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The Early Markers for Later Dyskinetic Cerebral Palsy Are Different From

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The Early Markers for Later Dyskinetic Cerebral Palsy Are Different From C. Einspieler1 G. Cioni2 P. B. Paolicelli2 A. F. Bos3 A. Dressler2 The Early Markers for Later Dyskinetic F. Ferrari4 M. F. Roversi4 Cerebral Palsy are Different from Those H. F. R. Prechtl1 for Spastic Cerebral Palsy Original Article Abstract Key words General Movements ´ Cerebral Palsy ´ Dyskinetic Movements ´ Qualitative abnormalities of spontaneous motor activity in new- Newborns ´ Fidgety Movements ´ Young Infants borns and young infants are early predictive markers for later spastic cerebral palsy. Aim of this research was to identify which motor patterns may be specific for later dyskinetic cerebral palsy. In a large, prospectively performed longitudinal study involving Abbreviations four European hospitals we identified twelve cases with the rel- atively rare condition of dyskinetic cerebral palsy and compared ATNR asymmetrical tonic neck response their early motor development with twelve spastic cerebral CP cerebral palsy palsy cases and twelve controls. From birth to the fifth month CT computer tomography post-term, all infants were repeatedly videoed and their sponta- FMs fidgety movements neous motor patterns, including general movements, were as- GMs general movements sessed. Until the second month post-term, the infants that later MRI magnetic resonance imaging became dyskinetic displayed a poor repertoire of general move- PMA postmenstrual age ments, ªarm movements in circlesº and finger spreading. Abnor- UIPE unilateral intraparenchymal echodensity mal arm and finger movements remained until at least five US ultrasound months and were then concurrent with a lack of arm and leg 73 movements towards the midline. Later dyskinetic infants share with later spastic infants the absence of fidgety movements, a Introduction spontaneous movement pattern that is normally present from three to five months. Qualitative assessment of spontaneous mo- The prevalence of cerebral palsy (CP) (about 2½) has changed tor patterns enabled us to identify infants at high risk for dyski- very little over the past forty years in spite of the many techno- netic cerebral palsy early in life. Additionally, we were able to logical advances in perinatology that have reduced mortality in discriminate them from those infants at high risk for later spastic compromised neonates [9]. The spastic form of CP is the more cerebral palsy. This is a matter of significant clinical relevance common form while dyskinetic CP has a frequency of only 10 to because the two types of cerebral palsy ask for different manage- 15% of all CP forms [15,29]. The dyskinetic group comprised syn- ment and early intervention. dromes of choreo-athetosis as well as the more disabling dyston- ic forms [15]. As long as there is no neonatal magnetic resonance Downloaded by: Bibliothek der Medizinischen Universität Graz. Copyrighted material. Affiliation 1 Department of Physiology, Karl Franzens University, Graz, Austria 2 Division of Child Neurology and Psychiatry, University of Pisa and Stella Maris Scientific Institute, Pisa, Italy 3 Department of Paediatrics, Division of Neonatology, Beatrix Childrens Hospital, University Hospital, Groningen, The Netherlands 4 Department of Obstetrical, Gynaecological and Paediatric Sciences, Division of Neonatology, University Hospital, Modena, Italy Correspondence Prof. Heinz F. R. Prechtl, M.D., D. Phil. ´ Department of Physiology, Karl-Franzens-University of Graz ´ Harrachgasse 21/5 ´ 8010 Graz ´ Austria ´ E-mail: [email protected] Received: August 28, 2001 ´ Accepted after Revision: February 14, 2002 Bibliography Neuropediatrics 2002; 33: 73±78 Georg Thieme Verlag Stuttgart ´ New York ´ ISSN 0174-304X imaging (MRI) easily available, it is still difficult to predict early, eight boys were born between 1986 and 1999. For comparison, which infant will be affected by CP, and it is even more difficult to 24 other infants were selected according to their postmenstrual predict its specific form. A number of signs might raise the index age (PMA) at birth, birth weight and number of movement as- of suspicion for developing CP. Infants who are hypotonic and sessments. Twelve of them had developed spastic CP (Group S). open their mouths excessively [9], who have attacks of opistho- These three girls and nine boys were born between 1988 and tonus [23], who are less active than expected, who move one 1994. The severity of motor impairment, assessed with the Gross limb less than the others, who keep their hands fisted after three Motor Function Classification System [22] was similar between months of age [32], who are late in beginning to smile [18], or the dyskinetic (mean = 3.7, SD = 1.4) and the spastic group (mean who have a persistent asymmetric tonic neck response [29] = 3.5, SD = 1.4). The other six girls and six boys, born between might be suspect. However, none of the motor development 1984 and 1990, were neurologically at risk because of pre- or scales such as the Movement Assessment of Infants, the Motor perinatal complications, but had developed neurologically nor- Scale of the Bayley Developmental Test, Paines Protocol, or the mal (Group N). For all 36 infants repeated observations of their Assessment of a Delay in Motor Milestones ever improved on a spontaneous motor activity at various ages until 26 weeks post- Original Article sensitivity of more than 70% [2,12,16,28]. By contrast, the num- term, results of brain ultrasound scans, and standardised repeat- ber of cases correctly identified in the first weeks of life by more ed neurological examinations, were available. In all three groups comprehensive neurological examinations [3,10, 31] is remark- eight infants were born at term, and four were born preterm (26 ably high, but the number of false positive results, however, is to 33 weeks PMA). The infants birth weights ranged from 820 g not low enough [7,8,21]. to 3800 g (median: 2945 g, P25: 1723 g, P75: 3480 g) in Group D; from 950 g to 4150 g (median: 2540 g, P25: 1375 g, P75: 3355 g) A new technique for assessing the spontaneous motor activity in Group S; and from 800 g to 4350 g (median: 3140 g, P25: has been recently introduced, suitable to identify and distinguish 1255 g, P75: 3723 g) in Group N. Details are given in Table 1. between those infants who require early surveillance and inter- vention for neurological abnormalities and those who do not Neuroimaging scans (cranial ultrasound [US], brain computer to- [24,26]. The quality of general movements (GMs), gross move- mography [CT] or magnetic resonance imaging [MRI]) were car- ments involving the whole body in a variable and complex se- ried out in all infants. In the neonatal period all infants had serial quence and occurring from early fetal age onwards until maxi- US scans. Neonatal MRI was performed in three infants of the mally six months after term [25], is assessed. The absence of dyskinetic group and in one of the spastic group. Most of the CP fidgety movements (FMs), small, circular and elegant move- cases had an MRI scan during their first year of life, but in a few ments of neck, trunk and limb [24], observable in the three- to cases, the first MRI was carried out in their second year of life. five-month-old infant, is a reliable and specific marker for CP. In This heterogeneity is due to the large scatter in the sampling of addition, infants who develop CP also have abnormal GMs before the cases. In addition, different MRI systems and protocols were their third month. Usually cramped-synchronised GMs precede used in the different centres. Only one infant with normal out- 74 the absence of FMs [24]. These rigid GMs, which lack the normal come had an MRI scan, performed at 6 months of age. All CP smooth and fluent character, have already been reported to be cases had signs of brain lesions. In the dyskinetic group, five in- highly predictive, particularly for the spastic forms of CP [13]. Re- fants showed basal ganglia lesions (associated with diffuse white turning to the data of the individual developmental trajectories matter lesions in two cases) and the other seven infants had peri- of the GMs reported in the multi-centre study by Prechtl et al ventricular white matter changes (associated with focal occipital [24], we found that the one infant who later developed dyski- infarct in one infant). In the spastic group, seven infants showed netic CP followed another general movement trajectory than the diffuse white matter lesions (associated with basal ganglia le- 48 infants who later developed spastic CP. During the first sions in two cases), four infants had periventricular white matter months of life, prior to the absence of FMs, the GMs of this infant damage, and one a unilateral periventricular lesion. US scans of Einspieler C et al. The Early Markers for ¼ Neuropediatrics 2002; 33: 73 ±78 lacked their normal complexity and variability although they the normal infants either were completely normal or showed had not been considered as cramped-synchronised. very mild intraventricular haemorrhage or short-lasting transi- ent periventricular density. Downloaded by: Bibliothek der Medizinischen Universität Graz. Copyrighted material. The considerable difference in the quality of life and in the type of management of patients with spastic versus dyskinetic forms All parents had given their informed consent and had agreed to of CP makes it important to search for early specific signs. This participate in the follow-up study. The procedure followed dur- investigation was possible within the context of our large, pro- ing this study was in accordance with the requirements of the spectively collected longitudinal data which included twelve ethical committees of the centres involved. dyskinetic cases. Observation of spontaneous movements During video recording, the infants were partially dressed (e.g., Subjects and Methods wearing a ªbodyº only), lying supine, at least half an hour after a feed, and during periods of active wakefulness (for details of the Subjects recording technique see [11]).
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