The Social-HD Study: A Cross-Sectional Observational Study of Social Cognition in Early Huntington Disease
Conditions: Huntington Disease
Sponsor: Region Skane

 
1 
 
 
 
Study protocol for 
The Social-HD Study: A Cross-Sectional Observational Study of Social 
Cognition in Early Huntington Disease 
 
 
 
 
2025-04424-01 
 
 
Date of documentation: August 13, 2025 
 
 

 
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Study protocol for The Social-HD study 
 
Background  
Huntington disease (HD) is a fatal neurodegenerative disorder without any disease-
modifying therapies available. It affects 1 in 10.000 individuals with around 1000 
people affected in Sweden (Furby et al., 2023). It is caused by an expanded CAG 
repeat in the huntingtin (HTT) gene which is inherited in an autosomal dominant 
fashion with full penetrance. It typically manifests at 30-50 years of age. Clinical 
diagnosis is based on overt movement disorder with chorea and a genetic test. The 
disease then progresses leading to premature death around 15 years after onset 
(Furby et al., 2023). HD is further characterized by cognitive deficits and psychiatric 
symptoms, which frequently emerge many years before any observable signs of 
motor dysfunction (Epping et al., 2016; Stout et al, 2011). The cognitive symptoms 
include executive dysfunction, reduced attention and reduced psychomotor speed, 
and eventually leads to a state of dementia (Peavy et al., 2010). Commonly reported 
psychiatric symptoms include depression, apathy, and irritability (Clark et al., 2023; 
Karagas, Rocha & Stimming, 2020). These non-motor symptoms are assumed to be 
a significant contributor to patient and informal caregiver burden (Dawson, 
Kristjanson, Toye & Flett, 2004; Eddy & Rickards, 2022; Mason et al., 2024). 
 
Families with HD often describe a personality change and social functioning to be 
the first noticeable change in a person with HD. These changes may partly be 
related to the movement disorder, psychiatric symptoms, and general cognitive 
impairment. In the past few decades however, there has been an increasing 
awareness of how specific deficits in social cognition affects persons with HD (Bora, 
Velakoulis & Walterfang, 2016; Considine et al., 2024; Escudero-Cabarcas et al., 
2024), possibly caused by the effects of mutant HTT gene. Social cognition is a 
multidimensional construct which is usually taken to include social perception, 
emotion recognition, theory of mind (the ability to attribute mental states such as 
feelings, thoughts, or intentions of others) and social behavior (Christidi et al., 2018). 
Being able to recognize, process, interpret and appropriately react to social signals is 
essential for humans when forming and navigating social relationships across the 
lifespan. Consequently, deficits in social cognition may have substantial negative 
effects on the persons’ well-being, beyond psychiatric symptoms and general 
cognitive decline, due to altered relationships and difficulties at work. Despite 
increased awareness of the impact that social cognition may have on patients’ 
quality of life, neuropsychological evaluation in routine clinical practice typically does 
not include socio-cognitive tests (McDonald, Wearne & Kelly, 2023; Quesque et al., 
2024; Rabin, Paolillo & Barr, 2016).  
 
Study rationale 
Increased interest in tests that reliably measure social cognitive deficits in HD has 
led researchers to investigate the several different tests; and among these, Reading 
the Mind in the Eyes Test (RMET) (Baron-Cohen et al., 2001) is the most widely 
used measure of social cognition in HD according to a recent literature review 
(Escudero-Cabarcas et al., 2024). A related example is The Emotion Hexagon Test 
(EHT) (Sprengelmeyer et al., 1996), which has been validated in a HD population 
(Larsen et al., 2016). RMET and EHT share similar features, since both are relatively 
brief in administration time; and both require the examinee to evaluate the emotional 
states based on still pictures of human faces. One assumed weakness of this type of 

 
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test, however, is the limited ability of inferring socio-cognitive deficits based on test 
performance from static images alone (Henry, Cowan, Lee, & Sachdev, 2015). 
To counter the above limitation, the development and usage of naturalistic and multi-
modal tests that assess socio-cognitive functioning has increasingly been advocated 
in recent scientific literature (Msika, Despres, Piolino & Narme, 2024; Osborne-
Crowley, 2020), including video-based or even interactive tasks. In this context, The 
Movie for the Assessment of Social Cognition (MASC), aims to sensitively 
capture the multidimensionality of the construct to a greater degree (Dziobek et al., 
2006). It is a promising candidate for several reasons, not least due to its video-
based format. The test has been utilized in addressing deficits in social cognition in 
several patient groups, including high-functioning autism, schizophrenia, bipolar 
disorder borderline personality disorder, and multiple sclerosis (Dziobek et al., 2006; 
Montag et al., 2010a; Montag et al., 2010b; Preißler et al., 2010; Pöttgen et al., 
2013). In a recent review of tests of social cognition in a neuropsychology setting, 
Msika et al. (2024) suggests MASC as a relevant task for assessing theory of mind 
(ToM) in clinical practice, mainly because of its psychometric properties, ecological 
validity, and the availability of norms. However, to the best of our knowledge, no 
research has yet been conducted to determine its usefulness in the case of HD 
patients. 
The central aim of the present study is to explore clinical utility of MASC (or 
rather, its Swedish translation, DMASC-MC) in a population of HD patients, 
with the purpose of developing a more comprehensive socio-cognitive test 
battery in the context of clinical practice. Our assumption is that a broader 
assortment of measures for social cognition would allow the neuropsychologist to 
tailor the assessment more flexibly and in turn provide in-depth analysis of socio-
cognitive functions. We further assume that this would ultimately benefit both 
patients and their social relations, for mainly two reasons. Firstly, after including tests 
of social cognition in the evaluation, a feedback-session with the patient and close 
relatives has the potential for therapeutic gains due to increased insight of any 
identified problems (cf. Waldron-Perrine, Rai & Chao, 2021). Secondly, there is a 
growing literature on treatment approaches aiming at remediation of socio-cognitive 
deficits (McDonald, Wearne & Kelly, 2023); and by appropriately assessing for such 
impairments, clinicians set the stage for informed recommendations on how to best 
alleviate or cope with them.  
 
Potential risks and benefits 
Partaking in neuropsychological evaluation is generally assumed to be of value for 
the examinees and their close ones, since it allows for greater insight and 
understanding of personal cognitive strengths and weaknesses. For people who 
suffer from a disease such as HD, this insight alone might in turn lead to a decrease 
in symptom burden as well as increased quality of life. At the same time, engaging in 
cognitive testing is typically mentally taxing, which for some people could be 
experienced as stressful or frustrating. Furthermore, test results indicating deficits 
could also be cause for emotional distress and further questions regarding how the 
disease or other factors may have impacted that examinee as a person. To alleviate 
the above risks, all subjects of the present study will be given the chance to reflect 
on any distressing thoughts or feelings during a feedback session with a 
psychologist during a clinical visit or via telephone. The feedback session will be 

 
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tailored specifically to each subject’s needs and includes the possibility to ask further 
questions and seek advice or emotional support. An additional feedback session with 
further support and guidance will be offered if deemed necessary either by the 
psychologist or the test subject. 
  
Beyond benefits for the individual study participants, the goal of the present study is 
to further the general understanding of socio-cognitive assessment as well as socio-
cognitive deficits in HD. This in turn is taken to increase the potential for optimal care 
of patients that suffer from impairments in this domain. Since the evaluations are 
administered during working hours, each study participant will have to consider the 
possible drawbacks of refraining from work or other personal matters. 
 
Patient and public involvement 
In the preparation for the Social-HD study, we invited patient organizations 
(Riksförbundet för Huntingtons sjukdom; https://huntington.se and YTAN- digital stöd 
för unga anhöriga; https://ytanforunga.se) and through them, persons in families with 
HD for patient and public involvement in designing the study. This was done during 
two separate meetings totaling approximately two hours that were held on the 7th of 
April and the 14th of May in 2025. During the meetings, the participants were given a 
presentation of the study background and purpose, as well as an overview of the 
study design and procedures. The participants were asked to give their spontaneous 
reactions and asks questions after the initial presentation. Further, we asked them 
questions prepared by us in advance, regarding the purpose of the study; inclusion 
and exclusion criteria; the time schedule for the study; and the proposed procedure 
for feedback to research participants. The feed-back from these sessions has been 
instrumental in the design of the study.  
 
Study design/ Schedule of assessments 
The study will take place at one center, i.e. the HD clinic at the Neurology clinic at 
Skane University hospital in Lund, Sweden. The study will begin in 2025 and the 
recruitment period will last into 2026. The assessments in this study are clinician-
reported outcome (ClinRO) and performance outcome (PerfO) instruments. 
 
Assessments (in the order they will be used):  
 
Montreal Cognitive Assessment (MOCA) (PerfO) 
The Montreal Cognitive Assessment (MoCA; Nasreddine et al., 2005) is a cognitive 
screening tool that can detect cognitive impairment in HD (Videnovic et al., 2010; 
Ringkøbing et al., 2020). It contains a series of basic assessments, including 
attention and visuospatial tasks. The total score ranges from 0 to 30, where lower 
scores indicate greater impairment. It takes approximately 10 minutes to administer. 
 
Total Functional Capacity Scale (TFC) (ClinRO) 
The Total Functional Capacity Scale (TFC) is a validated measure of global patient 
function in HD (Huntington Study Group, 1996). The TFC represents the 
Investigator's assessment of the participant's capacity to perform a range of activities 
of basic daily living, including working, chores, managing finances, eating, dressing, 
and bathing. The five-item assessment is based on a brief interview with the 
participant. The TFC score ranges from 0 to 13, with a higher score representing 
better functioning. The TFC takes approximately 10 minutes to administer. 

 
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Total motor score (TMS) (ClinRO) 
The UHDRS Motor Assessment contains the total motor score (TMS) which is a 
measure of motor function in HD (Huntington Study Group, 1996). The TMS score is 
the sum of the individual motor ratings obtained from administration of the 31-item 
motor assessment portion of the UHDRS by the Investigator. The score ranges from 
0 to 124, with a higher score representing more severe impairment. It takes 
approximately 15 minutes to administer. 
 
Montgomery-Åsberg Depression Rating Scale (MADRS) (ClinRO) 
MADRS (Montgomery & Åsberg, 1979) is a widely used rating-scale designed to 
assess depressive symptoms. It exists both as a clinician rated version, and as a 
self-report measure, MADRS-S (Svanborg & Åsberg, 1994). The clinician version 
alone will be used for the present study; it comprises 10 items rated on a 7-point 
Likert scale (0-6), which are added for a total score (range 0-60). 
 
Apathy Evaluation Scale (AES) (ClinRO/(PerfO) 
The Apathy Evaluation Scale (AES) (Marin, Biedrzycki & Firinciogullari (1991) is a 
wide-spread measure designed to capture symptoms of apathy due to brain-related 
pathology. It exists both as a self-report measure (AES-S) and informant 
questionnaire (AES-I), with 18 items covering cognitive, affective, and behavioral 
aspects of apathy, as experienced by the informants for the past four weeks. It has 
been shown that self-rated and companion-rated apathy is highly correlated in HD 
(Mason and Barker, 2015). A clinician version (AES-C) also exists, wherein a health 
professional rates the same items based on the patient’s behavior and information 
given during an interview. During administration, the rater is asked to answer each 
statement on a 4-point scale (0-3). Total score can vary between 0 and 72 points; for 
the Swedish translation (Johansson et al, 2017) a score of >36,5 has been 
suggested as a preliminary cut-off for apathy. For the present study, AES-S and 
AES-C will be administered. AES takes approximately 10-20 minutes to administer. 
 
Symbol digit modalities test (SDMT) (PerfO) 
The SDMT is used to assess attention, visuoperceptual processing, working 
memory, and psychomotor speed. It has been shown to have strong reliability and 
validity (Smith, 1982). The participant pairs abstract symbols with specific numbers 
according to a translation key. The test measures the number of items correctly 
paired (maximum of 110 correct pairs) in 90 seconds. The oral form of the test (used 
in the current study) allows for valid assessment of processing speed with minimal 
impact of motor deficits. It takes around 5 minutes to administer.  
 
D-KEFS Color Word Interference Test (CWIT) (PerfO) 
The CWIT is part of the Delis-Kaplan Executive function system (Delis, Kaplan & 
Kramer, 2001), based on the Stroop (1935) procedure. CWIT consists of four 
separate subtests, which include measures of speed of verbal output, verbal 
inhibition, and cognitive flexibility. The first two conditions serve as baseline 
measures and simply require the examinee to name colors or words (i.e. “RED”, 
“BLUE”, “GREEN”) on a printed page. For the third condition, words are printed in 
dissonant ink, and requires the examinee to inhibit an overlearned verbal response 
by naming the color of the print (rather than reading the words). The fourth condition 
introduces a measure of cognitive flexibility by asking the examinee to switch 

 
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between naming the dissonant ink colors and reading the words. The score is 
calculated from the amount of time taken for the examinee to finish each of the four 
tasks. The test takes approximately 15 minutes to administer. 
 
D-KEFS Verbal Fluency Test (VFT) (PerfO) 
The VFT is part of the Delis-Kaplan Executive function system and is based on other 
similar and often used measures of verbal fluency (Delis, Kaplan & Kramer, 2001). 
The test includes three similar conditions, all of which were designed to measure 
spontaneous word-production according to a certain set of rules. The conditions 
used in the present study (Phonemic fluency and Category switching) requires the 
examinee to orally produce as many words as possible for one minute. Further, 
these words must either begin with certain specified letters (phonemic fluency) or 
alternate between specific categories (category switching). The score is calculated 
from the amount the words that the examinee can produce; as well as his/her ability 
to conform to a few specified rules. The test takes approximately 5 minutes to 
administer. 
 
The Movie for the Assessment of Social Cognition (MASC) (PerfO) 
MASC (translated and published as in Sweden DMASC-MC) is a video-based test 
for the evaluation social cognition and “mentalization” (a construct that is closely 
related, or even synonymous, to ToM) (Dziobek et al., 2006; Bölte, Fleck & Dziobek, 
2014). The test presents the examinee with a 15-minute film which portrays four 
individuals, played by professional actors, during a dinner-party. The film pauses at 
45 different instances, whereupon the examinee is prompted to answer multiple-
choice questions about the thoughts and feelings of these individuals. The result is 
calculated as a total score and can be further analyzed using different subscales. It 
takes approximately 45 minutes to administer.  
 
Reading the Mind in the Eyes Test (RMET) (PerfO) 
RMET is used to measure socio-cognitive function in terms of ToM (Baron-Cohen et 
al., 2001). The test presents examinees with photographs of the eye-region of 36 
different individuals. For each photograph, the examinee is asked to choose which 
mental state best describes that of the person pictured (i.e. what he or she might be 
thinking or feeling). Each item provides a set of four possible answers (multiple-
choice), and the examinee is allowed to refer to a vocabulary list where the literal 
meanings of these answers are defined. The result of the test is the total amount of 
correct responses, excluding one practice item. It takes approximately 15 minutes to 
administer.  
 
Emotion Hexagon (EHT) (PerfO) 
The EHT, originally developed by Sprengelmeyer et al. (1996) includes 30 images of 
the same man, with morphed facial expressions along a spectrum of different basic 
emotions, is a measure emotion recognition. For the present study, an adapted 
Danish-language paper version (Vogel, Jørgensen & Larsen, 2020) consisting of 
printed cards was translated to Swedish with the permission from the original 
developers. The examinee is asked to choose which of six basic emotions (a wordlist 
is provided) best corresponds to each photograph. The test can be given in two 
separate ways: either as a quick screening, or a more in-depth administration. The 
result is calculated by adding up the total amount of correct answers, excluding the 
first practice item. Administration of the screening takes approximately 15 minutes.  

 
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Tests to evaluate HD stage  
The following tests have been chosen as they have shown to measure disease stage 
and progression in clinical trials: TFC, TMS, SDMT, CWIT (Boareto et al., 2025; 
Tabrizi et al., 2022). In HD it is thought that both onset and progression are driven by 
cumulative exposure to the effects of mutant (or CAG expanded) huntingtin (mHTT). 
The CAG-Age-Product (CAP) score (i.e., the product of excess CAG length and age) 
is a commonly used measure of this cumulative exposure and will be used in this 
project (Warner et al., 2022). 
 
 
 
 
 
 
 

 
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Study objectives  
The primary objective and corresponding endpoint are provided in Table 1. 
 
Table 1. Primary objective and corresponding endpoint 
Objective 
Endpoint 
To investigate if persons with HD have 
a significantly lower score than a control 
group in the test “The Movie for the 
Assessment of Social Cognition” 
(MASC) 
Significant difference in mean value of 
MASC total score between HD and 
controls 
 
The secondary objectives and corresponding endpoints are provided in Table 2.  
 
Table 2. Secondary objectives and corresponding endpoints 
 
Objective 
Endpoint 
To investigate if persons with HD have 
a significantly lower score than a control 
group in the Reading the Mind in the 
Eyes Test (RMET) test. 
 
Significant difference in mean value of 
RMET total score between HD and 
controls 
To evaluate if persons with HD have a 
significantly lower score than a control 
group in the Emotional Hexagon (EH). 
 
Significant difference in mean value of 
EH total score between HD and controls 
To investigate if there is a correlation 
between social cognitive test scores 
and MOCA scores (= cognitive scores).  
 
Significant correlation between social 
cognitive test scores and MOCA scores 
To investigate if there is a correlation 
between social cognitive test scores 
and TFC scores (=scores for everyday 
life function in HD). 
 
Significant correlation between social 
cognitive test scores and TFC scores 
To investigate if there is a correlation 
between social cognitive test scores 
and CAP scores (=scores for disease 
burden). 
 
Significant correlation between social 
cognitive test scores and CAP scores 
To investigate if there is a correlation 
between social cognitive test scores 
and TMS scores (= motor scores). 
 
Significant correlation between social 
cognitive test scores and TMS scores 
To investigate if there is a correlation 
between social cognitive test scores 
and MADRS scores (= depression 
scores). 
 
Significant correlation between social 
cognitive test scores and MADRS 
scores 

 
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To investigate if there is a correlation 
between social cognitive test scores 
and AES scores (= apathy scores). 
 
Significant correlation between social 
cognitive test scores and apathy scores 
To investigate if there is a correlation 
between social cognitive test scores 
and SDMT/CWIT/VFT scores (= 
cognitive scores). 
 
Significant correlation between social 
cognitive test scores and cognitive 
scores 
To investigate the proportion of both HD 
subjects and control subjects with 
impaired results on each of the social 
cognitive tests, compared to norms. 
Percentage of subjects within the HD group 
performing below 1,5 SD compared to norm 
data on DMASC-MC, RMET and EHT, 
compared to prior norm data. Percentage of 
subjects within the control group performing 
below 1,5 SD compared to norm data on 
DMASC-MC, RMET and EHT. 
 
 
 
 
 
 

 
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Overview of the research plan  
 
 
Consent/Screening 
Research: 1 
Research: 2 
Follow-up 
Presentation of results 
Visit 1 
MD (A+B) 
 
 
 
 
Visit 2 
 
MD (C1) 
Psychologist 
(C2) 
 
 
Visit 3 
or Phone  
 
 
 
Psychologist 
(D) 
 
Meeting  
 
 
 
 
MD, Psychologist (E) 
 
 
A.Information about the research project including inclusions/exclusion 
criteria orally and written, informed consent 
 
B. Screening:  
MOCA test  
Checklist of inclusion/exclusion criteria 
 
C. Research visit  
1. Psychiatrist, duration 1,5 h  
1.1: Information: 
Age: 
Sex: 
Education:  
CAG repeat: 
Age of diagnosis: 
Medication: 
 
1.2:Tests:  
TFC: General function 
TMS: Motor symptoms 
MADRS: Depression 
AES: Symptoms of apathy (patient + clinician: AES-S + AES-C) 
 
1 h break  
 
2. Psychologist, duration 2h  
2.1. Short cognitive battery similar to the global observational study Enroll-HD: 
SDMT, CWIT, VFT 
 
2.2. DMASC-MC 
 
2.3 EHT and RMET 
 
D. Follow-up visit or phone call by psychologist within 3 weeks 
 
E. Information evening about the results for all participants and the HD 
community 
 
 
 

 
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Study population 
 
Inclusion criteria 
Participants are eligible to be included in the study if all the inclusion criteria are met:  
 
Inclusion criteria for persons with HD  
1. Clinical diagnosis of HD  
2. CAG repeat: 39 and more 
3. Age:18-75 years  
 
Inclusion criteria for control group 
1. No heritage of HD or negative pre-symptomatic HD gene test  
2. Age:18-75 years  
 
Exclusion criteria  
Participants in both groups are excluded from the study if any of the following criteria 
apply: 
1. Dementia or MOCA<19, MMSE <19 
2. Other neurological disorders 
3. Ongoing psychosis 
4. Ongoing alcohol/drug addiction 
5. Other native language than Swedish 
6. Severe problems with vision and hearing 
 
Withdrawal 
The participant can withdraw from the study at any time-point.  
 
Screen failures 
Screen failures are defined as participants who consent to participate in the study 
but then do not meet inclusion criteria or where exclusion criteria apply. The primary 
investigator will maintain a screening log to record details of all participants screened 
and to confirm eligibility or record reasons for screening failure. 
 
Recruitment Procedures 
Recruitment of participants will be made at the HD clinic in Lund, Sweden and 
through ethical review board-approved advertisment material posted at social media 
platforms and websites hosted by the Huntingtoncenter at Lund and the Swedish 
patient organization Riksförbundet för Huntingtons sjukdom (RHS) as well as YTAN.  
 
Statistical analysis plan 
All statistical analyses will be performed using SPSS. The primary outcome 
measure, DMASC-MC total score, of the patient group will be compared to that of 
the control group using independent samples t-test. Assuming a difference of group 
means of at least one standard deviation (1 SD), we calculated the number of 
participants needed for 80% power to be 20 per group (40 in total), based on a 5% 
significance level. 
For secondary outcome measures, RMET total score and EHT total score, of 
the patient group will be compared to those of the control group using independent 
samples t-test. Additionally, we plan to assess correlations using Spearman testing 
between the above measures and the following measures: TFC score (everyday life 

 
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function); CAP scores (disease burden); TMS scores (motor scores); MADRS and 
AES scores (psychiatric symptoms); and MOCA/SDMT/CWIT/VFT scores (= 
cognitive scores). For calculating the proportion of impaired results on social 
cognitive tests for both HD and control subjects, norm data based on healthy 
subjects will be used from Bölte, Fleck & Dziobek (2014) (DMASC-MC); Söderstrand 
& Almkvist (2012) (RMET); and Vogel, Jørgensen & Larsen (2020) (EHT). 
 
If the underlying assumptions for t-test are violated the groups will be compared 
using Mann-Whitney U-test.  
 
Data handling and monitoring 
Data will be pseudonymized and source data will be stored in folders in fire safe 
lockers that only investigators in the study will have access to. Data entry in files will 
be made by the investigators and monitored by a research nurse. Data will be 
securely stored in password-protected folders in the computer system at Region 
Skåne. 
 
Ethical and regulatory considerations 
Research will be conducted in accordance with the declaration of Helsinki and 
GDPR regulations. Investigators are trained in GCP, MOCA and TMS.  
The study has approval from the Swedish Ethics Review Board (Dnr 2025-04424-01) 
 
Presentation and publication of the results  
The data will be published in peer-reviewed scientific journals and at scientific 
conferences for HD and related disorders, as well as at meetings for the HD 
community.  
 
Investigators at Region Skåne 
PI: Professor Åsa Petersén, MD PhD, Senior Consultant in Psychiatry, Lund 
university and Region Skåne 
Co-PI: Psychologist Isak Halling, Region Skåne 
Co-PI: Associate Professor Håkan Widner, MD PhD, Senior Consultant in 
Neurology, Region Skåne 
Nurses: Camilla Svensson (Neurology) and Susann Schrey (Psychiatry), Region 
Skåne. 
 
 
 
 
 
 
 
 
 
 
 
 
 
 

 
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