Emotion Processing Among Patients With ALS

NCT06566651 | Recruiting

• 1 other identifier: emotionperceptionALS11072308
• Study Type: observational
• Target: 180
• Locations: 1 country
• Sites: 2

Brief Summary

The goal of this observational study is to learn about the emotional perception in people with ALS disease compared to people with other neuromuscular disease and healthy controls. The main questions it aims to answer are:

• How people with ALS judge happy and angry faces and what their "insight" into these judgements are like
• How their autonomic responses differ from the other two test group Participants will asked to judge if a face presents a happy emotion or angry emotion. Researchers will compare the ALS group responses with neuromuscular diseases group and healthy control group responses to see if the ALS group judge more happy faces than angry.

Conditions

Amyotrophic Lateral Sclerosis; Neuromuscular Diseases; ALS; Motor Neuron Disease; MND (Motor Neurone DIsease)

Keywords

Amyotrophic Lateral Sclerosis; Neuromuscular Diseases; Emotion Perception; Emotion Processing; Emotion Recognition; Autonomic Responses; Respiratory Responses; Heart rate; Emotion Discrimination Task; ALS; Motorneuron disease; MND

Outcome Measures

Primary Outcomes (2)

• The perception of facial emotions measured using an Emotion Discrimination Task (EDT).

  The EDT requires subjects to assess the facial emotion of a face stimulus and report whether the facial emotion was angry or happy. This will be the operationalisation of subjects' emotion perception, which we anticipate will reveal a bias towards positive perceptions. There is thus two scores on this scale: "Angry" and "Happy". There is no one of these scores that is "better" than the other. It is an nominal categorical scare.

  20 minutes

• Metacognitive sensitivity measured using a retrospective confidence rating scale

  Metacognitive insight will be operationalized through a metacognitive sensitivity measure. The method employed for this is a confidence rating measure, where subjects assess their own performance on the EDT. Subjects will indicate on a sliding scale, ranging from "very confident" (maximum score) to a "pure guess" (minimum score), how confident they are that their previous answer was correct. Generally higher scores on this scale is considered better. This scales title is: " Confidence Rating Scale".

  20 minutes

Secondary Outcomes (2)

• Subjects' heart rate is monitored throughout the EDT

  20 minutes

• Subjects' respiration is monitored throughout the EDT

  20 minutes

Study Arms (3)

Amyotrophic Lateral Sclerosis (ALS)

People diagnosed or suspected of having the neurodegenerative disease Amyotrophic Lateral Sclerosis (ALS)

Other: Emotion Discrimination Task (EDT)

Neuromuscular

People diagnosed with a neuromuscular disease other than ALS

Other: Emotion Discrimination Task (EDT)

Control

Healthy people

Other: Emotion Discrimination Task (EDT)

Interventions

Emotion Discrimination Task (EDT) OTHER

It estimates the subjective bias and sensitivity in discriminating between happy and angry facial expressions of different intensities of emotional expression

Amyotrophic Lateral Sclerosis (ALS); Control; Neuromuscular

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

* Residents of Aarhus and Aalborg * Patients at Aarhus University Hospital * Patients at Aalborg University Hospital

You may qualify if:

• ALS patients, ambulant and hospitalized
• Able to give informed consent
• Diagnosed with ALS or probable ALS according to the existing revision of the El Escorial Criteria 21,22.
• Patients with a peripheral neuromuscular disease, ambulant and hospitalized
• Able to give informed consent
• Diagnosed with a peripheral neuromuscular disease, that does not affect CNS, including but not limited to Myasthenia Gravis and polyneuropathy
• Healthy controls
• Able to give informed consent
• Age and gender matched to ALS patients

You may not qualify if:

• All Participants
• Other severe medical, neurological, or psychiatric disorders
• Visual impairment to an extent that interferes with the ability to perform of the test
• Severe motor or cognitive deficits, to the extent that the test-task cannot be performed
• Alcohol or drug abuse to an extent the interferes with task performance
• Patients with a peripheral neuromuscular disease
• ● Familial predisposition to ALS
• Healthy controls
• Familial predisposition to ALS (first degree relatives)
• Medical treatment that affects the central nervous system (e.g., antidepressants)

Sponsors & Collaborators

• University of Aarhus: lead
• Aarhus University Hospital: collaborator
• Aalborg University Hospital: collaborator

Study Sites (2)

Aarhus University Hospital

Aarhus, Central Jutland, 8200, Denmark

RECRUITING

Aalborg University Hospital

Aalborg, Region Nordjulland, 9000, Denmark

NOT YET RECRUITING

Related Publications (22)

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• Sedda A. Disorders of emotional processing in amyotrophic lateral sclerosis. Curr Opin Neurol. 2014 Dec;27(6):659-65. doi: 10.1097/WCO.0000000000000147.

  PMID: 25333604 BACKGROUND

• Strong MJ, Abrahams S, Goldstein LH, Woolley S, Mclaughlin P, Snowden J, Mioshi E, Roberts-South A, Benatar M, HortobaGyi T, Rosenfeld J, Silani V, Ince PG, Turner MR. Amyotrophic lateral sclerosis - frontotemporal spectrum disorder (ALS-FTSD): Revised diagnostic criteria. Amyotroph Lateral Scler Frontotemporal Degener. 2017 May;18(3-4):153-174. doi: 10.1080/21678421.2016.1267768. Epub 2017 Jan 5.

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• Strong MJ, Yang W. The frontotemporal syndromes of ALS. Clinicopathological correlates. J Mol Neurosci. 2011 Nov;45(3):648-55. doi: 10.1007/s12031-011-9609-0. Epub 2011 Aug 2.

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• Bora E. Meta-analysis of social cognition in amyotrophic lateral sclerosis. Cortex. 2017 Mar;88:1-7. doi: 10.1016/j.cortex.2016.11.012. Epub 2016 Dec 5.

  PMID: 28002755 BACKGROUND

• Martins AP, Prado LGR, Lillo P, Mioshi E, Teixeira AL, de Souza LC. Deficits in Emotion Recognition as Markers of Frontal Behavioral Dysfunction in Amyotrophic Lateral Sclerosis. J Neuropsychiatry Clin Neurosci. 2019 Spring;31(2):165-169. doi: 10.1176/appi.neuropsych.18040086. Epub 2018 Dec 12.

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• Oh SI, Oh KW, Kim HJ, Park JS, Kim SH. Impaired Perception of Emotional Expression in Amyotrophic Lateral Sclerosis. J Clin Neurol. 2016 Jul;12(3):295-300. doi: 10.3988/jcn.2016.12.3.295. Epub 2016 Apr 19.

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• Zimmerman EK, Eslinger PJ, Simmons Z, Barrett AM. Emotional perception deficits in amyotrophic lateral sclerosis. Cogn Behav Neurol. 2007 Jun;20(2):79-82. doi: 10.1097/WNN.0b013e31804c700b.

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• Lule D, Kurt A, Jurgens R, Kassubek J, Diekmann V, Kraft E, Neumann N, Ludolph AC, Birbaumer N, Anders S. Emotional responding in amyotrophic lateral sclerosis. J Neurol. 2005 Dec;252(12):1517-24. doi: 10.1007/s00415-005-0907-8. Epub 2005 Jun 24.

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• Crespi C, Cerami C, Dodich A, Canessa N, Arpone M, Iannaccone S, Corbo M, Lunetta C, Scola E, Falini A, Cappa SF. Microstructural white matter correlates of emotion recognition impairment in Amyotrophic Lateral Sclerosis. Cortex. 2014 Apr;53:1-8. doi: 10.1016/j.cortex.2014.01.002. Epub 2014 Jan 18.

  PMID: 24534360 BACKGROUND

• Lule D, Diekmann V, Anders S, Kassubek J, Kubler A, Ludolph AC, Birbaumer N. Brain responses to emotional stimuli in patients with amyotrophic lateral sclerosis (ALS). J Neurol. 2007 Apr;254(4):519-27. doi: 10.1007/s00415-006-0409-3. Epub 2007 Mar 31.

  PMID: 17401515 BACKGROUND

• Aho-Ozhan HE, Keller J, Heimrath J, Uttner I, Kassubek J, Birbaumer N, Ludolph AC, Lule D. Perception of Emotional Facial Expressions in Amyotrophic Lateral Sclerosis (ALS) at Behavioural and Brain Metabolic Level. PLoS One. 2016 Oct 14;11(10):e0164655. doi: 10.1371/journal.pone.0164655. eCollection 2016.

  PMID: 27741285 BACKGROUND

• Finegan E, Chipika RH, Li Hi Shing S, Hardiman O, Bede P. Pathological Crying and Laughing in Motor Neuron Disease: Pathobiology, Screening, Intervention. Front Neurol. 2019 Mar 21;10:260. doi: 10.3389/fneur.2019.00260. eCollection 2019.

  PMID: 30949121 BACKGROUND

• Caga J, Hsieh S, Lillo P, Dudley K, Mioshi E. The Impact of Cognitive and Behavioral Symptoms on ALS Patients and Their Caregivers. Front Neurol. 2019 Mar 11;10:192. doi: 10.3389/fneur.2019.00192. eCollection 2019.

  PMID: 30915018 BACKGROUND

• de Wit J, Bakker LA, van Groenestijn AC, van den Berg LH, Schroder CD, Visser-Meily JMA, Beelen A. Caregiver burden in amyotrophic lateral sclerosis: A systematic review. Palliat Med. 2018 Jan;32(1):231-245. doi: 10.1177/0269216317709965. Epub 2017 Jul 3.

  PMID: 28671483 BACKGROUND

• Olney RK, Murphy J, Forshew D, Garwood E, Miller BL, Langmore S, Kohn MA, Lomen-Hoerth C. The effects of executive and behavioral dysfunction on the course of ALS. Neurology. 2005 Dec 13;65(11):1774-7. doi: 10.1212/01.wnl.0000188759.87240.8b.

  PMID: 16344521 BACKGROUND

• Borasio GD, Miller RG. Clinical characteristics and management of ALS. Semin Neurol. 2001 Jun;21(2):155-66. doi: 10.1055/s-2001-15268.

  PMID: 11442324 BACKGROUND

• Benbrika S, Desgranges B, Eustache F, Viader F. Cognitive, Emotional and Psychological Manifestations in Amyotrophic Lateral Sclerosis at Baseline and Overtime: A Review. Front Neurosci. 2019 Sep 10;13:951. doi: 10.3389/fnins.2019.00951. eCollection 2019.

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• Lule D, Ehlich B, Lang D, Sorg S, Heimrath J, Kubler A, Birbaumer N, Ludolph AC. Quality of life in fatal disease: the flawed judgement of the social environment. J Neurol. 2013 Nov;260(11):2836-43. doi: 10.1007/s00415-013-7068-y. Epub 2013 Aug 30.

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• Garcia-Cordero I, Migeot J, Fittipaldi S, Aquino A, Campo CG, Garcia A, Ibanez A. Metacognition of emotion recognition across neurodegenerative diseases. Cortex. 2021 Apr;137:93-107. doi: 10.1016/j.cortex.2020.12.023. Epub 2021 Jan 28.

  PMID: 33609899 BACKGROUND

• Brooks BR, Miller RG, Swash M, Munsat TL; World Federation of Neurology Research Group on Motor Neuron Diseases. El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord. 2000 Dec;1(5):293-9. doi: 10.1080/146608200300079536. No abstract available.

  PMID: 11464847 BACKGROUND

• Wang S, Adolphs R. Reduced specificity in emotion judgment in people with autism spectrum disorder. Neuropsychologia. 2017 May;99:286-295. doi: 10.1016/j.neuropsychologia.2017.03.024. Epub 2017 Mar 24.

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MeSH Terms

Conditions

Amyotrophic Lateral Sclerosis; Neuromuscular Diseases; Motor Neuron Disease

Condition Hierarchy (Ancestors)

Spinal Cord Diseases; Central Nervous System Diseases; Nervous System Diseases; Neurodegenerative Diseases; TDP-43 Proteinopathies; Proteostasis Deficiencies; Metabolic Diseases; Nutritional and Metabolic Diseases

Study Officials

• Mia B Heintzelmann, Cand.med

  Department of Neurology, Aarhus University Hospital

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Mia B Heintzelmann, Cand.med

CONTACT

78454255; minielse@rm.dk

Camilla Hakala, Bach.psych

CONTACT

26332857; caha@cfin.au.dk

Study Design

• Study Type: observational
• Observational Model: CASE CONTROL
• Time Perspective: CROSS SECTIONAL
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Ph.d. fellow

Study Record Dates

• First Submitted: November 28, 2023
• First Posted: August 22, 2024
• Study Start: December 15, 2023
• Primary Completion (Estimated): June 30, 2026
• Study Completion (Estimated): June 30, 2026
• Last Updated: August 22, 2024

Record last verified: 2024-08

Data Sharing

• IPD Sharing: Will share
• Time Frame: After the anonymizing the data, which is set to happen after collecting the data from the last participant being tested, the data will become available. The data will be available for minimum of 3 years.
• Access Criteria: Through approval by Aarhus University Hospital or Region Midtjylland can individuals receive the collected data. The data can potentially be used as part of a masters thesis.

Locations

DK (2)