New platform may help to ID cognitive problems in ALS

Patients found willing, able to take part in at-home monitoring: Study

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by Andrea Lobo |

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A new home-based neurocognitive platform from Cumulus Neurosciences is feasible to use and patient-friendly, and shows potential to identify cognitive problems and to discriminate between people with amyotrophic lateral sclerosis (ALS), those with frontotemporal dementia (FTD), and healthy individuals.

That’s according to preliminary data from a first-in-class study, dubbed CNS-102, that’s testing the new platform. The study is being conducted by Cumulus together with Precision ALS, an Irish collaborative research program hosted by Trinity College Dublin, in Ireland.

The Cumulus platform aims to collect data through repeated, intensive at-home sampling — which could one day allow people with ALS, FTD, and other disorders to take part in decentralized clinical trials.

“We are encouraged by the analysis from our CNS-102 feasibility study, which confirms that patients living with these devastating conditions are willing and able to participate in home-based studies with intensive protocols,” Brian Murphy, PhD, Cumulus’ founder and chief scientific officer, said in a company press release.

According to Orla Hardiman, MD, the study’s principal investigator, “the results of CNS-102 provide preliminary evidence that the Cumulus platform can identify cognitive deficits across these patient groups.”

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The preliminary findings of CNS-102 were presented in two posters at the European Network to Cure ALS (ENCALS) Annual Meeting, held July 12-14, in Spain. The study had focused on the feasibility of use of the Cumulus platform.

“Importantly, across study [groups] we saw very high rates of successfully completed sessions, which confirms that the platform is patient friendly and easy to use,” said Hardiman, who also serves as the lead investigator of Precision ALS, and is head of Trinity College Dublin’s academic unit of neurology,.

ALS is a progressive neurodegenerative disease that affects the nerve cells in the brain and spinal cord that control voluntary movements, causing these cells to degenerate and die. This leads to a gradual loss of muscle control, affecting the patient’s ability to perform everyday tasks.

Besides motor impairments, about half of all ALS patients experience cognitive problems and behavioral issues. These include deficits in social and emotional cognition, language difficulties, apathy, and irritability, as well as impaired executive function — meaning problems with the set of mental skills that include working memory, planning, attention, flexible thinking, and self-control. In this context, social and emotional cognition refers to how people process, understand, and use information from others to interact with them.

In some patients, these cognitive and behavioral symptoms reach clinical relevance and lead to the diagnosis of frontotemporal dementia, known as FTD, a neurodegenerative condition that shares biological mechanisms with ALS.

Importantly, across study [groups] we saw very high rates of successfully completed sessions, which confirms that the platform is patient friendly and easy to use.

“Detection of cognitive or behavioral changes in patients living with ALS or FTD can be challenging during clinical visits due to a variety of factors including fatigue and white coat syndrome,” Murphy said.

White coat syndrome refers to a patient’s anxiety in a medical environment — often manifested, for example, as abnormally high blood pressure readings in a doctor’s office.

As such, according to the researchers, at-home, regular monitoring may provide more accurate results relating to patients’ cognitive function and behavior, as well as in changes over time.

“A longitudinal home-based approach is more ecologically valid and provides less variable estimates of true performance,” the researchers wrote in on of the posters.

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Testing focused on cognitive problems and behavior issues

The Cumulus neuroassessment platform, which uses digital tablets, provides a set of functional assessments and simultaneous non-invasive monitoring of the brain’s electrical activity. To measure such electrical activity, the platform uses its U.S. Food and Drug Administration-cleared, self-applied electroencephalogram (EEG) headset.

The belief is that, by allowing the remote monitoring of patients at home, the platform may be more effective at detecting relevant changes, and may be a valuable tool for patient care and clinical trials testing potential ALS therapies.

The CNS-102 study, conducted in Ireland, enrolled 10 ALS patients, seven FTD patients, and 10 age- and education-matched healthy people, who served as controls.

Participants completed a full neuropsychological assessment covering cognitive function and behavior at the study’s start, and after four and eight months. At the same time, they were asked to complete three 25-minute Cumulus platform sessions every two weeks.

These sessions included gamified tasks to assess mood and emotion processing, language, executive function, memory, and motor skills, while measuring the brain’s electrical activity with the EEG headset. Key cognitive-behavioral and EEG measures were evaluated based on data from the first five sessions.

In the first poster, “Patient and technical feasibility of real-world sampling of cognition and functional neurophysiology in Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) (abstract #286), Cumulus researchers showed that 47.3% of ALS patients, 68.9% of FTD patients, and 68.2% of the controls adhered to the platform. Moreover, all groups successfully completed more than 85% of the sessions.

These interim data “from this first-in-class study suggest that older individuals, and those with FTD and ALS are capable and willing to participate in home-based studies with intensive repeated sampling protocols,” the researchers wrote.

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In a second poster, titled “Longitudinal cognitive assessment using the Cumulus home-based EEG platform in ALS and FTD” (abstract #272), researchers at Precision ALS and Trinity Biomedical Science Institute (a Precision ALS host) shared data on the platform’s ability to differentiate the groups.

The full neuropsychological assessment showed significant group differences in terms of cognitive function. Cumulus platform data also revealed significant differences between the ALS, FTD, and control groups in tasks related to psychomotor skills, general cognitive function, and descriptive speech rate.

The team noted that people with FTD performed worse in both Cumulus and full battery measures, followed by those with ALS, and then healthy controls.

These findings confirmed that the platform can identify cognitive deficits in these groups and separate them, “constituting positive initial evidence for task validity and technical feasibility,” the researchers wrote.

“Further research will establish how comparable the Cumulus platform is with gold-standard neuropsychological assessment and its sensitivity in detecting cognitive decline,” the researchers wrote.

The Cumulus researchers said the CNS-102 study results highlight a role for the platform in helping to develop new therapies — particularly those addressing cognitive problems — for both ALS and FTD.

“We are deeply grateful to the patients, caregivers and collaborators who dedicated their time to this important first-in-class study,” Murphy said.