I want to scream and cry

Lgb35

DH is 61. I am 55. We started this journey of finding out what is happening in 2019. We have been through so much testing. Lab work, CSF (borderline for AD) we have even driven to Cleveland Clinic several times for testing. MRI’s 2 years apart showed progression of atrophy in the left temporal lobe. Recent Amyloid PET was negative for AD. Yesterday had a FDG PET which showed hypo metabolism in the left temporal lobe. Everything else looked good so nonspecific results. What the hell are we dealing with? I am beyond frustrated!!

trottingalong

So often it is a diagnosis of non specific which is beyond frustrating. The reason for progression of atrophy of the left temporal lobe is often unknown, but the results from my understanding are the same and follow the pattern of dementia. Our journey started with possible MCI with depression. Mild shrinkage of the hippocampus. We have never pursued more testing and he has progressed over the years. Unfortunately you may never receive the concrete answer you are looking for. I’m really sorry you two are going through this at such a young age.

Dio

So sorry you're going through this. I'm in a slightly different situation where DH seems back to normal with the right combo of meds, so his diagnosis now is "dementia of an unknown etiology but may not be dementia." Good grief! It took 9 months at the onset to land on LBD, and he progressed rapidly which led to extreme aggression and violence. Now he's seemingly normal after having added one med to calm his brain. I don't know what to think any more. No one can explain away all the LBD symptoms that responded initially to rivastigmine. And he had minor hallucinations both visual and audio. His hands shake when in use, right side more so than the left on better and worse days. Sometimes it's simply a guessing game for those with atypical conditions. Where's House when you need him?!

SDianeL

there’s so much they still don’t know about diseases of the brain. My DH’s first diagnosis was white matter disease probably Vascular Dementia. Over a year later after I continued to send the Neurologist his behaviors, she looked at the last CT scan and sent me an email stating it was “consistent with Alzheimer’s-Posterior Cortical Atrophy. I know how frustrating it is not to get a definitive answer. Sadly a diagnosis won’t change the outcome.

Vitruvius

My DW had the similar results from a PET scan. That was preceded by hours of cognitive testing by both a neurologist and a neuropsychologist. With those tests they gave a tentative diagnosis of Semantic Dementia, also known as Semantic variant of PPA, a FTD sub variant. The PET scan showing lack of proper functioning in the left temporal lobe was considered a confirmation of that diagnosis. Now of course I am definitely not trying to diagnose your DH as I am utterly unqualified. I am just relating the conclusion made by doctors based on a similar PET scan result. My completely layman's understanding is that problems related to the temporal lobes indicate some variation of FTD. My DW, 73, is now in the very last stage, Stage 7f and they keep telling me it won't be long now, but although she hasn't opened her eyes, moved independently, or spoken a word in months, she refuses to give up it seems.

Lgb35

https://alzconnected.org/discussion/comment/244784#Comment_244784

I feel like it is semantic variant too. We see the neurologist on Monday to discuss the test. They have changed him from MCI to mild dementia.

Lgb35

https://alzconnected.org/discussion/comment/244784#Comment_244784

I would love to hear more about your experience. You are the only person I have found with semantic variant experience. When did your DW get diagnosed

Vitruvius

My DW was diagnosed in October 2020, the pandemic delayed this somewhat. Semantic Dementia (SD) is pretty rare and not well studied. I have read everything I could find on it. The studies that do exist indicate that it is not likely to be caused by genetics, less than 5% of cases could be possibly linked to genetics. My DW had no history of dementia in her family, and I have no history in my family as well, so we had no knowledge of dementia and it’s onset. This made it confusing when symptoms started showing up in about 2018-2019 when she was in her late 60s. Only in hindsight can I see the early warning signs, particularly because in the early stages the cognitive issues are not quite like the more common dementia types.

My DW was first diagnosed at a university medical center which gave a preliminary diagnosis of SD, but they said they needed the PET scan for confirmation. They also wanted a confirmation from an expert in rare dementias at a major national medical center. They thought it would take six months to get this appointment. I got a call from the expert in just two weeks and he was willing to see my DW within days. In the end they confirmed the diagnosis in a 14 page report. They seemed as interested in my DW as a case study, and had a post doctoral student participate in the assessment.

My DW’s progression has been very rapid, exceedingly so according to her neurologist and subsequent hospice nurses. This is somewhat surprising to me as she was very healthy, fit and active and remained so up through about mid Stage 6.

SD is not generally associated with behavioral issues like some other FTD variants and this held true for my DW. There were certainly times when she was agitated and anxious, and occasionally just bit oppositional due to her confusion about what was going on. But she was never aggressive or angry. She could be challenging to deal with only because she was often feeling like she needed to do something or go somewhere that she couldn’t really define let alone articulate.

There is of course much more I can tell you, and would gladly do so. But maybe it’s more useful to address any immediate concerns you have rather than me droning on endlessly.

Lgb35

https://alzconnected.org/discussion/comment/244855#Comment_244855

thank you for your story. We see the neurologist Monday and I will let you know what they say.

Lgb35

https://alzconnected.org/discussion/comment/244855#Comment_244855

so we are still in an unclear diagnosis. The FDG PET was non-specific. They are sending us back to Cleveland Clinic. DH is 61 and still working. Having a diagnosis and one that is on the social security disability compassionate allowance list is vital for us. The amount of money difference between early retirement at age 62 vs disability is substantial. All test, both scans and neuropsychological assessments show atrophy/dysfunction in the left temporal lobe. He struggles with word finding, naming objects or people and is forgetting how to get to frequently visited places.

Did your DW start with just temporal lobe atrophy? I feel like he has Semantic variation (it was also mentioned as a possibility in his chart). I am wondering if it is rare enough that they are struggling to confirm it.

How did your diagnosis experience go

Vitruvius

First off I have been told that Semantic Dementia is very rare. I have seen that my DW’s symptoms were not characteristic of the stereotypical AD symptoms in the early and mid stages. So I can see how it may be harder to diagnose. By the time she was in later Stages, past mid Stage 6, it seems most dementias are the same.

My DW’s problems started with word finding, just like you describe. Her diagnosis was delayed by several things as mentioned before. Or unfamiliarity with dementia being the main cause. Although otherwise healthy she did have hypothyroidism and one of the symptoms is surprisingly, temporary word loss. If the hypothyroidism is controlled then the words come back. So we spent some time getting her thyroid level in check, but the word finding got worse. She also did not have the typical memory or orientation problems of AD in the early stages, but she was beginning to have problems with executive functioning. She liked to bake, but she started having problems undertaking complicated recipes. It was then I came to understand there was a cognitive issue.

This was early in the pandemic and getting regular medical appointments was difficult. Her young regular doctor was useless and did not seem familiar with dementia diagnosis at all. My DW was given both a CAT scan and an MRI. The doctor’s conclusion was “normal brain shrinkage consistent with age” [she was 68 at the time]. This was clearly worthless.

So I was finally able to get her directly into the regional University dementia clinic, without even a referral. The doctors there were great and after hours of cognitive testing over two days, they suspected Semantic Dementia right away but as I mentioned before wanted confirmation from a PET scan and also referred her to a national university medical center that had a specialist in rare dementias. They provided me with quite a lot of info on Semantic Dementia and it correlated closely with my DW’s symptoms.

As her dementia progressed she had increasing problems with “concept” words as the doctor had pointed out. As an example when her birthday came she did not understand the concept of a birthday. It was not that she had just forgotten when her was birthday was. Her speech was judged to be “grammatically correct but devoid of content”. She began inventing words. Now to some extent this is true of other, if not all dementias, but it came earlier and more pronounced with her.

One thing that is different from your DH, is she did not have much problem with orientation. For example a little later, on our second visit to a doctor, she gave me proper turn-by-turn directions only having been there once before three weeks earlier, but she did so because she did not think I was the same person who took her there the first time!

I hope this helps somewhat and would be glad to provide more info if you desire.

jfkoc

You may never get a correct diagnosis. You are left, at least right now, with learning about all non-medical treatment.

What, you ask. Start with getting the book The 36 Hour Day and any book on validation by Naomi Feil. You will need to learn how to communicate all over again.

and…..do use this message board…we understand, we care and we have "cutting edge info".

SDianeL

I’m not sure you need a specific detailed diagnosis. He needs to be unable to work. Perhaps the Cleveland Clinic doctor can help with a general diagnosis of Dementia. Here’s what AI search says: Yes, the Social Security Administration (SSA) recognizes neurodegenerative diseases as qualifying for disability benefits, but the severity of the condition must prevent the individual from working. Specifically, the condition must be severe enough to prevent engaging in substantial gainful activity and must be expected to last for at least 12 months or result in death. 

SDianeL

here’s a checklist Alzheimer’s Org provides. I would call their toll free number for assistance with disability. https://www.alz.org/getmedia/69ae2aac-9759-4a6f-a36a-3c34a1394497/alzheimers-dementia-social-security-disability-insurance-ssdi-checklist-ts.pdf

Lgb35

https://alzconnected.org/discussion/comment/245358#Comment_245358

hi, I have been thinking about you and your DW. I know you were nearing the end stages. How is she doing? How are you doing?

difergtx

My husband has been diagnosed with SD with anomia. We have been going to a neurologist who is a very good doctor, but hasn’t been forthcoming about resources for me to understand what is going on. She says that everyone’s journey is different, almost like she doesn’t want to be held responsible for giving me incorrect information. Where did you learn about this variant? I find that most info is about AD. I vacillate between wanting to know everything and just accepting that there is nothing I can do about it. My husband is not that interested in doing more tests as he has always been frugal and can’t stand the thought of for paying for more tests. Interesting, as he was a PCP himself! Doctors are truly the worst patients!

Lgb35

We went to the Cleveland clinic for a work up about 5 years ago and they mentioned it could be semantic variant. There is not a lot out there on it but if you look up FTD and the 4 main variants you can learn some things. I would really like to know how this variant progresses.

Vitruvius

Lgb35,

Thanks for asking about how we’re doing. My DW’s condition has of course declined since early June when I last wrote. As I have previously noted, you would think she was comatose if it wasn’t for the fact that she will open her mouth and take food and liquids from a spoon if you tap her lip. They refer to this as minimally conscious with a feeding reflex.

I have not seen her with her eyes open in about six months, she has occasionally opened her eyes for a few minutes, but never when I’ve been there. This last Monday she opened her eyes for about five minutes for the first time in a month or so. The aides were so excited they took photos to show me when I came later that day. In June she was feeding three times a day but was eating less and less. In agreement with hospice we agreed that starting a few weeks ago she would only be given puréed food at breakfast and then only thickened liquids at lunch and dinner. She only eats a portion of her breakfast and only drinks about one glass of liquid other meals. At this stage, too much food causes intestinal problems.

Hospice is amazed at her constitution, they note that almost all dementia patients they see die before they reach this very advanced state. She is the farthest along at her MCF yet she is also the youngest. She was fit, athletic and other than hypothyroidism, had no real medical issues. Now her muscles are severely contracted and she makes no independent movements. She is still put in a large Broda chair for a while in the mornings and is taken outside by me or the aides most days.

As far as how I’m doing? Well I live day to day waiting for the inevitable, which they keep telling me is soon, but soon keeps getting pushed out. Her quality of life is zero and she would despise the condition she is in. I’m sorry to say the end can not come too soon.

I haven’t thought too much about my future, I’ll figure that out when we get to State 8. If I live as long as my parents, I have decades left to think about it. I do know that my DW made my life so much more wonderful than it would have been without her. After she’s gone I will live with her afterglow.

Lgb35

https://alzconnected.org/discussion/comment/249035#Comment_249035

good to hear from you! I agree with you it’s hard to say but there comes a point when you just want their suffering to end. Hang in there day by day, minute by minute.

We are going to Cleveland Clinic the beginning of August. I am hoping to get a solid guess diagnosis then. I feel like he does have semantic variant FTD and it’s rare enough not everyone recognizes it

feelingalone2

https://alzconnected.org/discussion/73191/i-want-to-scream-and-cry

hello, sounds almost identical to my DW, same place, CFS was borderline but inconclusive for AD, then a year later an MRI shows atrophy with which they consider is early onset dementia. It is frustrating to say the least. Keep fighting and keep searching. Love