ALS-Bulbar onset and FTD-Frontotemporal dementia

[Cereus]

Hi Everyone,

I've been reading this forum for a couple weeks. Over the last 4 months my hb has been in neurological testing for dementia. He's had speech difficulties for the last 10 months. Only recently did the docs put it all together. (Thanks to members of the FTD forum who posted similar symptoms.)

We have a "probable" diagnosis of ALS (bulbar) and FTD. They did two tests Friday, Nov 20, electomyography (EMG) and nerve conduction velocity (NCV). Then they did another MRI immediately of his cervical spine and brain stem area to make sure there is nothing there they missed that may be causing his symptoms. Last, they did more blood work to look for things specific to an ALS diagnosis I think.
We go back soon for the doctor to tell us the results of the last testing. He said it is likely ALS but to wait a couple more days, there is a slim chance. He never called the cognitive decline FTD by name, but confirmed there was dementia (earlier neuropsych testing confirmed mild cognitive dysfunction).

I'm not sure why but the docs are able to address ALS more than FTD. Maybe it's more tangible and actually "shows" on paper, charts and computers? My hb also understood it much better than he understood the cognitive decline discussions at our last few appointments. My hb was disturbed at the probable diagnosis but not to the extent a normal person would be. He quickly moved on mentally and was more upset that he had more tests that would delay the work at home he planned for the afternoon. He did look at me and ask if I was ok.

I'm so very sad but relieved to know what we are dealing with. It helps that my hb is understanding it. He was more lucid than usual. He even looked it up online. He's very unemotional and I can't tell if he's hiding his distress or no longer capable?

I'm glad to know of this forum and would like to connect with others who have the Bulbar and dementia situation.

 

[shelleynshaggy]

Just to show that all of these journeys are different - my husband's FTD was diagnosed first and realitvely fast. The ALS/MND is what is taking the time. His neurologists are hesitant to diagnose ALS because he doesn't have any muscular atrophy "yet." Both neurologists also commented about what good shape my husband is in physically.

Also, we were referred to the "behavioral" neurologist first - thus I think he recognized the FTD symptoms faster - literally almost immediately. He ran tests for both. We returned a month later for a diagnosis of ALS syndrome/MND which was confirmed in October by the ALS clinic. Now - like most we just have to wait and see.

Jim had no noticable physical sympotms in July - now you can't miss them. I think it is just what symptoms emerge first.

 

[indigosd]

You will find incredible support and insightful advice in this forum! Please let us know how your next appt goes and the results.

 

[Barden]

Hi. We also have a "probable ALS" diagnosis and although not mentioned by Mayo at all (unbeliveable) he has definite symptoms of FTD as mentioned in medical articles I have read on this forum adn the same exact behaviors you describe. I now think that Probable ALS means he has ALS and I know also think he has severely diminished mental capacity in terms of decision-making, efficiency, quickness, behavioral zombieness and sometimes extreme apathy. It is like living with a complete stranger. I wish there was a way to explore this further with you....

 

[Cereus]

Dear Barden, I'm very sorry to hear you and your LO are on a similar path. If you haven't joined already, the FTD Support Forum is an excellent place to get information and support from those who are dealing with FTD. Each pFTD has their own unique behavioral issues from apathy to horrible compulsions. There is so much information there on whatever stage you are in. Several of us there (and here) have the double situation of FTD and ALS. My knowledge of bulbar ALS is minimal - only what I've seen in my hb. I'm here to learn more too. I'm not sure how to PM (private message) here but am happy to share any information I have, listen to your frustrations or simply say hello. On the FTD forum my name is pcf. Take care, you are not alone.

 

[indigosd]

We are all here for you!

 

[Barden]

Thank you Cereus for the reply and the link to the FTD Support Forum. What a godsend. I will start to learn more. Is your situation the same with loss of speech and a negative EMG on the tongue but no place else (so far) along with the FTD? Are you also going to Mayo? Looking back, I am now thinking he had these mental symptoms perhaps years ago but I did not notice until the slurring, tiredness, weight loss and twitching started. His voice has steadily gone downhill since January...no good days anymore...will he totally lose his voice? Thank you thank you thank you. I will be in touch.

 

[Cereus]

Sorry for the delay. I thought I was subscribed to this thread but I guess not.
To answer your question Barden, it was either my hb's electromyography (EMG) or nerve conduction velocity (NCV) that supported the ALS diagnosis. (I have read the difference but forget right now) I was led to believe they found evidence in all 4 limbs and the throat. He has only small physical symptoms in his limbs. He drives, does a lot of physical work but only lasts a half day and is on the couch all afternoon, in bed by 7:30. His voice is at about 2 - 3 sentences before he has to take a break. He says it's not painful but difficult to form the words and sometimes to think of the right words. His voice was normal in July. I expect him to lose it all together but have not seen an ALS specialist yet so am no expert here. We got our "final diagnosis" Dec 3 and are trying to get an appt with a local specialist. If that is not adequate, we will head to Mayo in Phoenix. Oh, his cognitive symptoms. I too know there were symptoms several years ago. He is capable of day-to-day routine things around the house. Actually remembers some things better than I do. But he is beginning to forget people, places and cannot input any complex information. All new input must be slow, one thing at a time and not too much - otherwise he doesn't retain it. His behavior has changed from an impatient, negative critical man last year, to an impatient, mostly emotionless one, but on rare occasions shows short delightful bursts of true sweetness to me. Just when I think the lights are all but out, he gives me a smile or a hug.
Hope this helps.

 

[Gelthling]

Hi Ladies,

my mum has the Bulbar and the FTD - I am now starting to wonder if they go hand in hand or perhaps it depends on where the MND affects the brain? Just a thought.

Mum was diagnosed with MND in March, and started showing the FTD signs not long after...then she was just starting to use a cane and occasionally writing some things we couldn't understand. Now, she cant talk at all, uses mainly thumb up and thumb down rather than write, and in a wheelchair full time.The FTD makes her "spaced out" on occasions, she has become kinda obsessive compulsive with different things and you can loose her when your talking to her and have to bring back her attention to ensure it all goes in.

Mum had been getting bullied at work, and looking at a report I found for over a year ago I found the other day, she was having trouble with many things like communication and writing up minutes of meetings and had difficulty with spelling - things she never had trouble with in an admin position before. Still not a reason to be bullied, but were they due to the bulbar MND or the FTD? Who will know....at the moment we just take each day as it comes.

Best of luck to both of you Barden and Cereus.