CS Ferrari2002
CS Ferrari2002
CS Ferrari2002
Objective: To ascertain whether specific abnormali- worse was the neurological outcome. Transient cramped
ties (ie, cramped synchronized general movements synchronized character GMs (8 cases) were followed by
[GMs]) can predict cerebral palsy and the severity of later mild cerebral palsy (fidgety movements were absent) or
motor impairment in preterm infants affected by brain normal development (fidgety movements were present).
lesions. Consistently normal GMs (13 cases) and poor repertoire
GMs (30 cases) either lead to normal outcomes (84%) or
Design: Traditional neurological examination was per- cerebral palsy with mild motor impairment (16%). Ob-
formed, and GMs were serially videotaped and blindly servation of GMs was 100% sensitive, and the specificity
observed for 84 preterm infants with ultrasound abnor- of the cramped synchronized GMs was 92.5% to 100%
malities from birth until 56 to 60 weeks’ postmenstrual throughout the age range, which is much higher than the
age. The developmental course of GM abnormalities was specificity of neurological examination.
compared with brain ultrasound findings alone and with
findings from neurological examination, in relation to the Conclusions: Consistent and predominant cramped syn-
patient’s outcome at age 2 to 3 years. chronized GMs specifically predict cerebral palsy. The
earlier this characteristic appears, the worse is the later
Results: Infants with consistent or predominant (33 cases) impairment.
cramped synchronized GMs developed cerebral palsy. The
earlier cramped synchronized GMs were observed, the Arch Pediatr Adolesc Med. 2002;156:460-467
I
N THE PAST 20 years, there has been identification of cerebral palsy in very
a dramatic reduction in neonatal young infants is extremely difficult.10 It is
mortality of low-birth-weight and generally reported that cerebral palsy can-
very low-birth-weight infants but not be diagnosed before several months af-
a relative increase in the inci- ter birth11-15 or even before the age of 2
dence of cerebral palsy among children years.16 A so-called silent period, lasting
with low birth weight and short gesta- 4 to 5 months or more, and a period of un-
tion.1-4 An increased survival rate is associ- certainty until the turning point at 8
From the Institute of ated with an increased proportion of in- months of corrected age have also been
Paediatrics and Neonatal fants with cerebral palsy, and it has been identified.12,13 The neurological symp-
Medicine, University of
suggested that the reduction in neonatal toms observed in the first few months af-
Modena, Italy (Drs Ferrari,
Roversi, and Ranzi); the mortality and the concomitant relative in- ter birth in preterm infants who will de-
Institute of Developmental crease in cerebral palsy might be associ- velop cerebral palsy are neither sensitive
Neurology, Psychiatry, and ated5 because the prevalence of cerebral nor specific enough to ensure reliable prog-
Educational Psychology, palsy rises sharply the lower the weight of noses. Irritability, abnormal finger pos-
University of Pisa, and the the infant at birth.6-8 Cerebral palsy occurs ture, spontaneous Babinski reflex,17,18
Stella Maris Foundation, in 8% to 10% of very preterm babies, and weakness of the lower limbs,19 transient
Calambrone, Pisa, Italy approximately 40% of all children with ce- abnormality of tone,12,13,20-24 and delay in
(Drs Cioni and Paolicelli); rebral palsy were born preterm.8,9 achieving motor milestones11 are some of
the Department of Physiology, the neurological signs that have been de-
University of Graz, Austria
(Drs Einspieler and Prechtl);
For editorial comment scribed in these high-risk preterm in-
and the Department of see page 422
Paediatrics, Beatrix Children’s
An early prediction of cerebral palsy See the enhanced version at
Hospital, University of
will lead to earlier enrollment in rehabili- http://www.archpediatrics.com
Groningen, Groningen,
the Netherlands (Dr Bos). tation programs. Unfortunately, reliable
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460
fants. All these symptoms may be encountered before the baby. More than 10 years ago, Prechtl et al27-29 intro-
onset of cerebral palsy or during “transient dystonia,”21 duced a new approach to neurological evaluation based
dissociated motor development,25,26 and other transient on spontaneous motor activity, rather than reflexes and
neurological disturbances,23,24 which disappear during the evoked responses. Theoretical and empirical consider-
first or second year of life. Moreover, no correlations have ations suggest that the quality of endogenously gener-
been found among any of these symptoms and the se- ated motor activity is a better indicator of neural func-
verity of future motor impairment. Therefore, tradi- tion integrity than many items in the neurological
tional neurological examination fails to predict the de- examination.27 In fact, fetuses and newborn infants
velopment and severity of cerebral palsy.10,16 exhibit a large number of endogenously generated
Neurological examination of newborns and young motor patterns, which are produced by central pattern
infants is mostly based on the study of neonatal generators located in different parts of the brain. More-
reflexes, such as grasping, Moro reflex, rooting, and over, substantial indications suggest that spontaneous
tonic asymmetric response and evoked responses, such activity is a more sensitive indicator of brain dysfunc-
as those involved in passive and active muscle tone. tion than reactivity to sensory stimuli in reflex testing.
Little attention is paid to the spontaneous activity of the Various studies29-40 have demonstrated that in newborn
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infants affected by different brain lesions, spontaneous of GMs of fidgety character, or fidgety movements, at 47
motility does not change in quantity, but it loses its to 60 weeks’ postmenstrual age has been shown to be a
elegance, fluency, and complexity. General movements high-validity predictor of future neurological impair-
(GMs) have been selected from among the whole reper- ment, specifically cerebral palsy.34
toire of spontaneous motor patterns because of their A collaborative study with a large cohort of high-
complexity and frequent occurrence. A range of abnor- risk preterm infants was specifically designed to achieve
malities in the quality of GMs, such as hypokinesis, a better understanding of the relationship among cramped
poor repertoire, abnormal or absent fidgety movements, synchronized GMs, later cerebral palsy, and the severity
and chaotic and cramped synchronized GMs, have been of functional impairment. More specifically, 3 main ques-
described. Visual gestalt perception is a powerful and tions were addressed. First, are cramped synchronized
reliable instrument for detecting these alterations in the GMs an early and specific marker of later cerebral palsy?
complexity of movements. Cramped synchronized char- Second, is the emergence and development of cramped
acter, the most severe motor abnormality, has been synchronized GMs somehow related to the severity of ce-
found to be predictive of severe neurological im- rebral palsy? And third, are GMs as powerful a prognos-
pairment.29,32,34,35 Recently, the absence or abnormality tic tool as ultrasound abnormalities alone and tradi-
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*GMH-IVH indicates germinal matrix−intraventricular hemorrhage, graded according to Volpe42; CP, cerebral palsy, graded according to Palisano et al.49
Table 3. Types of Developmental Trajectories and Table 4. Fidgety Movements (FMs) and Neurological
Neurological Outcome in 84 High-Risk Preterm Infants* Outcome in 84 High-Risk Preterm Infants
0.50
25
0.25
No. of Infants
20
15
0 0.25 0.50 0.75 1.00
1-Specificity
10
Figure 3. The area under the receiver operating characteristic (ROC) curve
for quality of general movements (GMs) and ultrasound (US) scans in
high-risk preterm infants. The ROC curve is generated by plotting the
5 proportion of true-positive results against the proportion of false-positive
results for each value of a test. The curve for an arbitrary test (AT) that is
expected to have no discriminatory value appears as a diagonal line, whereas
0 a useful test has an ROC curve that rises rapidly and reaches a plateau.
Consistent N Transient PR Consistent PR Transient CS Predominant CS Consistent CS
Sample 13 4 26 8 19 14
Size
that of neurological examination throughout the age range.
Figure 2. Types of developmental trajectories and neurological outcome in The negative likelihood ratio for predicting cerebral palsy
84 high-risk preterm infants. N indicates normal movements; PR, poor based on GM quality was also much higher than that for
repertoire; and CS, cramped synchronized. neurological examination during the study period.
The GM observation was 100% sensitive through-
LIKELIHOOD RATIO AND ACCURACY OF GMs out the age range; neurological examination was less sen-
AND NEUROLOGICAL EXAMINATION sitive during the study period. The sensitivity of cramped
IN PREDICTING CEREBRAL PALSY synchronized GMs was low (46.5%) in the preterm pe-
riod because of those infants with cramped synchro-
The positive likelihood ratio of cramped synchronized nized GMs preceded by poor repertoire GMs. Sensitiv-
GMs for predicting cerebral palsy is much higher than ity rose to 65%, 78.7%, and 77.2% at term, early postterm
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Age Period
period, and fidgety age, respectively, because of pre- new. A detailed analysis of the developmental trajecto-
term infants who developed cerebral palsy after tran- ries of GMs in a large group of preterm infants affected
sient cramped synchronized GMs or consistent poor rep- by major or minor abnormalities detected by ultra-
ertoire GMs. Even more striking was the difference in sound demonstrates that the consistent or predominant
specificity when cramped synchronized GMs were com- cramped synchronized character of GMs, irrespective of
pared with neurological examination. The specificity of the severity of the ultrasound abnormalities, is always fol-
the former was extremely high (92.5% to 100%) for all lowed by cerebral palsy.
age ranges; it was invariably much higher than that af- The study has practical and obvious implications.
forded by neurological examination (Table 5). It offers clues about the selection criteria for a strict neu-
rological follow-up. Preterm infants who are waiting for
COMMENT a definite diagnosis of brain integrity are usually en-
rolled in prospective neurological follow-up programs
Two major findings emerge from this study. First, the based on their clinical history and the ultrasound find-
cramped synchronized character of GMs, if consistent in ings rather than a functional assessment, which in-
time or predominant from preterm birth to 5 months’ post- volves the evaluation of mental and motor perfor-
term age, specifically predicts later cerebral palsy. Sec- mance. Recent studies 53-55 have shown that mental
ond, the time of appearance of cramped synchronized GMs retardation and learning deficiencies are common among
predicts the degree of later functional impairment caused preterm infants tested at school age. We are not sure
by cerebral palsy: the earlier the appearance, the more whether normal, or only mildly abnormal, GMs at these
severe the functional impairment. early ages exclude these minor deficiencies at a later age.
Our study also confirmed observations from previ- This study stresses the importance of functional assess-
ous investigations.32,52 Normal fidgety movements fol- ment based on early observations of spontaneous motor
lowing transient abnormalities of GM quality point to a behavior. The normal quality of GMs identifies those in-
normal outcome, absence of fidgety movements sug- fants who are not affected by brain dysfunction and who
gests a neurological deficiency, and GMs are a more pow- will develop normally; they do not need strict neurologi-
erful prognostic tool than traditional neurological ex- cal surveillance. In contrast, prolonged cramped syn-
amination and ultrasound scan.34 chronized character GMs identify infants who are most
The severity and prognostic value of the cramped likely to develop cerebral palsy. They are the ones who
synchronized character of GMs were known from pre- need and can possibly benefit from early intervention.
vious studies.29,32 However, the finding that this motor The first videotape should be recorded as soon as
abnormality is a specific marker of later cerebral palsy is possible after birth, when the effects of analgesia and/or
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