Spasticity, dyskinesia and ataxia in cerebral palsy: Are we sure we can differentiate them?

OBJECTIVE: Cerebral palsy (CP) can be classified as spastic, dyskinetic, ataxic or combined. Correct classification is essential for symptom-targeted treatment. This study aimed to investigate agreement among professionals on the phenotype of children with CP based on standardized videos. METHODS: In a prospective, observational pilot study, videos of fifteen CP patients (8 boys, mean age 11 ± 5 y) were rated by three pediatric neurologists, three rehabilitation physicians and three movement disorder specialists. They scored the presence and severity of spasticity, ataxia or dyskinesias/dystonia. Inter- and intraobserver agreement were calculated using Cohen's and Fleiss' kappa. RESULTS: We found a fair inter-observer (κ = 0.36) and moderate intra-observer agreement (κ = 0.51) for the predominant motor symptom. This only slightly differed within the three groups of specialists (κ = 0.33-0.55). CONCLUSION: A large variability in the phenotyping of CP children was detected, not only between but also within clinicians, calling for a discussing on the operational definitions of spasticity, dystonia and ataxia. In addition, the low agreement found in our study questions the reliability of use of videos to measure intervention outcomes, such as deep brain stimulation in dystonic CP. Future studies should include functional domains to assess the true impact of management options in this highly challenging patient population.

Development of a set of activities to evaluate the arm and hand function in children with obstetric brachial plexus lesion.

OBJECTIVE: To develop an observational instrument that can be used to evaluate the quality of arm and hand skills in daily functional activities in children with obstetric brachial plexus lesion (OBPL). A set of functional activities was constructed and standardized, and the intra-observer reliability of the assessment of this set of activities was studied. SETTING: Department of Occupational Therapy and Department of Rehabilitation Medicine, VU University Medical Centre. SUBJECTS: Twenty-six children with OBPL in the age range of 4 -6 years. INTERVENTIONS: The children were asked to perform 47 bimanual activities, which were recorded on videotape. MAIN MEASURES: The videotapes were scored twice by the same occupational therapist. RESULTS: The percentage of agreement in scoring 'hand-use', 'speed' and 'assistance' was over 80% for a substantial number of activities, indicating a strong agreement. However, in scoring 'deviations in movements and body posture' the percentage of agreement was insufficient in most activities. CONCLUSIONS: This set of activities has good potential for assessment of the performance of functional activities in children with OBPL. This study, however, showed a number of difficulties in observing and scoring the activities that have to be considered when developing a standardized video observation.

Towards an economic model of the Dutch health care sector.

This paper describes the construction of a model of the Dutch health care sector. It discusses the behaviour of patients, general practitioners, medical specialists and hospital managers. It also analyses the various ways the actors interact, such as general practitioners supplying the services demanded by patients, specialists dispatching referrals made by general practitioners or hospital managers boosting output to match an increasing amount of specialist services. Numerical simulations illustrate the various mechanisms in the model.

The influence of head position and head position change on spontaneous body posture and motility in full-term AGA and SGA newborn infants.

No consensus exists concerning the influence of head position and head position change on body posture and motility. Especially the existence of an asymmetric tonic neck reflex (ATNR) in full-term newborns is an issue of discussion. Three-hour video recordings were made of 15 full-term appropriate for gestational age (AGA) and 15 full-term small for gestational age (SGA) infants between the third and eight postnatal day. During a playback of the video recording head position, head position change and several movement patterns of the four limbs were fed into a computer using an event-detecting program. Furthermore, spontaneous head turnings were selected and body posture just before, immediately at and 1 min after the head turning were sketched. The data were analyzed concerning: (1) influence of head position on symmetry of movement of the four limbs; (2) the existence of an ATNR and ATNR-related patterns. In both AGA and SGA infants all movement patterns except hand-face and hand-mouth contact showed a symmetrical distribution, independent of head position. Furthermore, the occurrence of an ATNR following a spontaneous head turning in both AGA and SGA infants was rare. From our results it may be concluded that the ATNR is rare in full-term AGA and SGA newborns. Furthermore, this study demonstrates that head position is not a major factor influencing quantitative aspects of spontaneous motor behaviour. The results are of clinical importance as they imply that in the examination of the neurological condition of the full-term newborn infant by means of observation of spontaneous posture and motility, head position is not of major importance.

Postural behaviour of term SGA and AGA newborn infants.

The authors studied the body posture at rest of 15 healthy term infants with birthweights appropriate for gestational age (AGA) and 15 infants who were small for gestational age (SGA). In both groups, there was wide inter-individual variability in postural behaviour. Apart from a preference for a position with all limbs in abduction between 1 and 90 degrees, independent of the degree of flexion or extension and internal/external rotation, no dominant preference posture could be seen for either group. The SGA infants showed a more flexed position of the arms, compared with the AGA group. One of the factors contributing to this difference might be the influence of intra-uterine malnutrition on the development of the central nervous system.

Corpus callosum agenesis, spastic quadriparesis and irregular lining of the lateral ventricles on CT-scan. A distinct X-linked mental retardation syndrome?

This report gives a description of 4 male patients, two of whom are sibs, two of whom are uncle and cousin. They appear to have psychomotor retardation, spastic quadriparesis and on CT (partial) agencies of the corpus callosum, and irregular lining of the lateral ventricles, without craniofacial abnormalities or seizures. Although the mode of inheritance of agenesis of the corpus callosum is still difficult to establish, in these 4 male patients an X-linked recessive inheritance is the most likely mode. A review of the literature with concern to the heredity of agenesis of the corpus callosum is presented. The clinical and neurological findings in the present four male patients allow for the delineation of a new X-linked mental retardation syndrome.