Received: 26 August 2021 Revised: 26 October 2021 Accepted: 9 November 2021

DOI: 10.1002/ajmg.c.31955

REVIEW ARTICLE

Updates on the psychological and psychiatric aspects of the

Ehlers–Danlos syndromes and hypermobility spectrum
disorders

Andrea Bulbena-Cabré1,2 | Carolina Baeza-Velasco3,4,5 |

Silvia Rosado-Figuerola6 | Antonio Bulbena6,7

1
Icahn School of Medicine at Mount Sinai,
New York, New York Abstract
2
Metropolitan Hospital, New York City Health The field of the psychiatric and psychological aspects of Ehlers–Danlos syndromes
and Hospitals, New York, New York
(EDS) has been understudied and neglected for many years. People with EDS are
3
Laboratoire de Psychopathologie et
Processus de Santé, Université de Paris, Paris, often classified as “somatizers” by untrained clinicians. However, research on the
France biological basis of EDS is improving our understanding of the physiology and psycho-
4
Department of Emergency Psychiatry and
pathology of the disorder. In this article, we consider the literature on the psycho-
Acute Care, CHU Montpellier, Montpellier,
France pathological dimensions associated with EDS as well as the EDS symptoms in
5
Institute of Functional Genomics, University psychiatric conditions since our review in 2017. Literature confirms that psychologi-
of Montpellier, CNRS, INSERM, Montpellier,
France
cal processes (i.e., fear, emotional distress, or negative emotions) in EDS have a sig-
6
Institut Neuropsychiatry and Addictions, Parc nificant impact on the outcomes of EDS. Common systemic associations are found
Salut Mar, Hospital del Mar, Barcelona, Spain between anxiety disorders and EDS as well as significant correlations with neu-
7
Department of Psychiatry and Forensic

noma Barcelona,
rodevelopmental, eating, mood, and sleep disorders. There is limited but increasing
Medicine, Universitat Auto
Barcelona, Spain evidence of an association between EDS and suicidal thoughts and behaviors, which
should be further explored. The broad spectrum of human anxiety and associated
Correspondence
Antonio Bulbena, Department of Psychiatry somatic symptoms (beyond anxiety disorders) appears to be the core of the psycho-

noma
and Forensic Medicine, Universitat Auto
pathology in EDS and therefore, detecting and assessing EDS might be a new oppor-
Barcelona, Barcelona, Spain.
Email: abulbena@gmail.com tunity for psychiatric nosology to develop more inclusive phenotypes like the
Neuroconnective Phenotype that include both somatic and psychological
manifestations.

KEYWORDS
anxiety, Ehlers–Danlos, psychiatry

1 | I N T RO DU CT I O N : FR O M SU R F A CE TO not only because of the significant stigma against mental illness but
FACTS also because of the subjectivity of many symptoms and the mis-
attributed psychological causality of them. Stigma and negative
The psychiatric and psychological aspects of Ehlers–Danlos syn- beliefs about mental health services appear as the most significant
dromes (EDS) have been neglected for many years. This neglect is barriers in seeking help (Aguirre Velasco, Cruz, Billings, Jimenez, &
Rowe, 2020). Signs and symptoms are abnormalities that can indi-

This article is an extension of a plenary guest speaker lecture presented at the EDS ECHO
cate and help to assess a medical condition. Whereas a symptom is
Conference on the Ehlers Danlos Syndromes held October 2–3, 2020. The Editors-in-Chief subjective, that is, apparent only to the patient (e.g., pain or
of this journal affirm that this article was evaluated editorially and rigorously edited by the
fatigue), a sign is any objective evidence of a disease that can be
expert Guest Editors for this issue, Dr. Hakim, Dr. Francomano, and Dr. Tinkle, and was not
anonymously peer reviewed. observed by others (e.g., a bleeding or blood pressure abnormality).

482 © 2021 Wiley Periodicals LLC. wileyonlinelibrary.com/journal/ajmgc Am J Med Genet. 2021;187C:482–490.


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To date, hypermobile EDS (hEDS) is a clinical diagnosis based on 3 | LI T E RA T U R E RE V I E W

the association of manifestations suggestive of changes in the con-
nective tissue (e.g., fragility, alterations of biomechanical character- 3.1 | Psychiatric and psychological dimensions of
istics). At present, there is no known genetic defect or other JHS and EDS
specific diagnostic tests (Tinkle et al., 2017).
Patients with EDS report a very high prevalence of subjective Most research about the psychological and psychiatric aspects of JHS
symptoms including pain (>95%), unspecific central nervous sys- and EDS focuses on evaluating psychiatric symptoms from a categori-
tem symptoms like headaches or dysautonomia (90%), profound cal or a dimensional point of view, but little is known about the psy-
fatigue (>90%), or gastrointestinal troubles like gastrointestinal chological processes and their impact on outcomes of individuals
acid reflux or gastroparesis (80%), etc. (Ferman & Hamm, 2016; living with JHS and EDS.
Hakim & Grahame, 2004). The main problem with the high subjec- A recent study by Simmonds (Simmonds et al., 2019) found that
tivity in EDS is that most of these symptoms may not have a read- pain, fatigue, and fear are common barriers to exercise and highlighted
ily identifiable organic origin, and most of these patients are often the role of therapeutic alliance and a skilled physical therapist to treat
classified as “somatizers.” This was reflected in the results of Fer- these patients. Another study by Baeza-Velasco, Bulbena, Polanco-
man & Hamm's, 2016 EDS Worldwide Survey in which close to Carrasco, and Jaussaud (2019) found that several psychological and
70% of patients confirmed that their doctors had told them state- cognitive factors affected pain chronicity and disability of hEDS.
ments like “it is all in your head” or “there is nothing wrong with These factors include cognitive problems and attention to body sensa-
you.” In fact, 97% of the participants stated that prior doctors tions, negative emotions, and unhealthy patterns of activity (hypo/
“labeled” them with a psychological condition to explain EDS hyperactivity). Along those lines, in his doctoral thesis about illness
symptoms. identity and psychological adaptation in individuals with hEDS,
However, newer research on underlying mechanisms in EDS is Heidlebaugh (2020) found unexpected relationships among the illness
helping improve the understanding of the disorder from a biological identity states, coping self-efficacy, and emotional distress in these
and molecular point of view. Greater body perception and emotional patients. This contributes to a better understanding of illness identity
reactivity, gastrointestinal (GI) intolerances, high sensitivity to bodily and psychological adaptation in individuals with hEDS or HSD which
senses, and autonomic nervous system dysfunction seem to play a may help develop more tailored psychological interventions.
particularly important role in EDS symptomatology (Bulbena Baeza-Velasco, Sinibaldi, and Castori (2018) also studied low and
et al., 2017). high anxious people with hEDS and found those with high anxious
Currently, hEDS is conceptualized as being the extreme of a con- hEDS showed greater levels of pain catastrophizing, somatosensory
tinuum (the so-called hypermobility spectrum disorders [HSD]) that amplification (SSA) as well as poor social functioning and general
ranges from isolated symptomatic single or pauci-articular joint hyper- health.
mobility to hEDS, and includes intermediate phenotypes In recent years, there has been a tendency to study the frequency
(i.e., symptomatic hypermobility that does not fulfill the criteria for of some psychiatric disorders/symptoms in EDS patients. Smith
hEDS) (Castori et al., 2017). Prior to 2017, the terms “joint hyper- et al. (2014) found that these patients often exhibit a range of symp-
mobility syndrome (JHS)” and “Ehlers-Danlos syndrome, type III toms related to anxiety and depression and have greater perceptions
(hypermobility type)” were used interchangeably by some in the litera- of fear, agoraphobia, and panic disorders. In 2016, a nationwide popu-
ture. In this review article, we will use JHS/hEDS as a generalized lation study of psychiatric disorders in patients with EDS or JHS
term to reflect this, and the term HSD or hEDS if applicable to more (Cederlöf et al., 2016) found that the relative risks for psychiatric dis-
recent studies. orders were substantial (i.e., neurodevelopmental, mood disorders, or
suicide attempts). However, one of the main limitations of this partic-
ular study is that they did not include anxiety disorders or other psy-
2 | METHODS chiatric disorders that have been widely associated with JHS and EDS,
so the interpretation of the results is limited.
Literature searches were conducted using the main electronic data- The relationship between pain and other related symptoms
bases including the Cochrane Library, Informit, PsycINFO, PubMed, (i.e., GI) and psychiatric and psychological symptoms in EDS is gaining
Google Scholar, and Scopus. The main search terms used were “joint recognition in the literature. Hershenfeld et al. (2016) found that psy-
hypermobility syndrome,” “Ehlers-Danlos,” “joint hyperlaxity,” chiatric disorders in EDS are frequent, diverse, and strongly associated
“anxiety,” and each separate psychiatric diagnostic category. Manu- with pain, particularly anxiety, and depression. Along those lines,
scripts in either English or Spanish published up to and including Wasim et al. (2019) found that pain and GI dysfunctions are signifi-
February 2021 were included if they reported any psychiatric condi- cant associations with psychiatric disorders in EDS in a retrospective
tions associated with joint hypermobility. Consensus was obtained study. They found that close to 50% of EDS patients had psychiatric
after all authors completed their contributions and reviewed each disorders, mood disorders followed by somatoform and anxiety disor-
manuscript to ensure general agreement. A total of 65 articles were ders being the most common ones. Interestingly, in the area of anxiety
included in the review. disorders, the authors only included generalized anxiety disorder and
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BULBENA-CABRE

unspecified anxiety disorder, which are not the most common anxiety depression, eating, and neurodevelopmental disorders as well as alco-
disorders associated with EDS (Bulbena et al., 2017), so it is likely that hol and tobacco misuse. We introduced the “neuroconnective
anxiety disorders were underrepresented in that study. In any case, phenotype” (Bulbena, Pailhez, Bulbena-Cabre, Mallorqui-Bague, &
the authors highlighted that the common systemic associations with Baeza-Velasco, 2015), a multidimensional approach and discussed
the presence of psychiatric manifestations in EDS reaffirm that the some therapeutic interventions. Since 2017, multiple studies have
conditions should be treated as a spectrum rather than as wholly sep- been published affording a better understanding of psychiatric disor-
arate entities, particularly with respect to psychiatric management. ders in EDS and HSD patients (see Figures 1 and 2).
Lastly, a national electronic cohort and case–control study in Wales
(Demmler et al., 2019) found that JHS and EDS were associated with
higher odds not only for musculoskeletal diagnoses but also for psy- 3.2.1 | Anxiety disorders
chiatric problems, nervous, and digestive symptoms, highlighting the
need to treat them as systemic conditions. New research has confirmed that anxiety disorders are very common
in patients with EDS (Bulbena-Cabré & Bulbena, 2018). Particularly,
several studies have shown that this association is also valid in differ-
3.2 | EDS in psychiatric conditions ent age groups, and in the presence of hypermobility per se (Eccles
et al., 2021). In pediatric populations, a study by Ezpeleta (Ezpeleta,
In 2017, our group published a review paper on the psychiatric and Navarro, Osa, Penelo, & Bulbena, 2018) found greater hypermobility
psychological aspects of EDS (Bulbena et al., 2017). We found a scores associated with greater anxiety symptoms. The authors
strong association between anxiety disorders and JHS/hEDS, and a suggested that children with frequent symptoms related to hyper-
limited but growing evidence that JHS/hEDS is also associated with mobility may benefit from screening for anxiety symptoms because a

FIGURE 1 Neuroconnective phenotype


ET AL.
BULBENA-CABRE 485

FIGURE 2 Assessment of the neuroconnective phenotype

subset of them experience clinical elevations and may need compre- In the recent years, there has been an increased interest in this
hensive physical and psychological treatment. These findings are con- modality, one study, for example, showing that patients with panic
sistent with a study by our group (Bulbena-Cabre et al., 2019) in disorder (a disorder that has been repeatedly associated with hEDS)
which we found that children with JHS have higher frequency of anxi- have lower olfactory detection threshold (i.e., higher sensitivity) along
ety disorders and greater intensity of physiological anxiety and with an enhanced reactivity to odors as well as a greater olfactory
somatic complaints and suggested that JHS might be used as a marker awareness (Buron, Bulbena, & Bulbena-Cabre, 2015). In 2018, Buron
for this anxiety phenotype in youngsters. Another group (Parvaneh (Buron, Bulbena, Bulbena-Cabre, Rosado, & Pailhez, 2018) studied
et al., 2020) also found a greater prevalence of hypermobility scores olfactory functioning in panic disorder in relation to JHS and found
in children with anxiety symptomatology. that JHS and anxiety measures emerged as predictor variables of the
Both anxiety and joint hypermobility tend to decrease with age as olfactory function.
shown by prior epidemiological studies (Reynolds, Pietrzak, El-
Gabalawy, Mackenzie, & Sareen, 2015; Wynne-Davies, 1971). Inter-
estingly, our group showed for the first time that the association 3.2.2 | Mood disorders
between JHS/EDS and anxiety is preserved in the elderly as well and
suggested that JHS could be used as a physical marker for anxiety in Major depressive disorder has also been widely associated with
this age range (Bulbena-Cabre et al., 2019). JHS/hEDS (Bulbena et al., 2017). Most of the updated data on this
Somatosensory function is the ability to interpret bodily sen- topic has been obtained from some large epidemiological studies or
sations. Arthur J. Barsky was a pioneer in this field and defined review papers as noted above (Cederlöf et al., 2016; Hershenfeld
SSA as a self-reported sensitivity to somatic and visceral sensa- et al., 2016; Smith et al., 2014). These studies evaluated several
tions, which are uncomfortable, but usually minor, and not gener- psychiatric aspects in JHS/hEDS and confirmed the relationship
ally regarded as symptomatic of serious disease (Barsky 3rd., 1979; with depressive states. Perhaps, one of the most significant
Barsky, Wyshak, & Klerman, 1990). Prior research has shown that advances in the area of mood disorders has been the study of bipo-
SSA is seen in both patients with anxiety (Freyler, Kohegyi, lar disorder in JHS/hEDS. Bulbena-Cabré et al. (2017) showed that
Koteles, Kokonyei, & Bardos, 2013; Koteles & Doering, 2016; patients with bipolar disorder and comorbid anxiety have a specific
Köteles & Simor, 2014) and those with hEDs (Bulbena-Cabré phenotype characterized by joint hypermobility, SSA, and increased
et al., 2017). body perception.
486
ET AL.
BULBENA-CABRE

3.2.3 | Neurodevelopmental disorders Knez, 2021) found a strong association between HSD or hEDS and
ADHD or ASD. They recommend routine screening for neuropsy-
Considerable interest has arisen about the relationship between chiatric symptoms in people with HSD or hEDS. Similarly, Baeza-
neurodevelopmental disorders such as autism spectrum disorders Velasco (Baeza-Velasco et al., 2018) conducted a review study and
(ASD) or attention deficit hyperactivity disorder (ADHD) since our found a significant association between ADHD and JHS and
review paper in 2017 (Bulbena et al., 2017). In ASD, this is not sur- hypothesized that their association may testify for the dyadic
prising as current clinical descriptions of children with autism nature of mind–body connections during critical periods of post-
include hypotonia, joint laxity/hypermobility, clumsiness, apraxia, natal development.
and toe walking as common findings. Baeza-Velasco et al. (2018)
reviewed the relationship between joint hypermobility, ASD, and
chronic pain, especially since the link between ASD and 3.2.4 | Eating disorders
hypermobility-related disorders (especially hEDS) theoretically
implies susceptibility to a wide range of pain (mainly musculoskele- Several studies have found a connection between JHS, EDS, and GI
tal but also headaches and visceral pain). The authors concluded problems which at the same time are associated with eating, nutrition,
that given the high probability that pain remains disregarded and and weight problems (Bulbena-Cabré et al., 2017; Castori, Morlino,
untreated in people with ASD due to communication and method- Pascolini, Blundo, & Grammatico, 2015; Lam et al., 2021; Nelson
ological difficulties, increasing awareness about the interconnec- et al., 2015; Wasim et al., 2019). These are probably related to com-
tion between ASD and hypermobility-related disorders is relevant, orbidities seen in HSD and EDS such as GI symptoms and food aller-
since it may help identify those ASD patients susceptible to gies which are also frequent in these patients, and Baeza-Velasco
chronic pain. Casanova et al. (2019) published a cohort study in proposed a model of eating disorders suggesting that several intra-
which they studied adult women with ASD with and without gen- and extra-articular features such as GI problems, temporomandibular
eralized joint hypermobility (GJH) and estimated the prevalence of joint disorders, and food intolerance could contribute to developing
immune and endocrine mediated conditions. They found that the and maintaining disturbed eating patterns and significant weight loss
hypermobile ASD group presented significantly higher rates of in JHS/hEDS (Baeza-Velasco, Van den Bossche, Grossin, &
autoimmune disorders (45% vs. 13%; p = .02), but also pain- Hamonet, 2016). The same group recently conducted a cross-
associated endocrine symptomatology such as dysmenorrhea and sectional study (Baeza-Velasco et al., 2021) comparing GI symptoms
endometriosis (85% vs. 28%; p < .001 and 30% vs. 5%; p = .01 and eating problems in EDS and found greater frequencies of GI
respectively), suggesting a potential system crosstalk which rein- symptoms in EDS and food allergies/intolerances.
forces the etiological relationship between ASD and GJH. In 2019, Another author (Santonicola et al., 2019) found that abnormal
they carried out a case–control study (Casanova et al., 2019) and GI function can induce certain symptoms of disordered eating like
evaluated immune, autonomic, and endocrine dysregulation in loss of appetite, self-induced vomiting, dysphagia, constipation, and
ASD with JHS/hEDS compared to healthy controls and found a bloating, which may be diagnosed as eating disorders like anorexia
significant overlap immune/autonomic/endocrine dysregulation, or bulimia. However, it is important to note that current models of
pain, and some tissue fragility, which is typically more severe in eating disorders emphasize body image rather than the digestion
the former. In 2020, they published a thorough review paper process and body perception like interoceptive processes, which is
(Casanova, Baeza-Velasco, Buchanan, & Casanova, 2020) about of great importance in JHS/hEDS. In this sense, the newly
the current state of knowledge on the relationship between ASD described “Avoidant/restrictive food intake disorder (ARFID)” is
and EDS. They concluded that there are significant neuro- characterized by a persistent failure to meet appropriate nutritional
behavioral, psychiatric, and neurological commonalities, shared and/or energy needs, which can result in significant weight loss or
peripheral neuropathies, and similar autonomic and immune dys- nutritional deficiency, dependence on enteral feeding or nutritional
regulation. Considering these findings, the authors suggest that supplements, and/or a marked interference in psychosocial func-
JHS/hEDS may represent a subtype of autism. tioning may shed some light about eating disorders in EDS. Little is
Along those lines, Csecs et al. (2020) tested for increased rates known about the psychopathology associated with ARFID, but
of joint hypermobility, autonomic dysfunction, and pain in there are some data showing that these patients suffer from greater
109 adults with neurodevelopmental diagnoses including ASD, somatic concerns compared to anorexia/bulimia and are more likely
ADHD, and tic disorder. They found neurodevelopmental patients to have comorbid anxiety but not depression (Canas et al., 2020;
manifest elevated rates of joint hypermobility compared to controls Fisher et al., 2014). Work is underway studying JHS/hEDS features
and they also report significantly more symptoms of orthostatic in ARFID populations and preliminary findings suggest that children
intolerance and musculoskeletal skeletal pain than controls. They with ARFID have greater JHS/hEDS features including SSA com-
concluded that there is a strong link between the expression of pared to controls, which may open new therapeutic avenues
joint hypermobility, autonomic dysfunction, and pain and targeting body perception (instead of body image) and digestion
highlighted the need for increased awareness to appropriately treat difficulties along with other JHS related features in these
those conditions. Similarly, Kindgren (Kindgren, Perez, & populations (Authors' communication).

ET AL.
BULBENA-CABRE 487

3.2.5 | Sleep disorders personality disturbances and anxiety disorders have been identified
(Baeza-Velasco, Hamonet, Montalescot, & Courtet, 2021).
Fatigue, sleep disturbances, and daytime sleepiness are clinical prob-
lems frequently reported by patients with JHS and EDS (Verbraecken,
Declerck, Van de Heyning, De Backer, & Wouters, 2001; Voermans 3.2.7 | Other conditions and perspectives
et al., 2010). Genetic cartilage defects, craniofacial abnormalities, and
increased pharyngeal collapsibility due to tissue flaccidity have been Of particular interest is the presence of hEDS/anxiety phenotype in
proposed to cause obstructive sleep apnea (OSA) in EDS patients schizophrenia and psychotic disorders, an area that has not been
(Guilleminault et al., 2013). Gaisl found that the prevalence of OSA is widely explored (Bulbena-Cabre & Bulbena, 2018). Other fields for
higher in patients with EDS compared to controls and concluded that exploration are conversion disorders renamed as neurological func-
this is clinically relevant due to the associated fatigue and impaired tional disorders in which hEDS is very often found (Barnum, 2014), as
quality of life (Gaisl et al., 2017). These findings are in line with well as the high association with fibromyalgia (Eccles et al., 2021). Of
another study (Muriello et al., 2018) that found that both pain and note, a recent paper (Aslanyan, Iodice, Davies, Critchley, &
poor sleep quality negatively impacted the quality of life of these Eccles, 2019) found that dissociative experiences in fibromyalgia are
patients. Stoberl et al. (2019) studied OSA in children and adolescents mediated by autonomic dysfunction, a very important feature of
with EDS and found an estimated prevalence of 42% and concluded JHS/hEDS (Hakim, De Wandele, O'Callaghan, Pocinki, & Rowe, 2017)
that OSA is highly prevalent in children with EDS but is under- so it may be relevant to further study the connection between these
estimated which increased the disease burden. Domany and his group conditions.
(Domany et al., 2018) also studied sleep disorder in pediatric Perhaps, one of the most significant findings is the association
populations with EDS and not surprisingly, results showed a high between joint hypermobility and emotional excitability in non-human
prevalence of OSA but also insomnia, periodic limb movement disor- species (Bowen et al., 2019). Our group confirmed for the first time
der, and hypersomnia in EDS children. Lastly, a systematic review that emotional excitability is correlated with joint hypermobility in
paper and meta-analysis (Sedky, Gaisl, & Bennett, 2019) found that dogs, which suggests that this association may be a positive evolu-
OSA is a high prevalent, previously underestimated EDS/Marfan tionary trait. If it was just an abnormality or a negative trait, evolution
syndrome-related complication that might be the result of bony and would have erased it.
soft-tissue abnormalities associated with these hypermobility In addition, recent biological trends on human fibroblasts may
syndromes. help to understand the relationship between hEDS and neuropsy-
Narcolepsy, a disorder characterized by excessive daytime sleepi- chiatry (Chiarelli et al., 2021; Mesdom et al., 2020). Nevertheless,
ness, involuntary sleep attacks due to rapid eye movement intrusion, clinical phenotypes particularly in psychiatry, need refurbishing
sleep paralysis, cataplexy, and hypnagogic or hypnopompic hallucina- beyond present nosologies which are mostly based on clinical
tions, has also been associated with EDS in a case series paper observations rather than neurobiological substrates. Along these
(Miliken & Sedky, 2019). However, the evidence behind this associa- lines, the Neuroconnective Model Phenotype (Bulbena et al., 2017)
tion is sporadic and limited and further studies should confirm this (Figures 1 and 2) might shed new light for a more comprehensive
association. and neurobiologically oriented psychopathology including somatic
and mental dimensions.

3.2.6 | Suicide and associated behaviors

4 | CONC LU SIONS
Suicidal ideation or behavior has been described in patients with EDS
(Benistan & Martinez, 2019; Cederlöf et al., 2016). It is interesting to There is significant and ever-growing evidence that psychiatric and
note that in the study by Benistan and Martinez (2019), a high rate of psychological aspects of health are relevant to JHS and EDS. How-
suicide attempts (22%) was found among young EDS patients. The ever, stigma, negative beliefs, and ignorance have hampered its clinical
authors did not study the prevalence of borderline personality disor- salience. The subjective nature of many of the symptoms in JHS and
der which is a risk for suicide (Soloff, Lynch, Kelly, Malone, & EDS prompts clinicians to make premature psychological mis-
Mann, 2000). Exploring whether this disorder is present is important attribution of them, which in turn leads to rejection of such diagnoses
as it responds well to dialectical behavior therapy as opposed to phar- by patients.
macotherapy. The same authors also hypothesized that this high rate The broad spectrum of human anxiety and associated somatic
of suicide attempts could be related to the distress experienced by symptoms (beyond anxiety disorders) appears to be the core of the
EDS patients before the diagnosis, which is usually highly delayed psychopathology, and thus, mood disorders, eating disorders, and
(10–14 years). Interestingly, Cederlöf (Cederlöf et al., 2016) found a neurodevelopment disorders show a surprising overlap with this con-
protective effect of early EDS diagnosis (i.e., before 19 years old) on nective tissue syndrome, which therefore extends its pathophysiology
later suicide attempt. Among the factors explored as increasing the beyond structural tissues, highlighting the importance of the psychiat-
probability of having suicidal behaviors in patients with hEDS, ric aspects of the disorder. Evidence confirms that sleep and eating
488
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BULBENA-CABRE

disorders as well as suicidal behaviors are common in these patient Baeza-Velasco, C., Van den Bossche, T., Grossin, D., & Hamonet, C. (2016).
groups. Difficulty eating and significant weight loss in joint hypermobility syn-
drome/Ehlers-Danlos syndrome, hypermobility type. Eating and
Nevertheless, JHS (now HSD and hEDS) appears to be even more
Weight Disorders, 21(2), 175–183. https://doi.org/10.1007/s40519-
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assessing hypermobility-related disorders might be a new opportunity response to variable and unusual symptoms. Adolescent Health, Medi-
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DATA AVAI LAB ILITY S TATEMENT Critchley, H., Chopra, P., … Porges, S. (2017). Psychiatric and psycho-
Data sharing not applicable to this article as no datasets were gener- logical aspects in the Ehlers-Danlos syndromes. American Journal of
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ORCID Velasco, C. (2015). Joint hypermobility, anxiety and psychosomatics:
Andrea Bulbena-Cabré https://orcid.org/0000-0001-7070-2014 Two and a half decades of progress toward a new phenotype.
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1159/000369113
Antonio Bulbena https://orcid.org/0000-0003-2404-2478
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Aguirre Velasco, A., Cruz, I. S. S., Billings, J., Jimenez, M., & Rowe, S. they are relatives not just neighbours. The British Journal of Psychiatry,
(2020). What are the barriers, facilitators and interventions targeting 213(2), 498. https://doi.org/10.1192/bjp.2018.126
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