Spectrographic analysis of cries prom children with maladie du cri du chat

V. VUORENKOSKI, J. LIND, T. J. PARTANEN, J. LEJEUNE, J. LAFOURCADE and O. WASZ-HÖCKERT

Ann. Paediat. Fenn. 1966:12, 174-180.

Résumé :

• Material.


• Results


• Discussion


• Annexes

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.

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Material.

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 Table 1.

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 cats.

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

13. Continuity

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.

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Results

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 sec.).

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.

Fig. 1 - Cri du chat signal - case 1.

Fig. 2 - Cri du chat signal - case 2.

Fig. 3 - Cri du chat signal - case 3.

Fig. 4 - Cri du chat signal - case 4.

Fig. 5 - Cri du chat signal - case 5.

Fig. 6 - Cri du chat signal - case 6.

Fig. 7 - Cry of a young cat.

Fig. 8 - Pain signal of a normal infant.

Fig. 9 - Pain signal of a normal infant.

Fig. 10 - Hunger signal of a normal infant.

Fig. 11 - Hunger signal of a normal infant.

Fig. 12 - Hunger signal of a normal infant.

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Discussion

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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References

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