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Posterior cortical atrophy

Diagnosis almost certain

Presentation

80 year old retired engineer presenting with an 18 month history of memory lapses associated with naming problems and handwriting dyspraxia. There were recent episodes of becoming lost in public. On neurological examination he demonstrated oculomotor dyspraxia.

Patient Data

Age: 80
Gender: Male

Mental State Examination

Neatly dressed and groomed elderly gentleman

Animated in conversation with spontaneous speech illustrated by vigorous gesturing

Intermittent word-finding difficulties

Mood "frustrated" and "depressed"

Affect generally reactive, but upset when unable to write name

No formal thought disorder, delusions or hallucinations

No overt impairment of insight or judgment

Bedside cognitive testing

Diagram

Attention: 11/20

Visuoconstructional: 16.5/20

Memory: 7/20

Executive: 10.5/20

Language: 12/20

Total: 47/100

mri

Diffuse cerebral atrophy is evident with a predilection of the parietal lobes and perisylvian regions.  There is mild atrophy of the mesial temporalstructures.  The differential diagnosis is between and the posterior variantof Alzheimer's disease and a frontotemporal dementia.Tiny focus of susceptibility artefact in the inferolateral right frontallobe may be secondary to amyloid angiography.  Minimal changes of small vessel chronic ischemic disease.

SPECT

Nuclear medicine

RADIOPHARMACEUTICAL:  99mTc Neurolite, 806 MBq

RESULTS: There is moderately decreased perfusion seen in the parasagittal areas bilaterally with reduction of tracer seen in the pre-cingulate gyrus. Mildly decreased tracer is seen homogeneously throughout both temporal lobes.  The occipital and frontal lobes appear normal.

OVERALL IMPRESSION: The findings are highly suggestive of Alzheimer's disease.

Case Discussion

This case is illustrative of the posterior variant of Alzheimer disease (posterior cortical atrophy) insofar as the patient presents with marked visual agnosia and oculomotor dyspraxia. As a previously fluent speaker of three languages, the patient also gave a striking history of losing words in his more recent languages first, and attached the best preservation of language function to his mother tongue.

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