TIRTHY-SIX MONTHS OF
DRUG THERAPY IN A CHILD
WITH LIGAMENTOUS LAXITY
AND CLUMSINESS (updating May 2005)
Renato COCCHI, neurologist and medical
psychologist
Sigmundson and others, in 1998, do the most updated revision of
rehabilitative programs of the clumsiness, in use since 30 years.
The more recently introduced kinaesthetic
training is shown to have an effect on general motor competence. But this may
be better explained in terms of the general principles on which this training
procedure lies rather than the influence on Kinaesthesis per se.
I did not
find any information on a possible drug treatment. Since I started this in a
boy who did not more benefit from rehabilitative therapies, first I want to
give here a five-months report, which, I hope,
will be further updated.
The case history.
27.05.2002: male, seven years old at the first
consultation. He suffers from ligamentous laxity and clumsiness mainly when
running. Dysmetria is present and inaccurate fine motility. He had the
diagnosis of a physiatrist as ligamentous laxity. He is easily tired. Nighttime, he wear leg
prostheses to improve the posture of his feet.
He has footwear with reverse peaks.
At school he goes better in arithmetic.
He is born following urgent Cesarean section,
because maternal pelvic-fetal disproportion. His birthweight was 4020 grams.
What is curious, he opened his eyes after a week, and there was eyes' blow up.
In the first year of life he has given some feeding problems, and was
oversleeping. He suffered from belly colics with the tendency to constipation, not clear if it was atonic or
spastic one. He started to catch frequents upper respiratory tract infections
when he was three. Usually is a little pale, and when running he sweats in an exaggerated way.
Normally, he is enough serene and cheerful,
but little self-complaining. He talks much. The parents are separated since
about four years ago. Habitually he eats much, many sweet things, mainly
chocolate. He tastes the meat broth, and
raw vegetable. He sleeps much, and he talks when sleeping, but he
doesn't have drooling nor bruxism. In the morning he is more efficient, and in
the afternoon needs rest. He bikes, but that tired him in a short time.
Initial therapy, daily doses:
Glutamine 250mg alternating with S-adenosil-l-methionine (SAMe) 200mg;
pyridoxine 150mg; diazepam 0.8mg.
01.07.2002: The child is little more tonic
and less asthenic, but always a little uncoordinated. More control in his
feeding, and eats little fewer sweet things. He is less complaining. He always
need to have rest in the afternoon. The nighttime sleep improved. He doesn't
speak more at night. He stretches more to the awakening. Not early-morning
penis erection. His oversweating did not vary.
He talks very much. There are fewer
differences between morning hours and the afternoon. His falling asleep goes
well. No variations reported on his biking. His face is a little less pale.
21.10.2002: His clumsiness improved. On it
there is a positive judgment of the physiatrist that followed it (see her
certification transcribed below). The fine motility improved too. He
doesn't need to sleep more in the
afternoon, because he says to be not
tired. He mourns on must taking the
drugs. He runs more, but the bending of
the knees is still reduced. Still bony scrunches to the shoulders, in abduction
of the arms. He did not take the SAMe in regular way. Since he assumes it regularly, the bony scrunches of the shoulders
disappeared.
His face shows more colour, and sweats less. Penis erection appeared at
morning. He tasted meat broth and chocolate. His health runs better, even if he
does intense sporting activity at school.
His classmates accept him more. Bowel function is now regularized, while
a little constipated in past. Now he is more slim. His sleeping is less heavy.
Following his mother he improved at least 20%.
The Emilian physiatrist that followed him,
has seen again him the last September and found him improved, so she has no
more insisted with the need of a genetic investigation, to justify the stop in
motor progresses of the boy. She was told that the boy practised intense
physical activity during the summer, information already reported in the
certificate written at the end of her checkup.
She noted besides, in the same certificate: Ligamentous laxity. Internal
torsion of the right shinbone = 10° and left = 15°. Plantar prostheses: general
right foreversion = 50° and left = 68°. The dysmetria is not noticeable today.
To the dorsal rachis light asymmetry L right convex and left dorsal.
Current therapy
(daily doses):
28.04.2003: He is doing better. According to
his the mother, from the beginning of the drug therapy to today he improved at
least 50%. In mountains, he walked for 4km without any complain (before the
drug therapy, after 500 metres he had to stop because tired). He has more
endurance when biking and he enjoys it much more. Now he attends the swimming
pool and swims by himself, without the life belts. The clumsiness is reduced.
His muscles are more tonic, for which he
appears less "uncoordinated," as his mother said. He runs better.
When he is going downstairs, he uses less the handrail support and now there is
no difference between going upstairs and go downstairs. He jumps from two
steps, as first, but now he doesn't fall more along the floor.
He doesn't succeed to run by overflexing the
leg on the thigh and touching the gluteus with his heel, as his classmates do
during the lesson of physical activity. The last consulted orthopaedic
suspended every kind of support, by esteeming that they do not need anymore.
Now, he is not wearing shoes with the reverse peaks. No more bony scrunches to the shoulders, in
abduction of the arms..
As for his health he did well, but a pharyngitis
with one antibiotic therapy in six months. He gained some body weight. There is
no more greediness for the meat or cube broth, - now he likes the pasta - and
even no more greediness for the chocolate. The quantity of food of his diet is
under his control, he eats more raw vegetables. When among other children he
inclines to become a little hyperactive ( a compensation symptom for his
previous sending off due to his clumsiness?)
His attention increased and at school he is going
better with increased notes both in Italian and in arithmetic. He has the habit
of refusing to do little help in his house ( eg. He says that he does not want
to clear the table ) but then, when alone, he does what asked him. He does want
the others see that he obeys at once to the orders. Regular bowel function,
regular sleep, his awakenings are well.
The fingernails grow stronger, while first
they were weak with easiness to broken. He sweats lesser. Some time puffs for
the taking the pills, but he is perfectly sentient of their utility. He speaks
more and stands better erect.
Current therapy
(daily doses): Creatine 2g;
27 October 2003: He is doing better, but he shows
till some awkwardness in the running. He shows even clumsy running in the last
part of the road that he crosses on foot, when going to school. His feet always
little face towards the internal side. Increased the endurance, but he is
biking only a few, even because the parents not leave it going out the
courtyard of his house. He does swim, but he does not finish the long side of
the swimming pool, so he does not go from a head to the other. Now, he jumps
from three stair steps. Like his classmates do during gymnastics lessons, he
succeeds to run by overflexing the leg on air on his thigh, touching the
gluteus with his calcaneum. Not more scapular noise, which reappeared when the
mother stopped the SAMe, and disappeared again after its resumption. In the
afternoon he does not go more to sleep, as he did to recover energy. The
relatives observed his improvement. No more long leg prostheses for the night in use, and the shoes are without plantar prostheses.
As for his health, he had some cough since
he started the school. Corticosteroids for five days were prescribed. The last
summer he suffered from colitis for about two months.
Towards 7 PM he has to eat, if not he does
it, he has colic ache. His mother says that he improved 55%, he increased in
its strength and in his tallness but not in weight. Less oversweating now,
which in past appeared five minutes after physical activity, and now only after
half an hour. At school he is doing well. The classmates do not take him more
in turn. He is no more isolate in the interval between the lessons. He speaks
very much, and stands more erected. Sometimes he mourns about his drug taking.
Therapeutic variation: Pyridoxine 75mg.
26.04.2004: As for clumsiness, there are
only fewer improvements. He is clumsy in running and in walking. His running is
slow. Touching the gluteus muscle with the heel is always a hard task. During
the winter he did poor physical activity. When he is going up and downstairs,
he does not need to hold more the handrail. Now he jumps down from 2-3 steps of
the stairs. He has more strength and more endurance. Since the classmates say
him as clumsy, he takes offence at it. He does not wear anymore leg prostheses
at night.
Oversweating did not vary, and the boy
sweats after 20-30 minutes of animate game, but perhaps, for the sweat, it is
lesser. He is afraid of anything (ants, flies, spiders, and, too being at home,
of the storm). The teachers said that he had poor self-esteem. When they
question him while they are explaining, he turns red and extends to jam.
Usually, he inclines to do less of what he may do.
He is speaking always much, he has his face
more pink, his weigh is 35kg and his height is 129cm. Now he has a reduced
sense of hunger and he has not more feeling of "a hole" in his
stomach. Now he controls more his appetite even in presence of the preferred
foods and so he lowered of one kilogram.
Last winter he suffered from coughs and
tonsillitis and he had to take antibiotics. His mother did not see any penis
erection. When relaxed, there is a kind of scratching on his genitals. He had a
period of agitation, and he was biting his nails as well as the short skin
around to the fingernails.
Therapeutic variation (daily doses, by the
oral via ): The s-adenosyl-l-methionine stopped. It is prescribed chlomipramine
10mg.
08 November 2004, The sixth checkup under
drug therapy, always maintained. He is doing better, even if not in all his
motor skills. His endurance became greater. Every week he plays two basketball
hours + another hour at school. Sweating appears a few more than his
classmates. Then, he feels tired till evening. He has a less ready sprint, in
comparison with the others, and this even in running, during the hour of
physical education. Now he is much biking. In plain he withstands an hour and
half biking. He climbs and goes down the stairs without the handrail. When he
jumps from the second step, he never falls, while some time he falls when he
jumps from the third step.
The school results were well gone and now he
attends the fourth year of elementary school. At home, as for the study, he
does not always put a lot.
There is no information on the judgment by
the teachers after the summer holidays.
Altogether improved, he has more desire to
do, he is more resistant, plays of more, more happy, more integrated in the
peers' group. He has done a survival camp, without fear. He does crack the
articulations of the hands' fingers. Now, he speaks better and is more
confiding with his mother.
He has reduced his moving aside less and he
is more aggressive against the little sister, who was taking advantage. He
defends himself of more. Some times he seems return to be timid.
His health did not go well. He had cough and
tonsillitis every 15 days, for which he was given many antibiotics. Now he is periodically
taking a vaccine, which seems it is protecting him. At night he does not speak
more during the sleep and not has any nighttime bruxism. The sleep is lighter
and inclines to throw off his clothes.
Eating is regulated, and he became taller
(130.5 cm) but not more weigh. Usually he eats more vegetable, when forced, and
fruit. His greediness for sweet things changed very few.
In summer he was taking a tablet of 10mg
chlomipramine every day, while now he takes one every two days, because he had
a quite exciting period. At the beginning of the new school year, he suffered
from a panic attack [?] for which he took again 10mg chlomipramine daily, for
15 days.
Therapeutic variation (daily doses, by the
oral via): 5-hydroxytriptophan, 25mg.
End May 2005, the seventh checkup. In this
period he did quite well, even as for his health, with an only tonsillitis.
Following a telephone agreement, his mother stopped the chlomipramine, because
it made him a little stunned. He is still slow, but stamina is going better. He
improved the relationship with the peer group. Now, he attends a basket
preparatory course, with three training every week [??]. Some time, during
running, he succeeds to touch the gluteal muscle with his heel. At school he
has more than discreet results. While first his smaller sister dominated him,
now he imposes to her.
His self-esteem is growing. When he wakes up
the presence of an early-morning erection of the penis is increased. He sleeps
well, and his sleep is less heavy. To his mother, I explain that, of fact, the
child is doing a substituting therapy, but I do not know if he will always take
it. Perhaps his body, at the end, will find an efficient balance, and so he
will stop the drugs taking.
No therapy variation.
I considered stress symptoms,
defective or of compensation (Cocchi, 1985):
- the asthenia;
- a tendency to
the hypersomnia;
- the need to rest
in the afternoon;
- the easiness to
upper respiratory tract infections;
- the overeating;
Either the need of sweet things and the
taste of the meat broth pointed out on a reduction of GABAergic pathways, with
reduced type A GABAergic inhibition (Cocchi, 1987).
On such neurochemical circuits I acted with
glutamine (A precursor of the glutamic acid and the GABA), pyridoxine (The catalyst of the GAD, as the cofactor of
the transformation of the glutamic acid into GABA ) and diazepam (as a
benzodiazepine, one sensitizer of the type A GABAergic receptor).
As
for the SAMe, in the preliminary communication on its effect on the ligamentous
laxity in the Down child (Cocchi, 1990) it seemed me that such action could be
justified from these reasons, which follow.
"Since homocysteine comes out from
the SAMe, the prescription of SAMe could then drive, among other results, to a
larger availability of homocysteine. Consequently it could drive to an
increased activity of growing bones cartilages, in the years when the body
grows.
This one is a suggestion not too ease to
verify, even because many variables coexist.
Finally, I cannot affirm that improved
ligamentous laxity has a necessary link with the SAMe. Nevertheless, I noted
this result on children with ligamentous laxity only after I added the SAMe to
the regimen."
In this non-Down child, but with ligamentous
laxity, this previously hypothesized effect was willingly looked for, and it was already observed at the first checkup after a month of drug
therapy.
On other hand, after thirty months, also
nearly all the stress symptoms are at least attenuated.
Both more evident symptoms, in this
child, the ligamentous laxity and the clumsiness benefitted from the drug
therapy prescribed (the effect of the creatine does not have still a checkup).
That happened when the phyisiatric intervention, as surely appropriate and with
his efficacy, did not seem may bring
other improvements.
Eventually, I personally have doubts that
the cerebellar involvement, which surely
exists for the presence of the dysmetria symptom, may be faced with the
physiatric therapy alone.
Moreover, the increasing of the physical activity, in a peripheral asthenic
subject, would have even worsened the asthenia itself for the consequent
physical tiredness. We need to remember, as for it, that the boy had to go to
rest in the afternoon, to recover his strengths.
This would be a self-perpetuating
circle that could be only interrupted by giving energy to the muscles. The
glutamine assumption, which the organism produces mainly from the muscular ATP,
save up this last one.
The creatine is a precursor of the ATP that allows to
improve the physical endurance, and its addition to the regimen has just
brought to results otherwise obtainable in a short time like that.
There is a new fact, concerning the SAMe.
The scapular noise, which was missing, reappeared after the suspension of the
SAMe his mother decided. It then disappeared again to drug resumption. The
A-B-A sequence, missing-reappearing-missing again, in the same case, as related
to the assumption of the SAME, confirms the action of this drug on the joints.
The last checkup revealed some symptoms of
depression, although the boy was apparently serene.
Thirty-six months later, as for the
motility, it is not clear if he is doing a substituting therapy, or even
curative one. The cracking of the articulations of the hands' fingers is surely
a sign of decreasing of the joint laxity.
In a child with ligamentous laxity and
clumsiness an antistress drug therapy, able also to the saving of the ATP and
to possible improve the ligamentous matter,
has brought to an amelioration in both basic symptoms of base, after 36
months. This clearly opens many questions to which I should try to answer in
the following updating of the case.
References
Agnew P.: Evaluation of the child with ligamentous
laxity. Clin Podiatr Med Surg 1997, 14:117-130.
Cocchi R.: A syndrome
from possible
GABA deficiency.
Clinical therapeutic
report of 15 cases. Acta Pschiat. Belg. 78,
407-424, 1978.
Cocchi R.: Le depressioni infantili. In: Strutture e dinamiche psicopatologiche
in età evolutiva. Montefeltro, Urbino 1985: 163-183.
Cocchi R.:
Hypo-A-GABA-erge Depression bei Kindern. Klinisches
Bild und mit neurochemischen Mechanismen verbundene Symptome. In Friese
H.-J., Trott G.-E. (eds): Depression in Kindheit und Jugend. Huber, Bern 1988: 126-133.
Cocchi R.: La
S-Adenosil-L-Metionina (SAMe) riduce la lassità articolare nel bambino Down?
(Comunicazione preliminare). Riv.Disturbo
Intellet. 1990, 3: 141-144.
Nork SE; Hennrikus WL; Loncarich DP; Gillingham BL; Lapinsky AS.
Relationship between ligamentous laxity and the site of upper extremity
fractures in children: extension supracondylar fracture versus distal forearm
fracture. J Pediatr Orthop B 1999, 8:90-92.
Sigmundsson H; Pedersen
AV; Whiting HT; Ingvaldsen RP. We can cure your child's clumsiness! A review of
interven-tion methods. Scand J Rehabil Med 1998 Jun;30(2):101-106.
Stramentinoli G.: Neuroendocrine effects of
S-adenosylmethionine (SAMe)
(dattiloscrit-to). BioResearch, Milano 1987b.
Posted in Internet on November 2002. Copyright by Renato Cocchi, 2002
42100 Reggio
Emilia (Italy)
renatococchi@libero.it