SUPPLEMENTARY MATERIAL A: SCORING SYSTEMS FOR ASSESSMENT OF GJH

Beighton Score (EDS Society, 2017)/Beighton and Horan Joint Hypermobility Index
(BHJMI) (Boyle et al., (2003)
Test Description Image
A. 5th FINGER/’PINKIES’.
Test both sides: Rest palm of the hand and forearm a flat surface with palm side down and
fingers out straight. Can the fifth finger be bent/lifted upwards at the knuckle to go back
beyond 90 degrees? If yes, add one point for each hand.

B. THUMBS

Test both sides: With the arm out straight, the palm facing down, and the wrist then fully
bent downward, can the thumb be pushed back to touch the forearm? If yes, add one point
for each thumb.

C. ELBOWS

Test both sides: With arms outstretched and palms facing upwards, does the elbow extend
(bend too far) upwards more than an extra 10 degrees beyond a normal outstretched
position? If yes, add one point for each side.

D. KNEES

Test both sides: While standing, with knees locked (bent backwards as far as possible), does
the lower part of either leg extend more than 10 degrees forward? If yes, add one point for
each side.

E. SPINE

Can you bend forward and place the palms of your hands flat on the floor in front of your feet
without bending your knees? If yes, add one point.

PLEASE NOTE: When reading about this in professional textbooks the formal language used is as follows:

(A) With the palm of the hand and forearm resting on a flat surface with the elbow flexed at 90°, if the metacarpal-
phalangeal joint of the fifth finger can be hyperextended more than 90° with respect to the dorsum of the hand, it is
considered positive, scoring 1 point.
(B) With arms outstretched forward but hand pronated, if the thumb can be passively moved to touch the ipsilateral
forearm it is considered positive scoring 1 point.
(C) With the arms outstretched to the side and hand supine, if the elbow extends more than 10°, it is considered
positive scoring 1 point.
(D) While standing, with knees locked in genu recurvatum, if the knee extends more than 10°, it is considered positive
scoring 1 point.
(E) With knees locked straight and feet together, if the patient can bend forward to place the total palm of both hands
flat on the floor just in front of the feet, it is considered positive scoring 1 point.

NB: Modified version of the BS as used by Romeo et al., (2016) involved exclusion of the palms to floor measurement and
replacement with the evaluation of passive dorsiflexion of the ankle joint bilaterally
Carter and Wilkinson Scoring System (Czaprowski et al., 2012)
The Carter and Wilkinson scale is similar to the Beighton method, though the former was developed a decade earlier.
Both methods focus on the mobility of the thumb, elbow and knee joint, but there are two differences between them:
first, in the Beigthon method, the assessment of passive hyperextension of the MCP in the 5th finger was replaced with
an assessment of passive hyperextension of all four fingers, 2nd through 5th, to a position parallel to the forearm extensor
aspect (Fig. 6). Second, instead of assessing the ability to touch the ground with one’s palms as adopted by Beighton, the
Carter and Wilkinson method assesses the range of ankle dorsiflexion; dorsiflexion past 45 degrees is considered a positive
result. Joint hypermobility may be diagnosed when three out of five symptoms are observed.
Modified Line Drawings (Cooper et al., 2018)

Self-report instrument for assessment of trunk flexion: Self-report instrument for assessment of elbow extension:

Self-report instrument for assessment of knee extension Self-report instrument for assessment of little finger extension

Self-report instrument for assessment of thumb opposition

Sasche Scale (Skwiot et al., 2018)
1) Lumbar spine retroflexion–startingposition: lying prone, elbows bent, hands placed flat on the floor at the height of
the shoulders, forearms placed along the long axis of the body. Motion: lifting the upper body by the pressure of the
arms, raising it as far as the lumbar spine. Extension degree of the elbow joint: A. <90 ;̊ B. 90 ̊-120 ̊; C. >120 ̊.

2) Lumbar spine anteflexion–starting position: Standing upright. Motion: bending forward to touch the floor with the
hands, knee joints extended. The patient: A. touches the floor with the tips of her fingers; B. touches the floor with her
knuckles (i.e. the metacarpophalangeal joints are bent) C. entire hands are placed on the floor; the chest is placed against
the thighs.

3) Lumbarside-bending–starting position: standing upright. Motion: side-bending. The plumb line from the fold of the
contralateral axilla: A. reaches no further than the intergluteal cleft; B. Reaches the middle of the buttock on the opposite
side; C. reaches beyond the lateral aspect of the buttock.

4) Trunk rotation–starting position: sitting a stride a chair without back support, hands placed on the nape of the neck,
elbows together. Motion: turning left and right in succession: A. <50 ̊; B. 50 -̊ 70 ̊; C. >70 ̊.

5) Rotation of the head and neck–starting position: sitting on a chair, cervical spine retracted. Rotation in one direction:
A. <70 ̊; B. 70 -̊ 90 ̊; C. >90 ̊. Passive dorsal hyper extension of metacarpophalangeal joints assessment of average angle
between the long axis of the forearm and the little finger: A. <45 ̊; B. 45 ̊-60 ̊; C. >60 .̊

6) Passive apposition of thumb-to-flexor aspect of forearm A. The thumb does not touch the forearm; B. The thumb
touches the forearm slightly; C. the thumb easily touches the fore- arm, can be placed against it.

7) Elbow joint extension–starting position: sitting upright, shoulder and elbow joints bent at an internal angle of 90 ̊,
forearms aligned and touching, hands with palms facing upward. Motion: extending elbow joints without separating the
forearms: A. >80 ;̊ B. 80 -̊ 65 ;̊ C. <65 .̊

8) Shoulder mobility within the shoulder girdle–starting position: sitting upright on a chair. Motion: bringing the elbow
joint towards the contralateral shoulder: A. the elbow reaches the midline of the body; B. the elbow crosses the midline
of the body and moves towards the contralateral shoulder; C. the elbow touches the contralateral shoulder.

9) Shoulder mobility–startingposition:standingupright.Motion:attemptingtomakeboth hands meet diagonally behind the

back: A. the fingers do not touch; B. the fingers come into contact or overlap at the height of the first phalanx bone; C.
Entire hands overlap.

10) Passive extension of knee joint–lying supine: A.full extension; B. Hyperextension<10 ;̊ C. Hyperextension >10 ̊.

11) Passive abduction of hip joint–lying supine: A.<40 ̊;B.40 ̊-50 ;̊ C.>50 ̊.

12) Rotation of hip joint–combination of passive internal and external rotation in one joint lying supine A. <90 ;̊ B. 90 ̊-
120 ;̊ C. >120 .
Kapandji Index (Jha et al., 2016)
Hakim and Grahame Questionnaire/ 5pQ (Hakim and Grahame, 2003)

1. Can you now (or could you ever) lay your hands flat on the floor during a forward bend
without flexing your knees?

2. Can you touch (or have you ever been able to touch) your forearm with your thumb?

3. As a child, have you been able to bend your body in different shapes or could you make a
split?

4. Did you have a sprained shoulder or patella more than once as a child or teenager?

5. Do you think you are a flexible person?

Self-Reported Beighton Score (Naal et al., 2014)

The Upper Limb Hypermobility Assessment Tool (Nicholson and Chan, 2018)
The Lower Limb Hypermobility Assessment Tool (Ferrari, Vennet and Hayward, 2005)
Rotes-Querol Criteria (Martinez et al., 2013)

Remvig et al (2007) state: Rotès-Quérol, et al9, in contrast to others7,8,18, have outlined some GJH testing
recommendations. They should: (1) be easy to measure; (2) be applied to joints in which the primary
movement takes place in only one plane (elbow, knee, finger joints); (3) be extension movements because they
are less affected by soft tissue interposition; (4) measure the angle of maximum mobility in degrees using lines
and planes defined by the segments of the skeleton; and (5) have a cutoff level that identifies 20% of the general
population as hypermobile.
The Contompasis Score (Vallis et al., 2018)

Six tests are used and a numerical score is assigned to each test in accordance with the
performance. Test 1-4 and test 6 are performed bilaterally. A perfect score would be 22 points=no
ligamentous laxity. This score is obtained by giving 2 points for a normal value in 5 tests bilaterally
and 2 points for spine flexibility. The highest score would be 72 points.

1. Passive apposition of the thumb to the flexor aspect of the forearm (thumb to wrist test)
2 points: normal, 30 to 75 degrees, no touch of thumb to forearm,
4 points: low hypermobility, thumb touches forearm,
5 points: hypermobility, thumb digs into forearm easily
6 points: high hypermobility, thumb overlaps outside of forearm,

2. Passive dorsiflexion of the fifth metacarpophalangeal joint

2 points: normal, 30 to 85 degrees
4 points: low hypermobility, 90 to 100 degrees
5 points: hypermobility, 100 to 120 degrees
6 points: high hypermobility, 120 degrees and over
Our modification:
2 points: 30-89 degrees
4 points: 90-99 degrees
6 points: 121 degrees and over

3. Passive hyperextension of the elbow

2 points: normal, 0 to 5 degrees
4 points: low hypermobility, 10 to 16 degrees
5 points hypermobility, 16 to 20 degrees
6 points high hypermobility, 20 degrees and over
Our modification:
2 points: 0-9 degrees
4 points: 10-15 degrees
6 points: 21 degrees and over

4. Passive hyperextension of the knee

2 points: normal, 0 to 5 degrees
4 points: low hypermobility, 10 to 16 degrees
5 points: hypermobility, 16 to 20 degrees
6 points: high hypermobility, 20 degrees and over
Our modification:
2 points: 0-9 degrees
4 points: 10-15 degrees
6 points: 21 degrees and over
5. Hyperflexibility of the spinal column (forward flexion of the trunk)
2 points: normal, no contact with the ground
4 points: low hypermobility, fingertip touch to ground
5 points: hypermobility, fingers touching the ground
6 points: high hypermobility, palms to ground
7 or 8 points: highest hypermobility, wrist or forearm tog round

6. Foot flexibility test (ankle dorsiflexion and calcaneal stance position)

2 points: normal, 0 to 2 degrees eversion of calcaneus
4 points: low hypermobility, 3 to 5 degrees eversion of calcaneus
5 points: hypermobility, 6 to 10 degrees eversion of calcaneus
6 points: high hypermobility, 10 to 15 degrees eversion of calcaneus
8 points: highest hypermobility, 15 degrees and up eversion of calcaneus
Our modification:
6 points: 11-15 degrees
8 points: 16 degrees and over
Hospital Del Mar/Bulbena Criteria (Bevilaqua, Maillard, Ferrari, 2019)

Parveneh-Shiari Score (Parveneh and Shiari, 2016)

Marshall Test (Calhil et al., 2020)
Hypermobile EDS (2017) Criteria (The Ehlers Danlos Society, 2017)
“Old” (Outdated) Brighton Criteria (Colombi et al., 2015) Recommended to be replaced by
the Castori et al (2017) diagnostic approach as discussed below:

Hypermobility Spectrum Disorders (Castori et al., (2017)

HSDs are a group of clinically relevant conditions related to JH and are intended as descriptive and exclusion diagnoses. They are
distinguishable from hEDS and the other syndromes with JH because the phenotypic domains of HSDs are usually limited to the
musculoskeletal system. The involvement of the musculoskeletal system is intended as the presence of one or more of the secondary
manifestations of JH as reported above (i.e., trauma, pain, degenerative joint and bone disease, neurodevelopmental manifestations,
orthopedic traits) (Fig. 1). In these patients’ category, a limited extension to other organs and tissues, particularly in form of JH-related
co-morbidities (see below), is possible, but the overall clinical picture does not fit the criteria for one of the various EDS types. Therefore,
HSDs are mostly intended as alternative labels for patients with symptomatic JH who do not have any rare type of EDS and do not meet
the criteria for hEDS in terms of severity/pattern of musculoskeletal involvement and/or due to the absence of the other necessary criteria
(as reported in the new EDS nosology—this issue). In many circumstances, the HSDs will become the updated diagnosis for all those
individuals who met the previous criteria for EDS-HTor JHS but do not match the new hEDS criteria. However, HSDs are not limited solely
to substituting the “old” Brighton criteria, that should not yet be considered for modern patients’ classification. HSDs are also intended to
identify discrete subtypes filling the full gap between asymptomatic JH and hEDS. There might be a scenario where the diagnosis of HSD
is given to an individual with a family history of hEDS (i.e., relatives with an independent diagnosis of hEDS). Such a presentation might
suggest the same underlying genetic trait with variable expression. However, from a classification perspective, the diagnosis of hEDS is
established by the presence of a positive Beighton score (i.e., GJH) plus two or more among musculoskeletal criteria, systemic involvement,
and positive family history (as specifically defined in the new nosology). Hence, the addition of a family history alone should not be
sufficient to change a diagnosis from HSD to hEDS according to the new criteria. One recognizes this in other areas of musculoskeletal
medicine where the same principle applies. For example, there may be a family history of rheumatoid arthritis (RA) (as defined by accepted
international criteria), but the individual presents with some clinical features to suggest an autoimmune rheumatic disease but has
insufficient clinical and biological markers to define RA. The term “sero-negative inflammatory arthropathy” might apply. This individual
would be managed on the basis of their presenting complaint and followed to determine whether their condition changed in any way that
might then lead to a diagnosis of RA. HSD should be considered in the same way, including the possibility of clinical evolution and transition
to another diagnosis (e.g., hEDS). Although HSDs share JH with the other conditions and, in particular with EDS, at present it is premature
to a priori define HSDs as Mendelian disorders of the soft connective tissue. In fact, their molecular basis remains unknown and they may
occur sporadically, may segregate within families as Mendelian traits (dominant, recessive or X-linked) or they may aggregate in families
as multifactorial or polygenic traits. In selected cases and, particularly, in some children and in individuals from families with other relatives
with a previous diagnosis of hEDS (according to the new criteria), a “relaxed” follow-up in clinical genetics services may be scheduled due
to a potential future revision of the diagnosis to hEDS or potentially another JHrelated syndrome. In line with the previously delineated
types of JH, four different HSDs may be identified:

(1) Generalized (joint) HSD (G-HSD): GJH objectively assessed (e.g., by the Beighton score) plus one or more secondary
musculoskeletal manifestations as previously identified. In these patients, the pattern and severity of the involvement of the
musculoskeletal system should be carefully assessed in order to explore the possibility of a full-blown hEDS. In this category usually fall
most patients with GJH and additional musculoskeletal manifestations but do not meet the full diagnostic criteria for hEDS.
(2) Peripheral (joint) HSD (P-HSD): JH limited to hands and feet plus one or more secondary musculoskeletal manifestations as
previously identified.
(3) Localized (joint) HSD (L-HSD): JH at single joints or group of joints plus one, or more secondary musculoskeletal manifestations
regionally related to the hypermobile joint(s).
(4) Historical (joint) HSD (H-HSD): selfreported (historical) GJH (e.g., by the five-point questionnaire) with negative Beighton score plus
one or more secondary musculoskeletal manifestations as previously identified; in these cases, physical examination aimed at excluding
the alternative diagnoses of G-HSD, P-HSD, and L-HSD as well as other rheumatologic conditions is mandatory.
Global Joint Mobility Index (Bird, Brodie and Wright, 1979)