Behavioral variant of fronto-temporal lobar degeneration

Case contributed by Dr Brad Hayhow

Presentation

60 year old self-funded retiree presenting with an 18 month history of disinhibited and "child-like" behavior associated with deteriorating self-care and increasingly concrete thinking.

Patient Data

Age: 60 years
Gender: Male

Mental State Examination

Casually dressed and neatly groomed gentleman playing air guitar to music from another room. Superficial, vague and occasionally inappropriate in engagement. Intermittent utilization behaviors. Speech diminished in prosody with occasional word finding difficulties and mild poverty of content. Mood euthymic, affect restricted. No formal thought disorder but confabulating speech content. No perceptual abnormalities. Alert and well oriented with poor insight into current mental state.

Bedside Cognitive testing - at presentation

Diagram

Attention: 18

Visuoconstructional: 19

Memory: 14

Executive: 11.5

Language: 16

Total: 77.5

This scan demonstrates severe atrophy of both the mesial temporal lobes as well as the temporal
lobes more generally with associated prominent compensatory temporal horn ventricular dilatation.
In addition there is quite pronounced albeit less marked atrophy of the frontal lobes, with  ommensurate thinning of the outer half of the body of the corpus callosum. Although the findings are mostly symmetric, there is perhaps marginally more pronounced atrophy on the left side. The parietal,
posterior temporal and occipitoparietal are relatively spared. Posterior fossa appears unremarkable.
Scattered supratentorial deep white matter T2 hyperintensities are consistent chronic small vessel disease. No regions of restricted diffusion or susceptibility artefact.

SPECT

Nuclear medicine

RADIOPHARMACEUTICAL: 99mTc HMPAO, 690 MBq

TECHNICAL PROCEDURE AND RESULTS: Tracer injection performed during conversation with the patient with the patient alert. Normalized against cerebellar perfusion, today's study shows severe bilateral temporal hypoperfusion, with moderate bilateral parietal hypoperfusion. Milder hypoperfusion also extends into the frontal lobes bilaterally. The posterior cingulate again shows loss of its normal prominence of activity.


OVERALL IMPRESSION: Findings are consistent  with both Alzheimer's disease and frontotemporal dementia. The severity of temporal involvement favors the latter.

Bedside cognitive testing - at follow-up 4 years later

Diagram

Attention: 6

Visuoconstruction:16

Memory: 4

Executive: 4.5

Language: 12

Total: 42.5

Case Discussion

This case represents a classical presentation of behavioral variant front-temporal lobar degeneration (bvFTLD) with early, pre-senile executive impairment associated with disproportional frontal and temporal lobe atrophy. There is subsequent progressive cognitive impairment with relative sparing of visuoconstructional capacity, which is helpful in differentiating the clinical diagnosis from Alzheimer disease.

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