VARIABILITY
OF MONGOLISM
By Abraham Levinson, M.D.,* Abraham Friedman, M.D.,t and
Fredrick Stamps, M.D.
43
T
HE SIMILARITY in the physiognomy ofchildren with mongolism has given rise
to a generally held concept that there is no
variability in the syndrome, that all these
children look alike and develop alike. This
over-emphasis on similarity is most evident in the description of mongolism in medical
textbooks where the variability usually
stressed in the discussion of other diseases
is notably absent in the discussion of mon-golism. Even Benda,1 recognized as an
au-thority on mongolism, cannot be absolved
of this “sin of omission.” Oster,2 in his book
on mongolism, does show the variability in
the physical features of mongoboids but
fails to bring out the dissimilarities in their
developmental behavior.
Early in our experience with mentally
retarded children we found so many
indi-vidual differences among the mongoloid
children who came to our Clinic, that we
began to wonder whether the idea of
uni-formity had not been over-emphasized.
With this thought in mind, we undertook
a systematic study of a group of mongoloid
children, noting carefully all variations in
the physical and mental characteristics
usu-ally attributed to them.
The results of our study are incorporated in this paper.
MATERIAL AND METHODS
Fifty consecutive patients with mongolism
were selected for study.
The patients were drawn from the Clinic
of the Dr. Julian D. Levinson Research
Founda-From the Dr. Julian D. Levinson Research Foundation, the Children’s Division of the Cook County Hospital and the Electroencephalography Laboratory of the University of Illinois Medical School, Chicago, Illinois.
(Submitted for publication January 10, 1955.) * ADDRESS: 30 N. Michigan Avenue, Chicago 2,
Illinois.
f Research Fellow in Pediatric Neurology.
tion for Mentally Retarded Children. This
Foundation, which is located at the Cook
County Hospital of Chicago, is closely
associ-ated with the Children’s Division of the
Hos-pita! and the Hektoen Institute for Medical Research.
The study consisted of a detailed history,
which included complete data on the course of
pregnancy, the birth and neonatal period. The family history included data on the ages of the parents at the birth of the child as well as the order of the birth. The growth and
develop-ment of each child, motor as well as speech,
were carefully noted. The symptoms were
studied with particular reference to convul-sions, hearing, vision, sleep, handedness, con-stipation, upper respiratory infections.
The physical examination was done with
special reference to the numerous physical
characteristics generally assumed to be
asso-ciated with mongolism. The results were
en-tered on a form specially prepared for this purpose.
A complete psychological evaluation was
done on every patient including testing for in-telligence, social maturity and personality
fea-tures. The results of the psychological study
will be published in a separate paper.
The laboratory studies included
noentgeno-grams of the skull, long bones and wrists, an
electroencephalogram, and also radioiodine
up-take on practically all the children. The results of the latter study are provided in a companion
paper.3 All the laboratory tests were done at
the Cook County Hospital with the exception
of electroencephabography which was done at
the University of Illinois EEC laboratory.
History
RESULTS OF ANALYSIS OF
PATIENTS’ RECORDS
AGE, SEX AND RAQi: Of the fifty patients
selected for study, there were 27 (54 per
cent) males and 23 (46 per cent) females.
There were 43 (86 per cent) white, 6 (12
per cent) negro and 1 (2 per cent) Korean.
Generally, negro children constitute about
44 LEVINSON - VARIABILITY OF MONGOLISM
Clinic. As only 12 per cent of the children
were negro, it appears that mongolism
oc-curred bess frequently in the negro than in
the white. It is obvious that mongolism may
occur in any race, as our Korean patient
exemplifies. The age distribution of our
pa-tients is shown in Chart I.
AGE MONGOLISM WAS DIAGNOSED: Only
17 patients (34 per cent) were diagnosed as
mongoboids at birth or during the neonatal
period and 15 patients (30 per cent) were
diagnosed between 1 and 6 months of age.
Seven (14 per cent) were diagnosed in the
second half-year of the first year of life, 5
(10 per cent) during the second year of life
and 4 (8 per cent) not until they were well
beyond the second year. In 2 patients this
information was not elicited (Chart II).
These findings may indicate a lack of
aware-ness of obstetricians and pediatricians to
this diagnosis, or reluctance on the part of
physicians to inform the parents of the
tragedy. However, in our opinion, this may
be a reflection of variability in the
“mon-goloid facies” which are not as apparent at
birth as is usually thought.
PREGNANCY, BIRTH AND NEONATAL
PE-RIOD: Forty (80 per cent) were full-term
babies, while 10 (20 per cent) were
prema-tures, i.e., under 2500 gm. birthweight. This
incidence of prematurity among mongoboids
in this series is much higher than that in
the normal child population which is
be-tween 7 and 11 per cent.4 It is, however,
much the same as the incidence of
pre-maturity we found among all our mentally
retarded patients which is 19 per cent.5
The delivery was spontaneous for 32 (64
per cent) patients and instrumental for 12
(24 per cent). In 4 (8 per cent) the delivery
was breech and 1 (2 per cent) was delivered
by caesanian section. For 1 patient the
in-formation was not elicited.
The birth weight distribution is
illus-trated in Chart III, showing greater
varia-bility than in normal babies.
Neonatal complications were relatively
frequent. Nine (18 per cent) were cyanosed
at birth, requiring resuscitation. None had
convulsions during the neonatal period.
Three (6 per cent) gave a history of
neo-natal jaundice. In 1 of these the jaundice
lasted 21 days.
DEVELOPMENTAL HISTORY: The ages at
which these 50 children acquired head
con-trol is shown in Chart IV. It is clearly seen
how great the variability may be as to the
age at which the child learns to hold up his
head. While, according to the mother, some
did so at 2 months of age, others did not
acquire the power until the age of 23 years.
The great variability in the age at which
the child sat up is shown in Chart V. The
usual time for sitting up was 12 months but
some sat up even as early as the normal 6 to
8 months. On the other hand, a large
num-ben did not sit up until they were much
older, even as bate as 3 years.
The age at which the child began to
walk also shows a variability which is very
great indeed (Chart VI). While some began
to walk at the normal age of 1 year, others
did not begin to walk until they were 3, 4,
or even years old with a peak incidence
at the 2-year level.
Tooth eruption also showed great
varia-bility. As seen in Chart VII, some had their
first tooth as early as 5 to 6 months of age.
In the barge majority there was a variable
delay with a peak at 1 year of age and the
age of delay extended up to 2 years. There
was also considerable variability in the
order of the eruption of the teeth. In some,
the first teeth were the bower incisors, in
others the upper incisors, and, in quite a
few, the molars were the first to erupt.
Language development showed a marked
variability. Chart VIII shows the
distribu-tion of patients according to the age at
which they first started speaking two words.
It is seen that although there is a peak at 2
years of age, the time at which words were
spoken varied from less than 1 year of age
to over 6 years of age. The distribution of
patients according to the age when
con-nected speech was first used is shown in
Chart IX. The left half of the table shows
that of the 20 patients using sentences at
the first examination, the age at which
AGE DISTRIBUTION
NO. OF
CASES 7
6
.5 5
44 4 44
JILIJ
#{149}L1.
[J1lU-’L
LS t6AGE IN YEARS
17 AGE WHEN MONGOLISM
WAS DIAGNOSED
7
NEONATAL 1-6 7-li 1-i OVER NOT
PERIOD MOS. MOS. YRS. ZYBS. ELICITED
CASE DISTRIBUTION ACCORDING TO
BIRTH WEIGHT
NO.OF 7
CASES 6
-5
rJ1Jljfti
4 “ ‘‘2 346 Y4 Yz #{190}q /4 7 ‘/48 Yz #{188}9
BIRTH WEIGHT IN LBS.
46 LEVINSON - VARIABILITY OF \‘IONGOLISM
AGE CHILD HELD HEAD UP
16
NO.OF
7
CASES 6
4
ifL1I--LrtJtJ1
FL
23456789 101112 18 30 UNKNOWN
AGE IN MONTHS
AGE CHILD SAT UP
NO.OF 9
CASES
6 6
5
4
_fljTflr]rJJflsJ11i
6 7 8 9 10 ii 12 13 14 iS 18 24 30 36 NOT UNKNOWN
AGE IN MONTHS EXPECTED
l5
AGE OF WALKING
NO.OF
CASES
8
7
6
5
- . 4
JLjTThu!dLj
I 1>’z 2 2y, 3 3), 4 NOT UNKNOWN
AGE IN YEARS EXPECTED DELAYED
ORIGINAL ARTICLES
AGE AT ERUPTION OF FIRST TOOTH
l0
C
NO. OF CASES
5
ZZZZ
78,
In
24 UNKNOWN NOT
EXPECTED
6
NGOF 9
CASES
-6
ti.IfL
<i 1 1Y2 2
S
4
fln
4
15
4 4
a
za
345678910 123456 891011 13 17
AGE IN YEARS AGE TN YEARS
CHART \7JJ (Top). CHART VIII (Middle). CHART IX (Bottom). 3
IIL-LfLJ’i(L
10 IL 12 13 14 15 16 17 18 AGE IN MONTHS
AGE OF FIRST SPOKEN WORDS
5
21’i 3 3 4 SYz 6 76 DELAYED UNKNOWN NOT
AGE iN YEARS EXPECTED
SPEECH DEVELOPMENT
I. AGE CHILD FIRST K. AGE OF CHiLDREN NOT YET uSiNG SENTENCES COMPOSED SENTENCES
NO-op
4 NO. OF
CASES
IILJb
55
3
C
aa
lHHHhl
J1HHH[11
19 2021 22 23 24 25
L
rinn
fl
26 27 28 29 3031 32 53 34 35 3637
AGE OF MOTHER IN YEARS
38394041 42
16
13
9
6
I
1
1
2 3 4 5 6 12
48 LEVINSON
-
VARIABILITY OF MONGOLISMHandedness was investigated in every
AGE
OF
MOTHER
AT
BIRTH
OF
MONGOLOiD
CHILD
NQ OF CASES
ORDER O.F BIRTH OF MONGOLOID CHILD
CHART X (Upper). CHART XI (Lower).
to 10 years. The right half of the table
shows the age distribution of the 30
pa-tients who, at the time of the examination,
were still unable to use sentences. Their
ages varied from less than 1 year to 17
years. This shows the extent to which
de-lay in speech development may vary,
corn-pared with the relatively small variations
in normal children.
The speech of these children showed
great variation not only in the delay of
speech development, but also in the quality
of the speech. The speech articulation
showed frequent defects with substitutions, omissions and distortions, and also varied from patient to patient. Some also showed
disturbance in rhythm with stuttering and
stammering as occasional manifestations.
Many had voice problems. It was raucous
in 54 per cent and low-pitched in 20 per
HEAD
16%
- 4 %
14%
60%
74% A. SKULL
1 OPEN FONTANEL
REYOND 1Y2 YEARS)
2 OPEN SUTURF.S
3 FLAT OCCIPUT
B. FACE
I WRINKLED FOREHEAD
2 RED CHEEKS
3 ROUGH AND SCALY CHEEKS
C. EYES I SLANTING 2 EPICANTHUS 3 BLEPHARITIS 4 STRABISMUS 5 NYSTAGMUS
6 SPECKLING OFIRIS
7 DOUBLE ZONE IN IRIS
U EARS
I PROMINENT
2 MALFORMED
3 SMALL ORABSENT LOBULE
S. NOSE
1 FLAT NOSE
2 SMALL NOSE
3 FLAT NASAL BRIDGE
F MOUTH
I CONSTANTLY OPEN MOUFH
2 SMALL MOUTH
3 BROAD LIPS
4 IRREGULAR LIPS
5 DRY LIPS
6 FISSURED LIPS
7 SMALL TEETH
8 CONICAL LATERAL INCISORS
9 IRREGULAR ALINEMENT
10 WIDELY SPACED TEETH
Ii CROWDED TEETH
12LARGE TONGUE
13FURROWED TONGUE
14 PROTRUDING TONGUE 15 HIGH ARCHED PALATE
16NARROW PALATE
17 CLEFT UVULA
18 RAUCOUS VOICE
19 LOW PITCHED VOICE
80% 50% 38% 14% 14% 30% -
22%
50% 48% 44,,.
54% 62% 62% 32% 36% 28% 32% 56% 56% 40% 68% 28% 38 V. 30% 44% 32% 74% 52% .4% 54% - 20% CHART XII.patient and was far more variable than
in normal children. Whereas among normal
individuals right handedness is present in
about 95 per cent6 in our series of
mongol-oids right handedness was found in 48
per cent, left handedness in 14 per cent,
18 per cent were ambidextrous and 20 per
cent undetermined.
SPECIAL SYMPTOMS: Constipation was a
complaint in 20 per cent and frequent colds
in 24 per cent of the patients, while 38 per
cent gave a history of blepharitis.
Convulsions occurred in 3 patients.
One was a 2-year-old white girl who had
49
“infantile spasms” at 3 to 4 months of age.
An EEG was taken and interpreted as
hypsarrhythmia, a term coined by Gibbs.7
Another, an 11-year-old white male, had a
history of a grand mal convulsion at the age
of 1 year. The third was a 3-year-old colored
female whose parents gave a history of what
were probably grand mal convulsions.
88% These 3 patients negate the idea that
con-vulsions never occur in mongoloids.
No gross defects in hearing were noted
in our series.
Defects in vision were far more frequent
than in normal children, but as no accurate
evaluation of their vision was made, no
figures can be given as to type.
FAMILY HISTORY: The family history also
revealed great variability, particularly with
respect to age of the mother at the birth of
the mongoloid child and the order of the
birth. Chart X shows the distribution of the
ages of the mothers at the birth of the
mongoloid children. With the exception of
the thirty-third year, every other year
be-tween 19 and 42 is represented among these
mothers. However, 64 per cent of the
moth-ers were 30 years and over, whereas only
36 per cent were younger than 30 years.
Chart XI shows the distribution of the
patients according to their order of birth.
Although the number of patients is small it
is evident that a mongoloid child may be
the first, second, third, etc.
Physical Examination (Charts Xli, Xlii, XIV)
A glance at the charts convinces one of
the great variability among mongoloids.
Not one of the mongoloid characteristics
occurred in every child in this series.
Al-though the 50 children were not all of the
same age, the results are illuminating.
HEAD (Chart XII): The “globular”
(brachycephalic) skull with a flat occiput
is very characteristic of mongolism but
it occurred in only 82 per cent of the
pa-tients.
Delayed closure of the anterior fontanel
is also considered a characteristic of
mon-golism. Among this group we found an open
TRUNK
A. NECK
I BROAD
2 SHORT
B CHEST I FUNNEL CREST
2 PiGEON BREAST
3 FLAT NiPPLES
4 HEART MURMUR
5 DORSOLUMBAR KYPHOS1Z C. ABDOMEN
I DIASTASIS RECTI
2 UMBiLICAL HERNIA
D. GENITALIA 1 SMALL PENIS
2 CRYPTORCHISM
3 SMALL SCROTUM
50% SO.,. 12% 14% 56% 26% 14% 76% .4%
50% (OF MALES) - 2O%(OV MALES)
42 %(0’ MALES)
28% 32% EXTREMITIES A. GENERAL 1 ACROCYANOS1S 2 MARMORATION
3 HYPEREXTENSIBLE JOINTS
4 HYPOTONIC MUSCLES 88%
B HANDS
1 SHORT AND BROADHANDS 74%
2 FLABBY HANDS 84%
3 PALMAR RORIZONTALLINF.S 48%
4 SHORT FINGERS 70%
5 TAPERING FINGERS 52%
6 SHORT 5TH FINOER 66%
7 CURVED 5THF1NGER 68%
8 ONLY I FLEXIOW FURROW - 100/.
IN 3 TR FINGER
50 LEVINSON - VARIABILITY OF MONGOLISM
were below 1% years of age, until which
time the fontanel is normally open,
leav-ing 16 per cent of the total as pathologically
open fontanels. The ages of these children
varied from 1% to 5 years. It is interesting
to note that all the patients below 23 years
of age had open anterior fontanels and all
patients above 5 years had closed fontanels.
Of those between 2% and 5 years, 80 per
cent had closed fontanels and 20 per cent
had open fontanels, again illustrating the
variability in the delay in fontanel closure.
Red (66 per cent), rough and scaly (74
per cent) cheeks are frequent but
incon-stant. Wrinkled forehead was present in
14 per cent of our cases.
Among the most characteristic signs of
mongolism, the eye signs certainly deserve
the place of distinction, but even among
these great variability was the rule.
Slanting of the palpebral fissures, which
is considered by many as synonymous with
mongolism, occurred in only 88 per cent
of the patients, which proves that
mongol-ism may occur even without slanting.
Epicanthus was present in 50 per cent of
the patients and, contrary to accepted
opinion, we found no correlation between
epicanthus and the age of the patient, the
oldest being 10 years 9 months. Blepharitis
to a greater or lesser degree was present in
38 per cent of patients in spite of frequent
use of antibiotics. Strabismus was present
in 7 patients (14 per cent), 6 of the
con-vergent type and one divergent. Nystagmus
was found in 14 per cent of patients and
was of the horizontal type in every case.
Speckling of the iris or Brushfield spots
and double-zoned iris which are supposed
to be among the most characteristic features
of mongolism were present in only 30 per
cent and 22 per cent of patients,
respec-tively.
The ear anomalies most frequently found
were the small or absent lobule which
oc-curred in 80 per cent of the patients. In
48 per cent the ear was considered
de-formed. The ears were prominent in 50 per
cent and flat in 50 per cent.
C FOOT
I GAPBETWEENTOES1AWD2 44%
2 TOE 3LONGERTHANTOEL 0%
PLANTAR FURROW 28%
CHART XIII (Upper). CHART XIV (Lower).
There was nothing characteristic about
the nose in this series.
The mouth signs are also believed to be
among the most characteristic features of
mongolism, but here, again, the inconstancy
of these signs was obvious at a glance.
High-arched palate occurred in only 74 per
cent and irregular alignment of the teeth
in 68 per cent.
TRUNK (Chart XIII): Signs of mongolism
referrable to the trunk are rather few in
number but some of them are important
in diagnosis. Flat nipples occurred rather
frequently (56 per cent). Heart murmurs
were present in 28 per cent, with pathology
varying from simple septal defects to a case
DISTRIBUTION OF CASES ACCORDING TO TYPE OF ELECTROENCEPHALOGRAM
ZO CABES
NORMAL BORDER- MULTIPLE BSPAR1ETAL
AWAKE AND LINE SEIZURE SLOWING
ASLEEP NORMAL FOCI
CHART XV.
FAST (Fi)
HYPSARYTh-MIA
ORIGINAL ARTICLES
before coming under our observation.
Diastasis recti was found in 76 per cent.
The frequency of this sign is probably
re-lated to muscular hypotonia, the basis of the
signs of mongolism in the extremities.
EXTREMITIES (Chart XIV): Even the 2
most frequent signs, hyperextensible joints
and flabby hands, were inconstant, occurring
in 88 per cent and 84 per cent of the
pa-tients, respectively. Other signs were even
less constant. The short, broad type of hand (74 per cent), the short fingers (70 per cent),
and the short (66 per cent) and curved (68
per cent) little finger are far more
charac-teristic than the palmar horizontal line
which was found in only 48 per cent of
these patients. In only 20 per cent was the
palmar horizontal linebilateral while in the
other 28 per cent it was unilateral (12 per
cent in the right hand, 16 per cent in the
left).
Eiectroencephaiographic Examination
(Chart XV)
Electroencephalograms of 42 mongoloid
children further emphasized the great
varia-bility in this disorder. Monopolar
record-ings, activated by sleep, were made on each
patient at the Consultation Clinic for
Epi-lepsy, University of Illinois. Eighteen of the
tracings were normal, awake and asleep.
Ten were classified as “borderline,” because
1
14 &6 SEC.
POSITiVE:
SPIKES
these records were slightly slower than
nor-mal. The slowing was maximal in the
parietal and frontal areas. Due
considera-tion was given to the individual patient’s
age and the fact that infants normally have
slower records than older children. Six of
the patients showed multiple seizure foci
but it was interesting to note that no history
of seizures or convulsions was obtained in
any of these particular patients.
Previous electroencephalographic studies
on large numbers of children with mental
retardation or cerebral palsy have shown a
high incidence of seizure activity in the
electroencephalograms even though many
have not had clinical seizures. Forty-two of
the mongoloid children in this series had
electroencephalograms; only 3 of these had
histories that might be interpreted as
in-dicative of seizures in the past. One child
was reported as having had a questionable
convulsion at the age of 1 year. The
de-scription of another child’s spells suggested
breathholding attacks. The child with the
hypsarrhythmia record was having many
short “infantile spasms” daily but never any
grand mal convulsions. The EEG in 5 of
the patients showed an atypical high
volt-age, 3 to 5 per second, steady slowing in
the parietal areas. This is a rather unusual
electroencephalographic finding and an
52 LEVINSON - VARIABILITY OF MONGOLISM
with motor, speech, or intellectual
retarda-tion in the children. No such correlation
could be found. The child with the
hypsar-rhythmia in her electroencephalogram had
an I.Q. of 10 and was having many short
spasms daily. This is in keeping with
pre-vious studies as presented by Gibbs, et a!.,
who found that a high percentage of
chil-dren with hypsarrhythmia patterns were
mentally retarded.
The present study shows that there is no
specific or characteristic
electroencephalo-graphic abnormality in mongolism. The
most usual finding in monogolism is a
nor-mal electroencephalogram. Different types
of seizure discharges, except for petit mal,
and slow and fast patterns suggesting
dif-fuse and localized cortical dysfunction of
an epileptic type occur in one-third of the
patients. These abnormalities are not a
nec-essary concomitant of mongolism, and in
this group they do not correlate with a
his-tory of seizures or damage to the brain.
DISCUSSION
Most physicians seem to believe that
mongolism not only has a constant clinical
picture with definite physical and
develop-mental characteristics, but that there can
be no question of degree of mongolism or
transitional forms between classical
mongol-ism and the normal state.
The origin of this opinion is found in
the views of early writers who were
im-pressed by the apparent similarity between
mongoloids and repeatedly stressed such
similarity.
Langdon Downs who was the first to
describe the condition, in 1866, termed it
‘Mongolian Idiocy,” which put these
pa-tients down as having the physical
appear-ance of Mongols and the mental behavior
of idiots. This view has persisted to the
present day in many quarters and is
re-flected in the terminology which has not
changed since. Even modem authors, e.g.,
Ford,#{176}still use the term “mongolian idiocy”
in their description of the condition.
Actually, the term “mongolian idiocy” is
a misnomer since only a relatively small
proportion of mongoloids are in the
so-called “idiot” range of I.Q. values. The
large majority are, as a matter of fact, in
the “imbecile” range. This has been pointed
out by many, including Ford himself.
The physical similarity among
mongol-oids has been exaggerated to such an
ex-tent that some actually believe that parents
of mongoloids may have difficulty in
recog-nizing their own child in a group of
mon-goloid children. Kroemer1#{176} (1928),
Wey-gandt’1 (1936), and Marfan12 (1926)
sub-scribed to the same opinion by stating that
mongoloids resemble each other physically
to such an extent that “when you have seen
one, you have seen them all.”
The data presented in this paper, we
feel, shows that there is a wide range of
variability in every single physical and
de-velopmental characteristic of mongoloids as
well as in the sum total of such
character-istics in each individual case.
We, of course, concur in the general
opinion that a great degree of similarity
exists among mongoloids. However, we are
convinced that mongoloids differ from one
another at least to the same degree that
normals do, and, in many respects to even
a far greater extent.
SUMMARY AND CONCLUSIONS
A detailed study of 50 children with
mon-golism was conducted with the objective
of evaluating the variability in their
char-acteristics. The data presented demonstrate
that none of the developmental
character-istics or physical features are constant.
Great variability exists in the frequency of
occurrence as well as in the extent and
de-gree of all the mongoloid characteristics.
The electroencephalogram also shows
con-siderable variability.
REFERENCES
1. Benda, C. D.: Mongolism and Cretinism,
2nd Ed. New York, Grune & Stratton, 1949.
2. Oster,
J.:
Mongolism. A Clinicopathologi-cal Investigation Comparing 526Neighbor-ORIGINAL ARTICLES
ing Islands in Denmark. Copenhagen,
Danish Science Press, Ltd., 1953.
3. Friedman, A. : Radioiodine uptake in
chil-dren with mongolism. PEDIATRICS, 16:
55, 1955.
4. Dunham, E. C. : Premature Infants; A
Manual for Physicians. Children’s Bur-eau Publication. No. 325, 1948.
5. Levinson, A., and Goldenberg, C. : Mental
retardation in children. J.A.M.A., 152:
781, 1953.
6. Bakwin, H., and Bakwin, R. M. : Clinical
Management of Behavior Disorders in
Children. Philadelphia, Saunders, 1953.
7. Gibbs, E. L., et al.: Diagnosis and progno-sis of hypsarhythmia and infantile spasms. PEDIATRICs, 13:66, 1954. 8. Down,
J.,
Langdon, H.: Observations inethnic classification of idiots. London
Hosp. Rep., 3:259, 1866.
9. Ford, F. R.: Diseases of the Nervous
Sys-tem in Infancy, Childhood and
Adoles-cence, 3rd Ed. Springfield, Thomas,
1952, p. 270.
10. Kroemer, F.: Zur Frage des Mongolismus
und endokrin bedingter geistiger
Sto-rungen bei Kindern. Allg. Ztschr.
Psy-chiat., 88: 198, 1928.
11. Weygandt, W.: Der jungendliche
Schwach-sinn. Stuttgart, 1936.
12. Marfan, A. B.: L’imb#{233}cillit#{233}mongolienne. Presse med., 34:1377, 1926.
SPANISH ABSTRACT
Variabilidad
del
Mongolismo
Existe el concepto general de que los ni#{241}os
mong#{243}licos con identicos y se desarrollan
den-tro del mismo patron. Los autores presentan
un estudio sistem#{225}tico de 50 pacientes con el
fin de analizar sus caracteristicas fIsicas y
mentales y resumen los resultados hallados,
con relaci#{243}n a embarazos maternos, edad, sexo,
raza, nacimiento, perlodo neonatal, desarrollo
fIsico y mental, sIntomas especiales (convul-siones, defectos visuales, etc.), y antecedentes
familiares de los niflos.
Los datos que obtuvieron de cada una de estas caracteristicas variaron tan ampliamente en grado y frecuencia que los obligan a sostener
que ninguna de las caracterIsticas fIsicas y de
desarrollo se pueden considerar como constante en el nino mong#{243}lico. Por ejemplo el factor
racial no intervino en el determinismo del
padecimiento; los ni#{241}oslevantaron la cabeza desde los dos meses hasta los dos aflos y medio de edad; unos comenzaron a caminar al aflo de
edad y otros a los cuatro o cuatro y medio
aflos; el 64% de las madres era mayores y el
36% menores de 30 aflos de edad. Los autores
no desconocen Ia veracidad de la impresi#{243}n
general de que los pacientes mong#{243}licos pre-sentan un alto grado de similaridad pero con-sideran que difieren entre si por lo menos en el mismo grado que entre ellos los sujetos normales y en muchos aspectos ann en forma m#{225}sdefinida.
INTERLINGUA ABSTRACT
Variabilitate
de Mongolismo
Le objectivo del presente studio es evalutar le variabilitate del characteristicas incontrate in
infantes con mongolismo Le resultatos denega
le notion traditional del alte grado de con-stantia in ille characteristicas.
Le material del studio consisteva de 50
con-secutive casos de mongolismo incontrate al
clinica del Fundation de Recerca Dr. Julian D.
Levinson pro Infantes a Retardation Mental in Chicago.
Le datos compilate coperiva le curso del
pregnantia, le nascentia, le periodo neonatal,
le historia familial (includente le etate del
parentes e le rango del patientes in le sequentia
de br fratres e sorores), le crescentia del
in-fante, su disveloppamento motor e lingual,
le symptomas (con attention special a
convul-siones, defectos auditori e visual, dextero- o sinistromanitate, e infectiones
superorespira-tori), un exacte examine physic (specialmente
in re le tractos que es communmente
con-siderate como typic in casos de mongolismo),
un complete evalutation psychologic, e studios
laboratorial (includente roentgenogrammas del
cranio, del carpo, e del longe ossos, como
etiam electroencephalogrammas, e determina-tiones del absorption de radio-iodo).
Le datos psychologic-includente
explora-tiones del intelligentia, del maturitate social, e del tractos characterologic-e le
determina-tiones del absorption de radio-iodo forma Ie
themas de duo separate publicationes.
In reguardo a omne factores studiate le
serie hic discutite exhibiva un grande variabili-tate.
Vinti pro cento del casos esseva nascentias
prematur, comparate con 7 a 1 1 pro cento in
le population de infantes normal e 19 pro cento
inter le infantes a mentalitate retardate al
repre-54 LEVINSON VARIABILITY OF MONGOLISM
senta circa un tertio del patientes total ‘idite al mesme clinica.
Nulle del characteristicas frequentemente designate como typicamente mongoloide
oc-curreva in omne membros del serie. Per
exemplo, cranios brachycephalic con occipite plan occurreva in 82 pro cento del casos;
fonta-nellas pathologicamente aperte esseva presente
in solo 16 pro cento del casos; e obliquitate del rima palpebral characterisava 88 pro cento del casos.
Le erronee conception de mongolismo como
un phenomeno invariabile e totalmente
dis-proviste de gradationes e transitiones al stato
normal ha supervivite depost Langdon Down
qui presentava in 1866 le prime description
de iste condition e introduceva pro illo le
nomine de “idiotia mongolian.” Iste designa-tion se trova ancora in nostre dies ben que ii es
cognoscite que solo un parve procentage del
mongoloides es technicamente idiotas.
Le autores non denega que il existe un
grande similaritate inter le mongoloides, sed
illes insiste que br datos demonstra que ille
