The results of my DW’s brain autopsy: Not at all the results I expected.

In a nutshell, if my DW is any gauge, our LO’s dementia is probably a lot more complex than we realize.

My DW was diagnosed in October 2020, at 69, with Semantic Dementia [SD], aka Semantic Variant of Primary Progressive Aphasia [svPPA], which in turn is a variant of FTD. This was not what the autopsy revealed. She passed away in February after nearly a dreadful year in Stage 7f. I chose to have an autopsy as they had requested when she was diagnosed with a relatively rare form of dementia.

Her original diagnosis was determined by a professor of neurology, and a neuropsychiatrist, both specializing in dementia at UCD, a large medical school, through extensive interview testing, an MRI, blood work, DNA testing, and a PET Scan. They then referred my DW to UCSF, a major national center for dementia research, where a neurologist specializing in rare dementias interviewed my DW, reviewed prior tests and confirmed the diagnosis.

The autopsy was performed and analyzed at UCSF Memory and Aging Center. The neurologist from UCSF covered the results with me over a long zoom call.

First off the neurologist stressed that any diagnosis on a living patient is still largely a clinical diagnosis, even with the advent of recent testing, meaning they are mostly assessing symptoms and drawing conclusions from that.

So what did my DW actually have? I still don’t really understand. I do know that the parts of the brain that were affected are the same as seen in Semantic Dementia, but it was caused by “High Alzheimer's Disease Neuropathologic Change (ADNC)”, in other words not SD/svPPA or any type of FTD. It was Alzheimer’s, yet it affected largely the same parts on the brain and produced the same clinical symptoms as SD.

Probably the most important finding was that my DW also had another type of dementia, a significant “Progressive supranuclear palsy (PSP), a subtype of frontotemporal lobar degeneration”. I had never heard of this, nor have I ever seen it mentioned on this forum. It is important because it apparently “causes progressive gait/balance impairment, ocular-motor dysfunction, parkinsonism and cognitive/behavioral changes. It is a clinicopathologic syndrome: definite diagnosis requires postmortem neuropathology”. The parts about gait/balance impairment explains my DW’s failing ability to walk in late Stage 6. The ocular-motor dysfunction explains the fact that she rarely opened her eyes in the last year of her life. How many other’s LOs have something like this and they don’t know it?

My DW also had “Lewy pathology, amygdala-predominant (LPC staging system).” This was apparently minor, but is yet another type of dementia that possibly affected her symptoms as well. On top of it all they also found “Cerebrovascular disease”. Could it all be any more cruel?

Hopefully this was informative and provided some insight into your own LO’s condition. I wish you all strength and peace in your incredibly difficult caregiving journey.