mild-to-moderate hypoperfusion of the temporal lobes and mild parietal hypoperfusion. Perfusion is preserved to the cerebellum, primary visual cortex and basal ganglia. No other significant abnormalities are seen.

CONCLUSION: The findings are consistent with frontotemporal dementia.

Case Discussion

Both MRI and SPECT support the clinical diagnosis of a behavioral variant of frontotemporal dementia. The diagnosis is further consolidated by EMG which support the possibility of FTD-associated motor neuron disease.

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