Mrs MT lives with her partner. She was referred by her GP to assess declining cognitive and visuospatial skills. She has a past history of depression which responded to antidepressants and counselling.
Over 4 years she had deteriorating memory- she could purchase ingredients for a meal then ask what is planned for dinner, often left taps or the stove on and was frequently repeating questions or items of information. She was putting objects in unusual places- her partner said the pantry had become “a mystery to (her)”. She had become uncharacteristically unreliable in recalling phone numbers.
Planning and visuospatial skills were also deteriorating- when her car starting rolling in a car park she couldn’t work out how to stop it, resulting in a nasty outcome for an expensive car downhill from hers. She was now unable to reverse park and on one occasion drifted from her lane, causing another collision. She was unable to find the exit when visiting gardens and on a recent trip couldn’t visualize the itinerary- eg where she was going next or where she was in the country. She was now struggling with sudokus, having previously been quite proficient.
Her daily activities had deteriorated- she was unable to recall a recipe so her cooking had become much simpler, she was no longer able to bank via the internet and was unable now to operate the DVD player or the sewing machine. She was previously a keen surfer but had lost those skills completely.
She was taking no medications and was a non-smoker and drank almost no alcohol.
Her father and two of her brothers died from alcohol abuse and there was a strong family history of alcohol-related problems in the others, including her mother and all the other brothers. There was no family history of dementia.
She is a retired physiotherapist and her first marriage of 8 years dissolved- there are 2 children from that.
Physical examination was unremarkable.
Cognitive assessment revealed an MMSE of 26. She was completely unable to copy intersecting pentagons, but her short term memory was 3/3. On the Montreal Cognitive Assessment she scored 22/30. She was unable to copy the cube but her clock was satisfactory. Short term memory was 2/5 but the missing words were recalled when cued.
The CT scan from the referring doctor was unremarkable, as were blood tests.
Posterior cortical atrophy, a form of Alzheimer’s disease characterised by prominent visuospatial dysfunction as well as amnestic features, was the initial diagnosis. Additional neuroimaging was arranged. The MRI scan showed biparietal atrophy with preservation of temporal, including hippocampal, volumes. The SPECT scan showed reduced right temporoparietal- occipital hypoperfusion only. FDG-PET scanning revealed hypometabolism in parietal and lateral temporal cortices, much worse on the right with occipital and primary sensorimotor cortices spared. These regions, the right precuneus and right posterior cingulate gyrus were hypometabolic when compared to pooled normal brains using the Neurostat 3D- SSP software. She subsequently had an amyloid scan (PiB PET) which was strongly supportive of Alzheimer’s disease with extensive amyloid deposition in cortical and striatal areas.
The neuroimaging confirmed the clinical diagnosis of posterior cortical atrophy as a presentation of Alzheimer’s dementia. The diagnosis was relayed, and contact with Alzheimers Australia Victoria (AAVic) recommended. The partner and the patient have subsequently become very involved with AAVic.
She was commenced on donepezil, her MMSE at that stage being 25. Three months later her MMSE was 28, and she was now able to copy the intersecting pentagons.
Souvenaid was commenced 3 months later. The carer noticed further improvement in visuospatial skills- she was more able to correctly position herself when sitting, and her memory- eg for phone numbers- had improved. Subsequently she developed gastrointestinal symptoms and her carer attributed these, possibly, to the Souvenaid. The daily consumption was reduced to second daily, with no improvement in gastrointestinal symptoms but a marked deterioration in visuospatial skills. She was again missing chairs and bumping into doorways. The Souvenaid was returned to once daily and the gastrointestinal symptoms resolved, as well as a return to her improved visuospatial function.
She remains on Souvenaid and donepezil, some 24 months after the initial diagnosis and 6 years from symptom onset.
A/Professor Michael Woodward,