FTLD, or not?

RE: report for David Rilsmith tested on 01/14/21
Some suggestions on this almost 64-year old fellow with mental state change over the last two years who the clinician felt had Mild NCD due to Frontotemporal Lobar Dementia (FTLD). However, analysis suggests we don’t have enough to diagnose Mild NCD, and the reasons for and against this diagnostic impression are reviewed below:

The case for FTLD:
In favor of consideration of FTLD “might” be the young age of onset (around 65 in this patient). However, FTLD sadly develops more commonly in people in their 50’s. Sixty percent of FTLD patients develop it between ages 45–64. There were significant deficits in mental control; i.e., complete inability to count backwards by sevens or spell “world” backwards which could imply sequencing difficulties consistent with the attention set problems commons to FTLD. This is seen in behavior as problems in organizing, multitasking, performing multi-step activities, prioritizing tasks (doing more important things first), and very poor decision-making; but, none of this was much apparent in this patient’s carefully taken history. A hallmark feature is “disinhibition” or popping out with inappropriate (often sexualized) comments to strangers. Again, nothing like this was evident. There
was a severe weakness in Verbal Fluency with only 9 animals, 4 fruits and just 2 vegetables named in a minute each – problems in verbal fluency are common to FTLD, but so are they to most other NCD causes. So there just isn’t much here to argue FTLD, which seems (see below) mostly offered as a diagnosis to recognize the neurologist referents’ MRI report.

The case against (or at least not very supportive of) FTLD:
It was reported that "A note from his neurologist’s 8/5/2020 appointment indicates that a July 2020 MRI showed possible atrophic changes in the medial temporal regions.” This supports some deterioration in the middle area on both sides of the brain, but nothing was found problematic about the
frontal lobe being atrophied.

In FTLD we might expect to see problems in the ability to shift attention set between competing stimuli (think problems on:
Trail Making or the Categories Test; Luria Hand Patterns, maintaining consistent flow in orthography on the Purdue or Kephart Repeated Patterns measures) which, of course, would require more neuropsychological testing then the NCDeval uses. Nothing in history really suggests signs of this, except this retired electrical engineer who designed and built an addition on to his house a few years ago, now struggled to assemble a simple shed – which seems more consistent with general ability decline, or DAT.

A hallmark feature of FTLD patients is reduced judgment and reasoning capacity. This concept is captured in the excellent recommendation:

…there is evidence that Mild NCD may be a bigger risk factor for susceptibility to scams or negative influence. He should be monitored as to spending patterns, gifting, etc. that either vary from his prior behavior or exceed his budget for signs of elder abuse.

And, he could not set time on a clock; however, this patient did “fairly” well on solving proverbs and determining similarities among concepts (e.g., answering “How are shirt, pants and socks alike?”) which does not support FTLD. He could not solve a simple verbally presented math problem about time, which is as indicative of an Alzheimer’s etiology as it is of FTLD. And, the above recommendation serves well for any NCD patient.

"Although he has a bachelor’s degree, his AMNART results suggested Low Average intellectual functioning.” This good observation suggests an new-onset (or acquired) dyslexia, which does certainly support an NCD. While this could be related to FTLD, it is just as strong evidence of emerging DAT.

We might expect a dulling of interpersonal affect (this has referred to a “bump on the log” behavior in FTLD, or simple having no interest in anything around him), yet he showed humor as reported in Observations, which argues against the flat interpersonal affect common to FTLD.

I note the summarization contains "Given the neurologist notation of temporal lobe involvement, a frontal-temporal dementia must be considered as underlying his cognitive decline.” But, remember that the temporal lobes are maximally involved in memory storage (they surround and hug the hippocampus where initial memories are formed and then stored in the the temporal regions) and thus are implicated in Alzheimer’s disease. The medial aspects (which were mentioned) of the temporal lobe play a role in language and emotions. Information stored there (memories of a sort) allow word comprehension, reading speaking writing and connect words/discourse to meaning. So they are not
just specific to symptoms of FTLD, and the MRI’s finding of atrophy in them could cause many deficits common to Alzheimer’s and/or other NCD causes.

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Ultimately, this fellow’s greatest deficit was seen on tests of short-term memory, which argues for a non-FTLD etiology to his NCD. More likely we are looking at a slightly idiosyncratic DAT which is Major and compensated for by above average IQ. I agree that with his well retained ADLs we should use a Mild NCD dx. I would suggest changing the diagnosis from:

G31.84 Mild Neurocognitive Disorder, due to Frontotemporal Degeneration to

F02.80 Major Neurocognitive Disorder due to
possible Fronto-temporal Lobar Dementia without behavior disorder

And, I’d definately alter the last sentence of the Summary which implies temporal region atrophy on an MRI means the neurologist is thinking of FTLD as I doubt that for the reasons above.

However, if it was my patient, I’d consider that all of the hallmark features of DAT: severe STM & verbal fluency; and mild reasoning & word-finding deficits are present and go for Mild NCD due to Alz. I might note that there were a few signs of FTLD which could be monitored for…
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