Sport Medicine Unit
of the USL 3-Pesaro ( Head: Dr. G. Tassani).
ATLANTO-AXIAL INSTABILITY
IN DOWN PERSONS. NEUROLOGIC RISKS
IN THE SPORT PRACTICE, MAINLY IN THE
JUDO.
Giuseppe TASSANI (*) Renato COCCHI (**)
Paolo Alberto PAGANI (***)
(*) A specialist in Sport Medicine; (**) A
neurologist; (***) An orthopaedist.
Summary.
About 20% Down children are carriers of
atlanto-axial instability, a risky condition for physical activities or sports
such as gymnastics, diving and Judo. This malformation is inversely age related
and is prevailing among male children. Although it could be intuitively linked
to laxity, no more than 10% of lax Down children are also affected by
atlanto-axial instability. This instability can be detected by screening X-ray
examination of the lateral cervical spine both in neutral position and in
flexing.
Over four mm forwarding dislocation of
the odontoidal tip lead to suspicion, but over 5 mm is overtly assumed as
pathological. Symptoms of medullary compression can be detected after
neurological examination and confirmed by ACT of the atlanto-axial region. The
screening of atlanto-axial instability in Down children should be included in
preliminary routine tests before medical certification of fitness for school
gymnastics and sports.
Key words: Atlanto-axial instability;
Down's syndrome; risky physical activities; Judo; detection.
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The Down syndrome, also called Trisomy 21,
works for more than 30% of the mental retardation forms from chromosomal anomalies.
The discovery and the use of the antibiotics deeply modified the life hope of
these individuals. Before 1945 it was difficult to meet a Down person aged more
than ten years. Mostly of them died before this age, for bronchopulmonary
infections.
The possibility of a longer life grew
parallelling to a general habit of better acceptance of this disability. The
spreading of the idea and of the practice of what is call of
"normalization" is driving their parents to start the children even towards
sport activities.
In such direction there was already the push
of the diffused practice of swimming, often started in early childhood as a
support of motor rehabilitation.
Approaching sport activity for Down
persons.
The meeting of both the parents wishful idea
and an always greater availability of either half-public or private sporting
trainers are bringing these subjects to be put in many sporting disciplines
compatible with their motor situation.
The motor skills of in Downs on one hand are
close to those of other mentally retarded without any motor damage. Their
features are primarily motor awkwardness, some balance troubles, a reduced
ability in fine motor actions.
On the other hand their motility can show
own negative aspects, in particular for the rare presence of pure hypotonia
(Favuto and Cocchi, 1992), primarily of cerebellar origin, and the more spread
ligamentous laxity.
The atlanto-axial instability, which would
be observed with a frequency of about 15-20%, is a recent finding (Vermeer and
coll., 1992).
In these Down persons to take up gymnastics,
diving and judo could have some danger, for an exaggerate excursion of the
tooth of the epistropheus, leading to compression or the lesion of medullary
nervous pathways. The damage, besides its clinical evidence, can be confirmed
by CAT (Alvarez and Rubin, 1986) and by NMR imaging.
This ligamentous anomaly has a higher
incidence in males with less than eleven years, but even this datum is in
contrast with preceding assertions (Alvarez and Rubin, 1986). Instead it is in
accord, as for the age, with an investigation on the presence of pure
hypotonicity in Downs, one of us carried on (Favuto and Cocchi, 1992).
Although we can think at once that the
atlanto-axial instability recalls the presence of ligamentous laxity (Semine
and co., 1987; Collacott et al., 1989), recent specific investigations observed
no more than 10% of Downs with ligamentous laxity is also the carrier of this
anomaly (Cremers, 1992).
As for the prevention of it, besides its
description and the spreading of the knowledge of the warnings symptoms
(Howard, 1985) it has tried a X-ray evaluation of the atlanto-axial
instability. It can be done by comparing the X-graphic examination of the head
in a neutral position and in bending.
An excursion of more than 3mm of the
epistropheus tooth peak became already considered as suspected, if more than
4mm as frankly pathological and a sign of atlanto-axial instability (Wang and
coll., 1984; Cremers, 1992).
Very new studies on this specific aspect led
the X-ray screening as doubtful, at least for distances less than 5mm (Cremers,
Ramos and Boll, 1992). On the other side, specific alternate investigations
with greater sensibility did not have been proposed.
The problem however exists, and the X-ray
investigation-graphic, even if controversial, still stands up as the only way
of checkup in order of prevention.
Finding an excursion larger than 4mm forces
therefore to caution in the gymnastic practice and in sports where is bending
and forced extensions of the neck can occur.
Orthopaedic considerations.
Non traumatic atlanto-axial instabilities
are rare events, and they can add to severe congenital pathology, as we are
observing in the Down syndrome of Down. They also can appear in late states of
degenerative illnesses, as it happens in the rheumatoid arthritis.
Being greater body of knowledge available,
for an easier explanation we refer on what happens in this last illness. Now,
there is not any evidence in the Down syndrome this phenomenon gives rising to
symptoms or to a specific pathological frame.
Most of these patients tolerate the
instability for long, without signs of neurologic troubles.
The capsule-ligamentous lesion of the
occipital-cervical joint and of the C1 and C2 vertebras, is going on in some
persons, with a deceitful onset of the symptoms. So because the loosening of
ligamentous formation without any bony compression, occurs in a zone where the
medullary channel is ample.
The atlanto-axial subdislocation till to a
move of 3.5mm in every direction, may be considered as not being to threaten. A
greater measure is an evident mark of joint instability.
For the evaluation of the instability we may
apply the clinical criteria described by Ranawat and coll. in 1979, and by
Conaty and Mongan in 1981. At least for what concerns the rheumatoid arthritis
in the II stage, we can already observe weakness, hyperaesthesias and
dysthesias. In the IIIa stage, it arrives to paresis, medullary damage, with
maintenance of the walking. At IIIb stage we may find evident tetraparesis and
walking inability.
The prevailing therapeutic approach, now, is
surgery aiming to stabilize the cervical tract, to take place when the paresis
starts, according to Louis, 1983.
From the theory to the practice: The
Judo.
Some sports are particularly prone to the
skull-cervical traumas. We have to exclude, of course, the motorcycle sports,
while we have to pay consideration diving, the American football, and generally
the "in touch" sports.
Among last ones, our attention goes to the
Judo, and mainly to the techniques of immobilization that involve the
strangulation, or Shime-Wasa of the traditional Judo language. In the following
examples there are enough risk elements.
1. Collar
strangulation with both collars (Okuri Eri Jime).
This technique involves, by whom attacks
("Tori") on anyone suffers ("Uke"), a triplex solicitation
on the neck and on its structures, such as respiratory airways, blood vessels,
cervical rachis. The constricting component associates a distraction and
inevitably to the rotating one, imposing in this way a forward bending of the
head with neck rotation.
2. Strangulation
by naked hands (Hadaka Jieme).
Even here there is an association of same
three components, constraint, distraction and rotation of the neck, which end
to impose the anterior bending, and in smaller measure, the rotation and the
lateral dislocation.
3. Strangulation
with a wing-like arm (Kataha Jime).
Still a solicitation example of the joint
where it prevails the rotating component in association with the lateral
dislocation.
4. Strangulation
with opposite hands (Kata Juji Jime).
We refer to a holding with potential
terrible consequences, when it is acted during the fight. Even here the joint
solicitations on the first cervical vertebras associate a violent distraction
with a marked constraint.
5. Strangulation
by a triangulate way (Sankaku Jime).
The roots of the arms and the neck of the
attacked one come imprisoned by the assailant as a triangle. The vertex of
which is the pubis, the second point is directly blocked by the flexing surface
of the right flexed knee, and the last point is blocked by the other knee with
the interposition of the other side ankle that has the fixation function.
The biomechanics' solicitation imposes at
once an attitude in bending of the head on the neck, with an exaggerate
distracting component that can even associate to a neck hyperextension.
As for these immobilization techniques, it
is easily to sense how to the muscle-ligaments apparatus of the neck,
generally, and the cervical rachis joints are strongly solicited also in static
conditions, when the held person has enough time to oppose the resistance of a
valid muscular tone.
If the same technique is applied during
fighting, when the gesture develops to elevated speed to make it unpredictable
to the adversary, the adjustment of the muscular tone of the sufferer could be
late and not incomplete. In this way the cervical joints are vulnerable to high
risks.
As it concerns us, these subjects can own
some systemic muscular hypotonus, added to ligamentous atlanto-axial
instability. The biomechanics solicitations applied there can involve the risk
of a true, anterior, roundabout or mixed dislocation. On the other hand the
back dislocation can occur only when accompanied from the epistropheus tooth's
fracture.
The improvement of a diagnostic technique is
a wishful thing, and it will allow of appraising the risk of a partial
incontinence of the atlanto-axial joint, to which to undergo Down subjects
inclined to practise Judo.
On the other hand it is a sport with
interesting aspects, beyond to get defence and attack techniques.
The control of the bodily scheme, for
example, the way to the dexterity, the study and the approach to the adversary,
the discipline and the behaviour in the Dojo, or the room of practising, the
personal hygiene and the respect for the teacher and for the adversary, are all
elements that make the judo an excellent educational proposal.
Conclusions.
The investigation on the possible presence
of atlanto-axial instability should usually be integrated within the
preliminary examinations for the medical certification of suitability for the
school physical education and for sport practising in Down children (Howard,
1985).
Since the difficulties met till now and
above exposed, it is always better to have false positive to whom giving
advices to avoid certain sport practices as the Judo (Gunasingham and Akuffo,
1988), than ignore this problem. The personal risks for the subject and that
legal ones for the pediatrician, for the general practitioners, or the sport
physician, could become too high.
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First printed in Italian on Riv. It.
Disturbo Intellet. !992, 5: 239-244. Posted on Internet on March 2004.
Copyright by Renato Cocchi, 2004.
Author's address: dr Renato COCCHI, via Rabbeno, 3
42100 Reggio Emilia (Italy).
renatococchi@libero.it
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