The term diplegia should be enhanced. Part II: contribution to validation of the new rehabilitation oriented classification.

Recent proposals of classification for cerebral palsy (CP), mainly revised for epidemiological purposes, suggest to abandon the use of the term diplegia. Conversely, in this paper data are presented to support the proposal to maintain the distinction between spastic tetraplegia and diplegia, and to subdivide this latter according to four main clinical patterns of walking observable in these children. This proposal of classification was validated by testing a group of 467 subjects with CP, of whom 213 with diplegia and 115 with tetraplegia, consecutively admitted between January 2005 and December 2006 to two national reference centers for this disability. The results were compared with findings obtained by other methods of classifying gross and fine motor function and associated disorders. The subjects with tetraplegia strongly differ from those of diplegia, both for motor functions and for other disabilities. The four main walking patterns of spastic diplegia were easily recognizable and observers were able to assign most of the subjects to one form of the classification. Significant correlations between walking forms of diplegia and distribution of Gross Motor Function Classification System (GMFCS) levels were found. Some of the forms significantly differ also for fine motor and mental disability. These findings suggest that in clinical practice the category of diplegia not only can be kept as a separate form of CP, but it may be enhanced, through the identification of different subcategories of children, divided according to their walking patterns.

The term diplegia should be enhanced. Part III: inter-observer reliability of the new rehabilitation oriented classification.

AIM: The aim of this study was to validate a recent classification of gait in children with the spastic diplegic form of cerebral palsy (CP) by checking the reliability of different scorers in assigning subject walking performance to one of the four specific patterns described in the classification. METHODS: The gait patterns of 50 children and adolescents with CP (23 males, 27 females; age range 3-17 years) were selected among patients whose videos were stored in the archives of the Pisa and Reggio Emilia Hospitals. Only video recordings of gait with homogeneous features (duration of at least 90 s, simultaneous recordings on sagittal and frontal views, and other criteria) were taken for examination. The videos were blindly scored using an observational gait scale, at first by two of the authors of the classification system (defined as ''maximum experts''), then by ten expert observers, and finally by 206 professionals of rehabilitation after a one-day training on the classification. Cohen's kappa statistics (k) and intra class correlations (ICC) were calculated. RESULTS: Kappa and ICC indicate an almost perfect agreement both between the two maximum experts and among the ten expert observers. Good results were also obtained in the group of one-day trained scorers. Only a few cases were assigned to the ''unclassified'' category. The profession of the observer (doctor or therapist) and previous knowledge of the classification had no significant influence on reliability scores. CONCLUSION: The results suggest that the proposed classification can be reliably applied, even utilizing short video recordings, to arrange diplegic children into different patterns. Further studies are needed to validate the use of this classification system for clinical and research aims.

General movements detect early signs of hemiplegia in term infants with neonatal cerebral infarction.

Background. Studies have reported that infants with hemiplegia of congenital origin may have a period between birth and up to 12 months when clinical signs of hemiplegia are not evident. The aim of this study was to establish whether the assessment of general movements (GMs) may help in the earlier detection of signs of hemiplegia. Subjects and Methods. Eleven infants with cerebral infarction on brain MRI, and eleven normal controls were enrolled in the study. Quality of GMs was assessed from videotapes between 3 and 6 weeks and between 9 and 16 weeks. Neurological outcome was evaluated at least at two years. Results. Seven of the 11 infants had an assessment performed between 3 and 6 weeks: abnormal GMs were observed in all the infants who developed hemiplegia, but one child had abnormal GMs and a normal outcome. All 11 infants had a scorable assessment between 9 and 16 weeks. In all a specific type of GMs, fidgety movements (FMs), were predictive of neurological outcome. The presence of early asymmetries at both 3 - 6 and 9 - 16 weeks was also significantly associated with later signs of hemiplegia. Conclusions. The assessment of GMs after the neonatal period appears to be very useful in the early identification of hemiplegia in infants with cerebral infarction. Whilst the prediction of hemiplegia should be possible from early neonatal MRI brain scans, this facility is not always available. Observation of GMs is a bedside clinical approach that allows confirmation of early prediction from MRI, early rehabilitation if needed and reassurance that neurological outcome will be good where that is appropriate.

The early markers for later dyskinetic cerebral palsy are different from those for spastic cerebral palsy.

Qualitative abnormalities of spontaneous motor activity in newborns 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 four European hospitals we identified twelve cases with the relatively rare condition of dyskinetic cerebral palsy and compared their early motor development with twelve spastic cerebral palsy cases and twelve controls. From birth to the fifth month post-term, all infants were repeatedly videoed and their spontaneous motor patterns, including general movements, were assessed. Until the second month post-term, the infants that later became dyskinetic displayed a poor repertoire of general movements, "arm movements in circles" and finger spreading. Abnormal arm and finger movements remained until at least five months and were then concurrent with a lack of arm and leg movements towards the midline. Later dyskinetic infants share with later spastic infants the absence of fidgety movements, a spontaneous movement pattern that is normally present from three to five months. Qualitative assessment of spontaneous motor patterns enabled us to identify infants at high risk for dyskinetic cerebral palsy early in life. Additionally, we were able to discriminate them from those infants at high risk for later spastic cerebral palsy. This is a matter of significant clinical relevance because the two types of cerebral palsy ask for different management and early intervention.

Early neurological signs in preterm infants with unilateral intraparenchymal echodensity.

UNLABELLED: The aim of the study was to document the early developmental course of neurological signs in a group of preterm infants at risk for hemiplegia due to unilateral intraparenchymal echodensity (UIPE). Sixteen preterm infants with UIPE and sixteen controls were given serial neurological examinations, according to the protocols currently adopted in the different NICUs of the project. Moreover, the quality assessment of their general movements (GMs) was assessed subsequently from videotapes, from birth until around four months postterm. At two years, 12 of the UIPE infants showed hemiplegia and one suffered from asymmetrical diplegia. The findings of the traditional neurological examination were abnormal for the large majority of the UIPE infants, although normal findings were also recorded in some cases, especially during the preterm period. Asymmetries were found after term age in nine UIPE and in two control infants. From the first observation onwards, all infants with UIPE showed bilaterally abnormal GMs and in those with unfavourable outcome fidgety movements (FMs) were absent. At the FMs period (9-16 weeks postterm), all infants with subsequent hemiplegia showed asymmetry of distal segmental movements which were reduced or absent on the side contralateral to the lesion. CONCLUSIONS: Unilateral brain lesions induce clear neurological signs and abnormal GMs in particular, although these abnormalities are not initially asymmetrical. A reduction of segmental movements on one side of the body during the third month postterm is highly predictive of hemiplegia.

MRI and clinical characteristics of children with hemiplegic cerebral palsy.

Magnetic resonance imagings of 91 children with hemiplegic cerebral palsy were analysed with the aim of clustering their features into fairly homogeneous forms. In addition, the different clinical patterns of each form were described. Four main types of lesion were distinguished: form 1 (13 cases), which comprised brain malformations, form 2 (41 subjects), which grouped abnormalities of the periventricular white matter, form 3 (27 children), which was represented by cortical-subcortical lesions, and form 4 (10 subjects), which grouped non-progressive postnatal brain injuries. None of the children had normal MRI and a high incidence of bilateral lesions was found, especially in form 2. A left motor involvement was prevalent in the sample and was noted in all but the third form. The severity of impairment was mainly moderate in forms 1 and 3, mild in the others. The upper limb was found to be more affected in all forms except the second one, which presented a greater involvement of the lower limb. Mental retardation occurred in about one-third of the children with forms 1 and 4, less often in the other two. Seizures occurred in about half of the children with forms 1 or 3, while the incidence was lower in forms 4 and 2. A strong correlation between the presence of seizures and mental retardation was observed. The results of this study show the importance of MRI in the evaluation of children with hemiplegic cerebral palsy.

Kinematic and qualitative analysis of lower-extremity movements in preterm infants with brain lesions.

BACKGROUND AND PURPOSE: The purposes of this study were to evaluate the effects of preterm birth, severe brain lesions, and postterm age on kicking movements of young infants and to compare the prognostic value of kinematic analysis of kicking with a qualitative assessment of infants' spontaneous movements. SUBJECTS: The subjects were 12 full-term infants without brain injury, 12 low-risk preterm infants without brain injury, and 11 preterm infants with severe brain lesions (periventricular leukomalacia). METHODS: Videotape recordings of each infant's motor behavior in a supine position were made at 1 and 3 months postterm age. Kicking frequency, temporal organization of the kick cycle, coordination among different joints, and interlimb coordination were measured. A qualitative assessment for lower-extremity movements and a Gestalt judgment of general movement quality according to Prechtl's method were made from the same videotape recordings. RESULTS: Kinematic analysis showed only mild differences among the 3 groups of infants. Qualitative assessment of the lower-extremity movements, however, showed that preterm infants with brain lesions, and particularly those who later were found to have cerebral palsy, consistently had fewer segmental movements of the foot and abnormal general movements at both ages. CONCLUSION AND DISCUSSION: The data suggest that the mechanisms responsible for kicking movements in newborns and young infants do not appear to be influenced by the extrauterine environment or by brain lesions, at least at the ages studied. Qualitative assessment of lower-extremity and general movements seems to be more appropriate for clinical purposes.

MRI findings and sensorimotor development in infants with bilateral spastic cerebral palsy.

The correlation between MRI findings and sensorimotor development was investigated in a group of 48 infants with bilateral spastic cerebral palsy (CP). The ages at MRI examination and cognitive assessment were fairly homogeneous (mean 15 months and 17 months, respectively). The following MRI parameters were scored: size of lateral ventricles, extension of white matter lesions and of white matter reduction, thinning of corpus callosum, presence and size of cystic areas, dimension of subarachnoid spaces and presence of cortical abnormalities. Cognitive assessment included Griffiths Developmental Scales and Uzgiris-Hunt Scales. The patients were subdivided into six classes according to intellectual level (DSM-III-R). For the whole group a highly significant correlation was found between all MRI parameters and the level of cognitive development. This result was probably due to the inclusion of 14 untestable, severely mentally retarded infants, who showed very severe MRI abnormalities. However, when the untestable infants were excluded from the analysis, it was the presence of cysts and the entity of white matter reduction that correlated with both Griffiths Scales and Uzgiris-Hunt Scales. These results indicate the clinical value of MRI findings and particularly of white matter abnormalities for early identification of sensorimotor impairment in infants with bilateral spastic CP.

Comparison between observation of spontaneous movements and neurologic examination in preterm infants.

OBJECTIVE: The Prechtl method of qualitative assessment of general movements (GMs) has been shown to be a good predictor of neurologic outcome in fetuses, preterm infants, and term infants. The aim of this study was to compare the results of this new technique with those of traditional neurologic examination and of cranial ultrasonography in preterm infants. METHODS: Serial videotape recordings (with off-line assessment of GMs), ultrasound examination of the brain, and neurologic examinations were performed from birth until about 6 months of corrected age, on a group of 66 preterm infants whose gestational age ranged from 26 to 36 weeks (mean 30.7 weeks). The agreement between the two techniques and their predictive power, with respect to the neurologic outcome at 2 years of corrected age, were evaluated for five different age groups from preterm age to 65 weeks of postmenstrual age. RESULTS: Overall agreement of the neurologic and GM findings was 80.3% and strongly age related (lower during the preterm and term periods and higher thereafter). At all ages the results of GM observation correlated highly with neurologic outcome; they showed higher sensitivity and specificity than the neurologic examination. This held true in particular before term age, when poor neurologic responses might be related to transient complications, and at term age, mainly because of infants with normal neurologic examination results but unfavorable outcome. During the preterm period the ultrasound results showed a better specificity and a lower sensitivity to outcome than GM findings. CONCLUSIONS: The results of this study indicate that quality assessment of GMs should be added to traditional neurologic assessment, neuroimaging, and other tests of preterm infants for diagnostic and prognostic purposes.

Which better predicts later outcome in full-term infants: quality of general movements or neurological examination?

The qualitative assessment of general movements (GMs) has been shown to be a better predictor of neurological outcome than the traditional neurological examination in brain-damaged preterm infants. The aim of this study was to compare the results of the two techniques in term infants. Off-line assessment of GMs from videorecordings and neurological examinations were carried out, from birth till about 6 months of postterm age, in a group of 58 term infants, the majority of which were affected by mild to severe hypoxic-ischaemic encephalopathy. The agreement between the two techniques and their predictive power, with respect to the neurological outcome at 2 years, were evaluated for four age groups. The range of agreement between neurological and GM findings was between 78 and 83%. At all ages the results of GM observation correlated highly with the neurological outcome; their sensitivity and specificity with respect to outcome were consistently slightly superior to those of neurological examination. In infants normalize after an initial period of transient abnormalities, GMs normalize earlier than the neurological results.

Posture, spontaneous movements, and behavioural state organisation in infants affected by brain malformations.

Posture, quantity of spontaneous movement patterns, quality of general movements (GMs), and behavioural state organisation were studied in nine infants affected by documented brain malformations. A single 1 h video recording of five infants and two or more serial video recordings of another four infants were performed after birth. The graphic representation of single movement patterns (actogram) and of behavioural states of one video recording was performed in eight out of nine infants. The quality of GMs was assessed according to Prechtl's method in all video recordings. All nine infants showed a less variable posture than normal newborn infants and an unusual resting posture was detected in seven infants. Poor behavioural state organisation without sleep cycles was common to the nine infants and excessive wakefulness was observed in six infants. As for the quantity of single movement patterns, six infants lacked one or two movement patterns normally present in healthy newborn infants. An abnormal quality of GMs was noted in all nine infants and distinct motor abnormalities were observed in single infants. A monotonous and sometimes stereotyped sequence of different body parts involved in the movement (i.e. poor repertoire GMs) was common to all infants. In the four infants of whom two or more video recordings were available, initial poor repertoire GMs were followed by a further deterioration in movement quality. No relationship was found between the quantity of defective brain tissue, lack of a specific part of the brain, type and severity of GM and posture abnormalities.

Differences and variations in the patterns of early independent walking.

The early development of walking was investigated in 25 normal fullterm and in 25 low-risk preterm infants. All subjects were videorecorded within 3-4 weeks of the beginning of independent walking and again 4 months later. Analysis of the videos was carried out according to a predetermined list of items, with a semiquantitative score for each. The results indicate wide interindividual differences in normal infants in the form of independent walking. Several items seemed to be associated in different ways in different individuals. Age of onset, as long as the corrected age was considered, did not differ between preterm and fullterm infants, neither did their walking patterns. Gait asymmetries were often observed in both groups and they were related with asymmetries observable in prewalking locomotor behaviour. Toe-strike often occurred at the beginning of walking, but not after 4 months; in preterm infants the toe strike pattern correlated significantly with certain motor characteristics observed during the first weeks of life.

Sensorimotor development in cerebral-palsied infants assessed with the Uzgiris-Hunt scales.

The cognitive development of a group of 89 cerebral-palsied infants, aged six to 24 months, was investigated using the Uzgiris-Hunt scales. The results were compared with normative data for the Italian population and with data obtained in a group of low-risk term and preterm infants, 11 to 13 months old. The test was easy to carry out, even on infants with a severe motor impairment. The majority of the infants showed cognitive delay on most of the scales. Tetraplegic patients performed significantly worse than those with diplegia or hemiplegia. There were no differences between preterm and term infants, for either normal or cerebral palsy groups, if age was corrected for preterm birth. Sensorimotor development appeared to be organized similarly for cerebral-palsied infants and normal controls; however, these data raise the question of the role of action in early cognitive development.

Hyperekplexia and stiff-baby syndrome: an identical neurological disorder?

Hyperekplexia (startle disease) is an unusual, familial, neurological disorder characterized by abnormally enhanced startle response, followed in most cases by momentary generalized muscular stiffness. These attacks may cause the patients to fall rigidly, while remaining fully conscious. Startle symptomatology has generally an onset in infancy and is often accompanied, during the first years of life, by rigidity, sleep myoclonus, motor delay, regurgitation and apneic spells, which may cause sudden death. Stiff-baby syndrome is a familial disorder characterized by marked rigidity, with neonatal onset and gradual reduction during infancy, regurgitations, motor delay and attacks of stiffness. We report 4 new cases of hyperekplexia from two different families and another infant with stiff-baby syndrome discussing clinical, electrophysiological and genetic aspects of both neurological disorders in relation to other reported cases. We suggest a continuum between these familial syndromes, which are often misinterpreted as epilepsy or other disorders.