The major symptom which has attracted interest to the syndrome Maladie
du cri du chat is indicated by its name. This symptom is the very peculiar type
of cry, reminiscent of the cry of a cat, occurring specially during early
infancy. The cases reported in the last years (1, 2, 3, 4, 6, 7, 10, 11, 12)
have, except for the typical cry, mental and physical retardation as main
symptoms. Other symptoms are: microcephaly, moan face, hypertelorism,
epicanthus, non-mongoloid slant of palpebral fissures, laryngeal abnormalities,
low-set ears and abnormal dermatoglyphics.
The first cases of Maladie du cri du chat have been reported in 1963
by Lejeune et al. (6). They explained this disease as a deletion of the short
arm of a chromosome in the 4-5 group, which has been designated as number 5.
Today, 61 cases with similar clinical features, chromosome findings and
cat-like cry have been reported from several countries, and a suggestion has
been made that as the clinical ramifications of the syndrome become more widely
recognized, many more cases will be diagnozed (10).
When Lejeune et al. reported their series consisting of 8 cases at the
XI International Congress of Pediatrics in Tokyo 195 (5), a suggestion was made
to perform a cooperative study to analyse spectrographically the cry produced
by infants with this disease. The intention was to determine the specificity of
the spontaneous crying of these cases which could serve as diagnostic-tool.
Recordings of spontaneous crying of 8 cases with the syndrome Maladie
du cri du chat have. been included in this study. The clinical findings and the
chromosome analyses are reported elsewhere (5). A total number of 4-1
different, cry signals of these cases have been analyzed. The number of the
cries analyzed and the age of each case on the recording day are presented in
The normal reference material consisting of 75 spontaneous (hunger)
and 60 pain signals from 105 infants, analyzed and published in previous papers
(8, 14), have been used. For the division of this material according to age,
see Table 2.
Additionally, same recordings have been made from newborn and young
The cry material was collected on magnetic cape and recorded on a
sound spectrograph (Sona-Graph 661 A, made by Kay Electric N. J.) as reported
elsewhere (8, 9, 14). The spontaneous cries of both the case and normal
material were obtained before the meal; thus these cries heve also been called
hunger cries. As the emotional situation of the baby with the syndrome Maladie
du cri du chat cannot be compared without consideration of that of the normal
infants, a second normal cry type has been used for comparison, namely pain.
The pain cry recordings have been made in connection with BCG or PDT
vaccination or after pinching the infant's arm (8).
The following variables were measured from sound spectrograms and
included in the material:
1. Length, sec.
2. Minimum pitch, cps
3. General pitch, cps
4. Maximum pitch, cps
5. General form of melody
6. Occurence of subharmonic break
7. Occurence of shift
8. Occurence of vocal fry
9. Occurence of glottal plosives
10. Occurence of nasality
11. Occurence of tenseness
12. Occurence of inspiration-expiration phona-tion
For détails about these variables and technique of measurement, see
Wasz-Höckert et al. (14). The
data obtained from these measurements were statistically analysed with
computers (Computations were carried out with programs and computers of the
Medical Computing Center of the College of Medicine at the University of
Cincinnati, Cincinnati, Ohio). In order to find significant differences between
cri du chat and normal reference material, two different analyses were
performed: the first was concerned with discrimination between cri du chat and
normal pain signal, the second between cri du chat and normal hunger signal. A
method (13) was used that searched for the best discriminating sets of the
variables studied. The screening of the variables took place by the criterion
of the joint predictability of the dependent, variable (cri du chat - normal)
provided by the different sets of independent variables. The predictability
figures are sample estimates of correct classifications, multiplied by 100.
Table 1. - Cri du chat material: number of spontaneous cry
analyses and age of different cases
|Case||Number of Analyses||Age of Case,
Table 2. - Normal material: division according to cry
situation and age
|Situation||1-3 Months||4-5 Months||6-7
The typical spontaneous cri du chat signal is characterized by the
following acoustical values in comparison with normal spontaneous (hunger) and
pain cry signals (see Table3) :
The average length of the cri du chat signal (2.6 sec.) is practically
equal to that of the normal pain cry, but the s.d. value is greater in the
former (1.6). The average length of the normal hunger cry is shorter (1.2
All the pitches are very high (general pitch 860 cps, maximum pitch
1.030 cps, minimum pitch 750 cps) and there is a remarkable difference with the
normal material - both normal pain and hunger signals have lower values. The
shift does not occur in cri du chat or normal hunger cry signals, while it is
very common in normal pain cries (.83). The subharmonic break is also typical
for normal pain cry (.62), it occurs seldom in cri du chat (.09) and normal
hunger cry signals (.03).
Flat melody form is most common in cri du chat signals (.36); in our
normal cry material only birth and pleasure signals have a corresponding amount
of flat melody form (14). The next common melody form in cri du chat signals is
rising (.23); only .02 of normal pain signals are of this form, and none of the
hunger signals. One of the cri du chat signals has a very unusual
falling-rising melody form. - The most common melody form in normal hunger
signals is rising-falling (.69), in pain signals falling (.58).
The cri du chat signals are voiced (.84), tense (.95) and oral (1.00)
and seldom joined to glottal plosives (.20).
Two phenomena occuring in some cri du chat signals are not found in
normal cry material used in this study:
1) Dichotomous signal: the phonation stops for a short interval
(0.4-0.6 sec.) and continues without any glottal attack (.23).
2) Inspiration-expiration signal: one part of the signal is phonated
during the inspiration, the other during the expiration (18).
In f igures 1-6 there are examples of cri du chat signals, while fig.
7 presents a cry of a real cat. Normal pain signals are seen in figures 8 and
9, and normal hunger signals in figures 10-12 (In tape recording of the cry
samples presented in figs. 1-2 will be supplied by request.
The signals in figures 1, 3, 4 and 5 are of flat form, signal 2 is
falling-rising, signals 6, 7 and 8 rising, signal 9 falIing and signals 10-11
rising-falling. In signal 12 there are only glottal plosives. Signals 2, 3 and
4 are dichotomous, and signal 2 is at the same time with inspiration-expiration
phonation. Signals 9 and 11 are half-voiced and the former one contains also a
shift. Subharmonic break can be seen in figures 1 and 10.
Comparing the cri du chat signals with normal ones it can easily be
seen, that the former ones are all higher in pitch.
From the small material of cries of real cats analyzed (q cries from 5
cats) several similarities can be found with signals of cri du chat cases,
specially when taping account of younger cats (1-3 months). The cries of young
cats have an equal pitch (600-1.200 cps) and melody form (rising or flat). It
is evident that these are shorter than the signals of cri du chat cases, but a
short cri du chat signal can shoow an amazing identity with the cry of a young
cat (see Figs. 6 and 7).
Discrimination between cri du chat and normal signals: In terms of
predictability, number of observations and number of variable subcategory
combinations, best discrimination was obtained by using one single variable,
namely minimum pitch. Table 4 illustrates the relationship. If minimum pitch is
equal to or higher than 500 cps, the signal is very probably a cri du chat;
otherwise it should be classified as a normal pain signal. This rule results in
correct classifications in 97.1 per cent of the cases, as estimated from the
information provided by the sample. No other single variable reached this level
of predictability. Using more than one independent variable simultaneously, the
predictability was higher than 97.1 per cent in some combinations. However, the
improvement was slight, and, considering the number of observations and the
number of variable subcategory combinations involved, it was nonexistent.
The other single predictors that resulted in predictability of more
than 80 per cent are lined in table 5 along with their respective decision
rules and predictability percentages.
In the next best discriminator to general pitch, the predictability
dropped more than 6 per cent, and, consequently, the probability of
misclassification rose to over 26 per cent.
The best discriminator between cri du chat and hunger signal was again
a single variable, and again minimum pitch (Table b).
Its predictability was 91.5 %, the classification being normal hunger
signal, if minimum pitch is not measurable or less than 500 cps; otherwise cri
du chat. No combination of independent variables improved the predictability
from this level. There was another variable that provided a predictability
above 80 per cent, namely, general pitch. The decision rule for it is :
Not measurable or less than 600 cps normal : hunger;
Equal or more than 600 cps : cri du chat;
resulting in 87.2 per cent predictability.
Table 3. - Means, medians and percentages of different
variables in the groups of normal hunger and pain signals and spontaneous
signals from children with maladie du cri du chat
Material||Cri du chat
|Pain 60 Cries||Hunger 75 Cries||8 Babies 44
|Length, sec. ||2.7||1.2||2.6
|Stand, déviation ||1.1||0.6||1.7
|Général pitch, cps||530||500||860
|Stand, déviation ||80||70||165
|Maximum pitch, cps ||680||520||1
|Stand, déviation ||80||80||200
|Minimum pitch, cps ||380||420||750
|Stand, déviation ||70||70||165
|Occurrence of shift, % ||83||
|Pitch of shift, cps ||910||
|Occurrence of subharm break, %
|Rising-falling, % ||10||69||5
|Falling, % ||58||3||16
|Rising, % ||2||23
|Flat, % ||3||36
|Falling-rising, % ||2
|Interrupted, % ||27||28||18
|Occurrence of nasality, % ||25||
|No glottal plosives, % ||72||31||
|Glottal plosives, % ||28||54||20
|Oniy glottal plosives, % ||15||
|Occurrence of vocal fry, %
|Occurrence of tenseness, %
|Dichotomous signal, % ||23
|Expiration-inspir signals, %
|Voiceless signals, % ||8||9||
|Half-voiced signals, % ||20||23||16
|Voiced signais, % ||72||68||84
Table 4. - Distribution of minimum pitch in normal pain and
cri du chat signals
|Minimum Pitch||Normal pain||Cri du
|< 500 cps||55||1||56
|> 499 cps||2||43||45
Table 5. - Classifications based on vocal fry and general
|Vocal fry||Presence : normal pain||84.4
|Absence : cri du chat
|General pitch ||< 600 cps : normal
|> 599 cps : cri du chat
|Not meas. : cri du chat
Table 6. - Distribution of minimum pitch in normal hunger and
cri du chat signals
|Minimum Pitch||Normal hunger||Cri du
|< 500 cps||54||1||55
|> 499 cps||10||43||53
Fig. 1 - Cri du chat signal - case 1.
Fig. 2 - Cri du chat signal -
Fig. 3 - Cri du
chat signal - case 3.
4 - Cri du chat signal - case 4.
Fig. 5 - Cri du chat signal - case 5.
Fig. 6 - Cri du chat signal -
Fig. 7 - Cry of a
Fig. 8 - Pain
signal of a normal infant.
Fig. 9 - Pain signal of a normal infant.
Fig. 10 - Hunger signal of a normal
Fig. 11 - Hunger
signal of a normal infant.
Fig. 12 - Hunger signal of a normal infant.
The validity of the results of the present study is lowered by the
fact that there were only 8 cases with cri du chat disease in the sample, the
total number of 44 cri du chat signals having been obtained by multiple
recordings of the same infants. In other words, the total variability in the
present case sample is partly interindividual, partly intraindividual.
However, it has been shown that the spontaneous cry produced by
infants with the chromosome disease known as Maladie du cri du chat is typical
and specific, mostly for its relatively constant high pitch and voiced, tense
phonation. The dichotomous and inspiration-expiration cry types found in this
material are of special interest.
The cries analyzed in this study are different from the normal
material and no resemblance has been found with other pathological cry material
studied by the group. On the other hand, it was interesting to note
similarities between the cri du chat signals and cries of young cats. This
observation shows that the name given to this syndrome is pertinent.
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