Vascular dementia with amyloid angiopathy

Diagnosis almost certain

Presentation

Widowed 65 year old woman presenting with a 3-4 year history of progressive decline in occupational function, idiosyncratic behaviors and coarsening of the personality following coiling of right MCA aneurysm. Associated LBBB, QTc prolongation (503ms) and TIAs of long standing.

Patient Data

Age: 65
Gender: Female

Mental State Examination

Slim 64 year old woman appearing stated age. Mildly dishevelled with long, untrimmed finger nails, partial dentures, no make-up or jewelry.

No psychomotor agitation or retardation. Blinking and grimacing motor tic.

Speech spontaneous, mildly increased in volume and at times difficult to interrupt.

Mood "normal" affect mildly expansive.

Conversation over-inclusive with mild circumstantiality. No delusions, no depressive themes.

No hallucinations or illusions.

Alert and well-oriented, mildly concrete in thinking.

Denies any psychiatric or neurological illness and dismissive of need for assessment and treatment.

Bedside cognitive testing

Diagram

Attention: 14/20

Visuocontructional: 19/20

Memory: 16/20

Executive: 12/20

LanguageL 16/20

Total: 77/100

Magnetic susceptibility centered on the lateral aspect of the right Sylvian fissure, attributed to the previous coiling, limits assessment of this region.

There are multiple FLAIR hyperintensities throughout the periventricular and subcortical white matter. The GRE sequence shows about six punctate foci of susceptibility, likely to represent previous micro-hemorrhages. Grey-white matter differentiation is preserved. No diffusion restriction is appreciated.

Otherwise age-appropriate cerebral volume loss appears a little more pronounced in the left temporal lober. No corresponding perfusion abnormality is appreciated.

The MRA shows a small right-sided ACommA infundibulum.aneurysm. A foetal left PCA is noted.

CONCLUSION: The combination of findings is best explained by amyloid angiopathy with associated advanced chronic small vessel ischemic changes. Small right-sided ACommA infundibulum/aneurysm warrants follow-up annually.

SPECT

Nuclear medicine

RADIOPHARMACEUTICAL: 99mTc HMPAO, 684 MBq

RESULTS: Mild hypoperfusion is seen bilaterally in the orbitofrontal cortices and in the mesial temporal lobes. Mild hypoperfusion is also seen in the left parietal lobe. Perfusion to the posterior cingulate gyri is preserved. No other significant abnormalities are seen.

Case Discussion

This case demonstrates mixed ischemic and hemorrhagic cerebrovascular disease in an adult woman with cardiac comorbidity. The referrer was concerned that the patient had acquired a frontal brain injury associated with the remote coiling of a previous right MCA aneurysm, but her cerebrovascular disease is probably sufficient to explain her dysexecutive syndrome as the clinical manifestation of a more insidious vascular dementia.

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