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More than a third of people diagnosed with <a href="https://alsnewstoday.com/what-is-als/">amyotrophic lateral sclerosis</a> (ALS) also showed signs of frontotemporal dementia (FTD), according to a new study of brain tissue.
In more than 90% of tissues examined, patients had clumps of the TDP-43 protein, a hallmark of ALS that contributes to nerve damage, but ALS patients with FTD signs showed more extensive involvement. Moreover, when the tissue analysis was compared with clinical examination, ALS was more accurately diagnosed than ALS-FTD due to a wide variability in clinical presentation in FTD, the researchers noted.
&#8220;Neuropathological [tissue-examination] studies and correlation with clinical characteristics help us expand our knowledge of the basis of diseases such as ALS and frontotemporal dementia, opening doors to future studies on biomarkers and specific therapies,&#8221; Álvaro Carbayo, MD, co-lead author and a researcher at the Sant Pau Research Institute, in Spain, said in a <a href="https://www.recercasantpau.cat/en/now/desxifren-les-connexions-cerebrals-entre-lesclerosi-lateral-amiotrofica-ela-i-la-demencia-frontotemporal/">press release</a>.
The tissue examination study, &#8220;<a href="https://academic.oup.com/brain/advance-article/doi/10.1093/brain/awae011/7560436">Clinicopathological correlates in frontotemporal lobar degeneration: motor neuron disease spectrum</a>,&#8221; was published in <a href="https://academic.oup.com/brain">Brain</a>.
ALS, also called motor neuron disease, is marked by progressive muscle weakness and wasting (atrophy). It&#8217;s <a href="https://alsnewstoday.com/causes-of-als/">caused</a> by the degeneration of motor neurons, the specialized nerve cells that control voluntary muscle movements, in the brain, brainstem, and spinal cord. FTD is a group of dementias marked by changes in behavior and language. Like ALS, it&#8217;s also caused by motor neuron degeneration, but it mainly affects neurons in two different brain regions, the frontal and temporal lobes.
In most ALS patients, and up to half of those with FTD, motor neuron function is disrupted by the toxic accumulation of a misfolded version of the TDP-43 protein. The two diseases are also associated with mutations in the C9orf72, TBK1, and TARDBP genes. Moreover, about half of ALS patients will develop behavior and cognition changes, with an estimated 10-15% meeting the criteria for an FTD diagnosis.
[recommended-reading id=76077]
<h3 class="p1"> Frequency of FTD in ALS explored</h3>
Because brain tissue analysis remains the reference for a definite diagnosis of FTD, and not many studies have performed those analyses in ALS, the exact frequency of FTD in ALS patients is difficult to estimate.
To find out, a team of researchers led by Carbayo and Ricard Rojas, MD, PhD, examined clinical data and brain tissue from ALS patients with and without FTD to analyze the frequency of FTD and also to define specific subgroups based on their clinical, genetic, and tissue-related characteristics.
&#8220;It is crucial that we work together to better understand this spectrum of neurodegenerative diseases and to better care for our patients,&#8221; Carbayo said.
The researchers examined brain samples from 124 patients, whose mean age at death and brain donation was 66.4. Among them, 44 (35.5%) showed features associated with FTD (ALS-FTD), which exceeded previous clinical estimates, the researchers noted.
Nearly all brain samples (93.6%), with or without FTD, had TDP-43 protein aggregates. Among those with signs of FTD, 90.9% had TDP-43 aggregates, which were more widely distributed across brain tissue than in people with ALS alone.
Cognitive or behavioral changes during clinical follow-up were seen in 38.7% of patients, but significantly more patients in the FTD group had these changes than those with ALS alone (88.6% vs. 13.3%).
A clinical diagnosis of FTD was more common in ALS patients with FTD signs than in those with ALS alone (67.4% vs. 6.3%). Five patients received an FTD diagnosis without tissue signs of FTD, but they had other disease-related changes in brain tissue that could explain cognitive-behavioral impairment.
When the team compared diagnosis between clinical data and tissue analysis, ALS disease was more accurately diagnosed than ALS-FTD (93.8% vs. 61.4%).
No statistically significant differences were found in the survival rate between ALS and ALS-FTD, with both groups showing a similar disease duration. Similar survival rates were also found regardless of area of onset, the presence of symptoms affecting the face and throat, or disease-causing mutations.
Genetic mutations related to ALS occurred more frequently in ALS-FTD than in ALS (31.8% vs. 5%) and C9orf72 mutations were independently associated with signs of FTD in brain tissue.
&#8220;We have observed astonishing heterogeneity [variability] in the clinical presentation and pathological and genetic characteristics of patients, with a higher frequency of frontotemporal dementia than described in other series,&#8221; Carbayo said.
Said the researchers: &#8220;We describe a frequency of [FTD] of 35.5% in our series of neuropathologically confirmed cases of MND. The [ALS-FTD] spectrum is highly heterogeneous in all aspects, especially in patients with [FTD], in whom it is particularly difficult to define specific subgroups.&#8221;

Signs of frontotemporal dementia seen in about 35% of ALS patients

Researchers have unveiled a new AI-powered avatar platform — one making use of artificial intelligence (AI) technology — that aims to help people with <a href="https://alsnewstoday.com/what-is-als/">amyotrophic lateral sclerosis</a> (ALS) to communicate, even when the neurodegenerative disease progresses to the point that speaking and moving become difficult or impossible.
The platform was developed through a collaboration between tech companies <a href="https://www.lenovo.com/us/en/">Lenovo</a> and <a href="https://www.deepbrain.io/">DeepBrain AI</a>, as well as the <a href="https://www.scottmorganfoundation.org/">Scott-Morgan Foundation</a>, a nonprofit that focuses on assistive technologies.
“This is a fantastic example of the untapped potential of generative AI to empower individuals and build a more inclusive future,” Scott Tease, vice president and general manager of high-performance computing and AI at Lenovo, said in a <a href="https://www.businesswire.com/news/home/20240109052328/en/AI-Powered-Avatar-from-Lenovo-DeepBrain-AI-and-the-Scott-Morgan-Foundation-Creates-New-Communication-Possibilities-for-People-with-Severe-Disabilities">press release</a>.
The AI-powered platform was revealed at CES 2024, a tech showcase being held Jan. 9-12 in Las Vegas. Andrew Morgan, CEO of the Scott-Morgan Foundation, said the nonprofit&#8217;s vision “is built on a commitment to ensuring a universal right to thrive through technology.&#8221;
&#8220;AI is accelerating work we began years ago, and our brilliant collaborators are helping us completely change the accessibility landscape,&#8221; Morgan said. &#8220;Collectively, we are lighting a beacon for what’s possible—not just for people with ALS, but for everyone.”
[recommended-reading id=87020]
<h3>AI-powered assistive tech platform aims to help those with severe disease</h3>
ALS is characterized by the progressive death and degeneration of the nerve cells that control voluntary movement. This eventually leads to paralysis, which can make it hard for people with the condition to communicate.
DeepBrain has been developing AI-based avatars — video portrayals that capture the look and personality of people. As part of an ongoing collaboration with the Scott-Morgan Foundation, Lenovo spearheaded an effort to adapt this technology to help people with ALS and other disease maintain communication abilities even when they can no longer do so with their own bodies.
&#8220;Leveraging our expansive solutions portfolio and in-house expertise, we love finding these opportunities to reimagine what’s possible and help solve humanity’s greatest challenges,&#8221; Tease said.
Eric Jang, CEO of DeepBrain AI, said his company&#8217;s goal is to use AI-powered tech to &#8220;enable hyper-realistic avatars for everyone.”
“We are constantly amazed at how our partners and clients think of new ways to use AI-generated video,&#8221; Jang said. &#8220;Lenovo had the foresight to share their vision on how this technology could be implemented as a component of an assistive technology platform.”
As a proof-of-concept, Erin Taylor — a 24-year-old woman who last year was diagnosed with ALS — underwent full-body video capture at a studio. That was then used to <a href="https://www.youtube.com/watch?v=4LxqB6gS7Dc&amp;feature=youtu.be">create an avatar for her</a> that offers 96% true-to-life accuracy. The avatar was debuted at CES 2024.
Taylor, who lives in California with her mother, was pursuing a career in botany and forestry when she experienced &#8220;a brief series of escalating symptoms&#8221; that resulted in her diagnosis.
“Feeling so devastated and lost, we were grateful to the Scott-Morgan Foundation for inviting us to help build something new and shape the future,” Taylor said.
[quote=&#8221;center&#8221;]The avatar is incredible, but what matters most to me is that this technology could help change the lives of many more people facing ALS or other diseases — a way of showing how bright the future can still be.[/quote]
Lenovo also has used AI to help develop predictive typing tech, with the aim of making it easier for people with ALS and other motor-limiting diseases to find the right words when a traditional keyboard or voice-to-speech is no longer an option.
Researchers are working to integrate the predictive system along with the avatar and an eye-tracking keyboard, with the ultimate goal of letting people with conditions such as ALS be seen and heard in the digital space.
“I have no doubt this pioneering proof of concept will one day help shape even more staggering innovations,” Morgan said.
“Our foundation was built on the dream that technology, and AI especially, can break through severe disabilities and ensure a right to create, connect, and live life joyfully. We’re making that happen,” Morgan added.
For Taylor, the project is also about the end goal.
“The avatar is incredible, but what matters most to me is that this technology could help change the lives of many more people facing ALS or other diseases — a way of showing how bright the future can still be,&#8221; Taylor said.

Novel AI-powered platform aims to help ALS patients to communicate

One important habit I enjoy doing every day is keeping up with the latest <a href="https://alsnewstoday.com/category/news/">ALS-related news</a> and research. But every once in a while, I come across an article that has me muttering, “Was this research even needed?” And more often than not, I answer my own question with, “Well, no.” The researchers could’ve just asked me or any other <a href="https://alsnewstoday.com/what-is-als/">ALS</a> patient for our opinion.
What pushed my buttons was <a href="https://alsnewstoday.com/news/anxiety-als-patients-family-soon-after-diagnosis/">reading about a recent two-year study</a> that found that many ALS patients and their relatives experience anxiety and depression after a <a href="https://alsnewstoday.com/als-diagnosis/">diagnosis</a>. Really?
Plus, I knew this study was just one more in a long line of variations on the same theme, published over and over. Several years ago, I <a href="https://alsnewstoday.com/columns/als-depression-link-anxiety-prognosis/">wrote</a> about this same topic. In it, I referenced several studies dating back to 2005, all looking at the possible association between anxiety-depressive disorders and ALS. Really?
Why are medical experts still trying to figure out if ALS causes patients to feel negative emotions, especially when concise and useful <a href="https://alsnewstoday.com/als-mental-health/">resources on the subject</a> are already available?
[recommended-reading id=86545]
<h3>What we want</h3>
What do ALS patients want? My advice: Don’t guess; ask us and we’ll gladly tell you.
An excellent example of asking is the recent survey that <a href="https://alsnewstoday.com/">ALS News Today</a> reported on in &#8220;<a href="https://alsnewstoday.com/news/survey-offers-look-life-treatment-priorities-those-living-als/">Survey offers look at life, treatment priorities of those living with ALS</a>.&#8221;
Even though the survey represented ALS patients from nine European countries, I believe similar results would be found in the U.S.
Among the many insights shared in the survey, this statement stood out to me: “When asked about what they valued more about current and new <a href="https://alsnewstoday.com/approved-treatments-for-als/">treatments</a>, most patients wanted therapies that could stop disease progression.”
In other words, the next best thing to a cure.
<h3>Looking forward</h3>
While this past year brought us improved ALS treatments, support services, and increased focus on mental health, let’s continue to keep our focus on the goal: a cure for ALS.
I believe it&#8217;s possible. I look forward to the day when I read the grand announcement that there will be a world without this disease.
While we wait for that day, let’s help each other learn to live well while we&#8217;re <a href="https://alsnewstoday.com/living-with-als-2/">living with ALS</a>.
<hr />
Note: ALS News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of ALS News Today or its parent company, BioNews, and are intended to spark discussion about issues pertaining to ALS.

Just ask and ALS patients will tell you what we want: a cure

Disaster preparedness, including stockpiling goods, owning and knowing how to use emergency equipment, and being aware of community resources, is lacking among <a href="https://alsnewstoday.com/what-is-als/">amyotrophic lateral sclerosis</a> (ALS) patients and caregivers, a recent Japanese study suggests.
While patients who needed a ventilator were generally found to be better prepared during power outages or emergency evacuations than those who didn&#8217;t, their awareness was still insufficient, the researchers said.
“Academic societies and local governments have been responding to disaster preparedness for patients with intractable diseases,&#8221; Osamu Kano, MD, PhD, a professor at Toho University in Tokyo, Japan, and the study&#8217;s senior author, said in a <a href="https://www.eurekalert.org/news-releases/1001818">press release</a>. &#8220;We have taken better measures than before, but it is still not enough. We believe that our paper has the potential to provoke and advance further discussion on disaster preparedness for patients with ALS.&#8221;
The study, &#8220;<a href="https://www.sciencedirect.com/science/article/abs/pii/S0967586823002011#:~:text=Patients%20with%20advanced%20ALS%20have,patients%20with%20ALS%20in%20Japan.">The necessity to improve disaster preparedness among patients with amyotrophic lateral sclerosis and their families</a>,&#8221; was published in the <a href="https://www.sciencedirect.com/journal/journal-of-clinical-neuroscience">Journal of Clinical Neuroscience.</a>
In ALS, the loss of nerve cells needed for muscle function leads to <a href="https://alsnewstoday.com/symptoms-of-als/">progressive weakness</a> that affects mobility and breathing. Many ALS patients are wheelchair-bound and require external respiratory support.
Disasters, such as earthquakes, snowstorms, typhoons, or even war, can lead to power blackouts, a big concern for people with ALS, whose life-sustaining equipment relies on electricity. It may also be difficult to transport patients to evacuation centers.
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<h3>Patients not prepared for a disaster</h3>
Because of these, disaster preparedness is critical for ALS patients and may require extra planning. Few reports have investigated how prepared patients are, leading Kano and his colleagues to investigate disaster preparedness among 48 ALS patients and 23 caregivers in Japan where several earthquakes in the last few decades have impacted people receiving at-home respiratory care. The patients (27 men, 21 women; mean age, 60) and the caregivers (mean age, 55.7) responded to a questionnaire.
A quarter of the patients were on a ventilator for respiratory support and 56.5% of the caregivers cared for patients on a ventilator. No patient lived alone — two were hospitalized and the rest lived with caregivers.
Only 28% of survey respondents indicated they were generally prepared for disasters. A significantly lower proportion of those not using a ventilator (10.9%) said they were prepared relative to those that did use one (60%).
About 86% didn&#8217;t have plans for communication if phone lines were down. Fewer than half of the responders indicated they had secured transportation (36.6%), had an emergency contact list (28.2%), had human resources for transport (23.9%), were registered with the government as needing support in an evacuation (23.9%), or had contacted local support centers (12.7%).
There was a general lack of awareness about available resources, such as local government support or intractable disease consultation and support centers, the researchers noted.
About a quarter of participants indicated they didn&#8217;t stockpile food, water, or medical products, but those who used ventilators were significantly more likely to stockpile medicines and sanitary materials than those who didn&#8217;t.
Among those using a ventilator, 52.4% indicated their power supply wouldn&#8217;t be maintained for a half a day or longer after an outage.
In the event of an emergency, the government recommends having backup emergency equipment such as an external ventilator battery and a bag-valve mask, which can be used to manually assist breathing. Of those using a ventilator, 60% had a bag-valve mask and 68% had an external battery. About 12% didn&#8217;t have any of this equipment. For those who did have it, 75% didn&#8217;t know how to use the bag-valve mask and 41% had insufficiently charged external batteries.
&#8220;It is necessary not only to have emergency equipment but also to ensure that it can be used,&#8221; wrote the researchers, who said their study &#8220;shows that patients with ALS and their families without ventilators have a low awareness of disaster preparedness and even the level of awareness among patients with ALS and their families with ventilators is insufficient.&#8221;
Efforts are needed to increase preparedness, they said, noting these could include establishing evacuation centers in communities for patients who need ventilation and maintaining a list of people who would require it.
&#8220;Support from the government and academic societies is essential for these efforts, and the activities of the Disaster Counter-measures Committee, which is currently underway, should be used to further raise awareness of disaster preparedness,&#8221; the researchers said.

Tag: ALS patients

Signs of frontotemporal dementia seen in about 35% of ALS patients

Novel AI-powered platform aims to help ALS patients to communicate

Just ask and ALS patients will tell you what we want: a cure

Disaster preparedness insufficient among patients, caregivers in Japan