Early-onset Alzheimer disease - posterior cortical atrophy

Case contributed by Dr Gabrielle Matta

Presentation

Four year history of increasing cognitive and functional decline, with prominent difficulties in speech, dressing himself, memory and activities of daily living.

Patient Data

Age: 55-year-old
Gender: Male
MRI

MRI Brain non contrast (Encephalopathy protocol)

No focal signal abnormality identified. Significant bilateral parietal lobe volume loss, slightly more prominent on the left, and correlated with local reduced perfusion in those areas. Mesial temporal lobes appear normal. No evidence of restricted diffusion.

No extra-axial collections or mass are evident.

Conclusion:  The presence of bilateral parietal lobe volume loss in the this age group and clinical setting favours Alzheimer's disease as the most likely diagnosis.

Nuclear medicine

Cerebral Perfusion Study

These studies show moderate hypoperfusion of the prefrontal lobes. There is also a small focal zone of severe hypoperfusion located at the left superior parietal lobe. Left cerebral hemisphere perfusion is generally mildly reduced compared to right possibly due to diaschisis from this zone of focal intense hypoperfusion. There is also an unexpected increase in perfusion seen at the left anterior temporal pole mesial cortex. Perfusion of the brainstem and cerebellum and basal ganglia is normal.
 

Case Discussion

The most striking feature of the initial clinical presentation was expressive dysphasia. He also initially developed visuoconstructional difficulties, later becoming increasingly ataxic.

The imaging is a good demonstration of parietal volume loss. The intraparietal sulci are widened. There is pronounced volume loss of the superior parietal lobules. The medial temporal lobes are of normal appearance. There are no significant chronic small vessel ischaemic changes.

The features, both clinical and on imaging, are characteristic of early-onset Alzheimer disease / posterior cortical atrophy.

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Case information

rID: 43384
Case created: 7th Mar 2016
Last edited: 1st Mar 2017
Inclusion in quiz mode: Included

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