Kids+ Parent Infant Program (PIP): a community model for supporting partnerships in early developmental follow-up and support.

High-risk infants are discharged home from hospital with increased care needs and the potential for the emergence of developmental disabilities, contributing to high levels of parental stress and anxiety. To enable optimal outcomes for high-risk infants and their families, developmental follow-up programs need to continue following hospital discharge. However, current follow-up care for high-risk infants is variable in terms of type, access and equity, and there seems to be a gap in existing services such as supporting the transition home, parental support, and inclusion of all at-risk infants regardless of causality. Routine follow-up that identifies developmental delays or neuromotor concerns can facilitate timely referral and access to targeted intervention during critical periods of development. The Kids+ Parent Infant Program (PIP) is a unique model of developmental follow-up that shares some characteristics with established programs, but also includes additional key elements for a seamless, wrap-around service for all high-risk infants and their families living in a regional area of Australia. This community-based program provides integrated assessment and intervention of infants, alongside parent support and education, embracing a holistic model that accounts for the complexity and interrelatedness of infant, parent, medical and developmental factors. By prioritising the well-being of high-risk infants and their families, the Kids+ PIP paves the way for improved developmental outcomes and provides an innovative model for developmental follow-up, with the potential for reproduction in other healthcare settings.

The School Function Assessment: identifying levels of participation and demonstrating progress for pupils with acquired brain injuries in a residential rehabilitation setting.

BACKGROUND: Delivering and monitoring residential rehabilitation services for pupils with acquired brain injuries (ABI) is challenging because of variability in aetiology, age and pre-morbid characteristics. Therapists and educators in this residential rehabilitation setting identified the need for a comprehensive tool which would capture the pupils' ability to participate in typical, everyday, school-based functional activities. This study aimed to explore the ability of the School Function Assessment (SFA) to identify levels of participation in school-based functional tasks and demonstrate progress for pupils with an ABI in a residential rehabilitation setting. METHODS: The SFA was conducted on admission and discharge for pupils with ABI receiving residential rehabilitation between January 2007 and October 2011. Data analysis used Kruskal-Wallis to determine between-group differences in age, time post injury and weeks in rehabilitation. Linear mixed effects modelling was used to establish differences between admission and discharge scores. Case vignettes demonstrated how the SFA was used with individual pupils. RESULTS: Seventy pupils were identified (31 traumatic brain injury; 29 non-traumatic and 10 anoxic) 42 boys and 28 girls whose age at injury was 4.5-17.2 years (median 12.8). The SFA demonstrated that 54/70 pupils had made progress with their ability to participate in school activities. Significant differences were found between admission and discharge SFA scores for participation, physical and cognitive assistance and adaptation and activity performance (P < 0.05). There was wide variability between individual profiles reflecting the heterogeneity of ABI. CONCLUSIONS: The SFA determined levels of participation in school-based functional tasks for these residential pupils with an ABI and demonstrated progress both at the group and individual level in this residential rehabilitation setting. It identified the amount of support a pupil would need when they return to their local school and their strengths and needs in relation to participation in physical and cognitive school-based functional activities.

Abnormal cortex-muscle interactions in subjects with X-linked Kallmann's syndrome and mirror movements.

X-linked Kallmann's (XKS) subjects, who display mirror movements, have abnormal corticospinal tracts which innervate motoneurons of the left and right distal muscles of the upper limb. The size of the abnormal ipsilateral projection is variable. We have used coherence and cumulant analysis between EEG and first dorsal interosseous muscle (1DI) EMG to explore mechanisms underlying mirror movements in three XKS subjects. Results are compared with those of three normal subjects. We argue that significant coherence is functionally relevant when associated with a negative cumulant at an appropriate lag. Given this, normal subjects showed coherence at approximately 22 Hz between the EEG recorded over the sensori-motor cortex contralateral to the voluntarily moved hand and the 1DI EMG of this hand. No significant coherence was seen between 1DI EMG and the sensori-motor cortex ipsilateral to the muscle activity. In contrast, two of the XKS subjects (K2 and K4) had significant coherence at 22 Hz, together with a negative cumulant at an appropriate lag, between the ipsilateral cortical EEG and the 1DI EMG of the voluntarily activated hand. This implies that activity in the abnormal ipsilateral corticospinal projection can contribute to the voluntary drive. For these two subjects, the ipsilateral corticospinal projection was greater than the contralateral projection, as revealed using magnetic brain stimulation. In one of these subjects, K4, significant 22 Hz coherence and negative cumulant was also seen between the EMG of the voluntarily activated hand and the cortex contralateral to this hand. In the third subject, K4a, coherence and negative cumulant was detected between the EMG of the voluntary side and the cortical activity contralateral to this hand. The contralateral cortico spinal projection of this subject was greater than the ipsilateral projection. Regarding the mirroring hand of the XKS subjects, coherence (with negative cumulant at an appropriate lag) was seen in all three subjects between the EMG recorded from the mirroring hand and cortical EEG ipsilateral to this hand. This provides evidence that activity in the aberrant ipsilateral projection is involved in producing the drive that results in mirror movements. In one subject, K4, coherence and negative cumulant was also seen between the EMG of the mirroring hand and motor cortical activity contralateral to this hand. Thus, in this subject, activity in the corticospinal projection contralateral to the mirroring hand also contributed to the mirror movements. In conclusion, this study has provided further evidence that the 22 Hz coherence seen between EEG and EMG is dependent upon corticospinal activity and has furthered our understanding of mechanisms underlying mirror movements.

People with cerebral palsy: effects of and perspectives for therapy.

The movement disorder of cerebral palsy (CP) is expressed in a variety of ways and to varying degrees in each individual. The condition has become more complex over the last 20 years with the increasing survival of children born at less than 28 to 30 weeks gestational age. Impairments present in children with CP as a direct result of the brain injury or occurring indirectly to compensate for underlying problems include abnormal muscle tone; weakness and lack of fitness; limited variety of muscle synergies; contracture and altered biomechanics, the net result being limited functional ability. Other contributors to the motor disorder include sensory, cognitive and perceptual impairments. In recent years understanding of the motor problem has increased, but less is known about effects of therapy. Evidence suggests that therapy can improve functional possibilities for children with cerebral palsy but is inconclusive as to which approach might be most beneficial. The therapist requires an understanding of the interaction of all systems, cognitive/perceptual, motor, musculoskeletal, sensory and behavioral, in the context of the development and plasticity of the CNS. It is necessary to understand the limitations of the damaged immature nervous system, but important to optimize the child's functional possibilities.

Physiological tremor in human subjects with X-linked Kallmann's syndrome and mirror movements.

Human physiological tremor consists of mechanical-reflex and neurogenic components. The origin of the neurogenic component, classically detected in the frequency range 7-12 Hz, has been much debated. We have studied six subjects with X-linked Kallmann's syndrome (XKS) and mirror movements. In these subjects unilateral magnetic brain stimulation results in abnormal bilateral EMG responses. Furthermore, abnormal sharing of central nervous inputs between the left and right motoneurone pools results in both abnormal motor unit synchronisation between left and right EMGs and abnormal sharing of long but not short-latency cutaneomuscular reflexes. XKS subjects with mirror movements thus provide a model for studying the central origin of physiological tremor. During sustained co-extension of the left and right index fingers, simultaneous finger tremor and extensor indicis (EI) EMGs were recorded and cross-correlated. The tremor and EMG signals were also subjected to time and frequency domain analysis.Results of frequency domain analysis between ipsilateral finger tremor and EI EMG were similar for both control and XKS subjects. However, in contrast to the controls, short-term synchronisation of left and right EI motor unit activity and significant coherence between left and right EMG, left and right tremor, left EMG and right tremor and right EMG and left tremor were found in XKS subjects. The frequency range (6-40 Hz) and coherence values between left and right were similar to ipsilateral coherence values of rectified EMG and tremor. These data provide strong evidence to support the hypothesis that the neurogenic component of physiological tremor is supraspinal in origin and ranges from 6 to 40 Hz.

Reactive control of precision grip does not depend on fast transcortical reflex pathways in X-linked Kallmann subjects.

It has been shown that subjects maintain grasp stability by automatically regulating grip force in response to loads applied tangentially to a manipulandum held using a precision grip. Signals from cutaneous mechanoreceptors convey the information necessary for both the initiation and scaling of responses. The central neural pathways that support these grip reactions are unknown. However, the latency of the increase in force is similar to that of 'long-latency' transcortical reflexes recorded from muscles following muscle stretch or electrical stimulation of digital nerves. This study assessed the importance of fast transcortical pathways for reactive grip responses by examining these responses in subjects with X-linked Kallmann's syndrome (XKS). Subjects were selected whose corticospinal projection, as assessed by magnetic brain stimulation, is essentially ipsilateral, and in whom the long-latency reflex components following digital nerve stimulation are only found contralateral to the stimulated side. Despite this anomaly of the fast corticospinal pathway, these XKS subjects responded in the same way as control subjects; grip response latencies were similar and responses were appropriately scaled. However, the non-operating hand of these XKS subjects often mirrored the grip force changes of the operating hand. Reflex force mirroring was most marked during the first 50 ms and the force output was always less than 20 % of that of the operating hand. We conclude, firstly, that somatosensory driven precision grip responses that support grasp stability do not depend on fast conducting corticospinal pathways in these subjects and, secondly, that such responses do not use those 'long-latency' reflex pathways probed by cutaneomuscular reflexes elicited by electrical stimulation of digital nerves.

A neurophysiological study of mirror movements in adults and children.

The mechanism underlying mirrored activity/movements in normal individuals is unknown. To investigate this, we studied 11 adults and 39 children who performed sequential finger-thumb opposition or repetitive index finger abduction. Surface electromyographic (EMG) activity recorded from the left and right first dorsal interosseous muscles (1DI) during unilateral sequential finger-thumb opposition (voluntarily activated muscle, 1DIvol) showed mirrored EMG activity (homologous muscle of the opposite hand, 1DImm) that decreased with increasing age. The time of onset of involuntary compared with voluntary EMG activity was variable but could start at the same time. A significant increase in E2 (transcortical component) size of the cutaneomuscular reflex recorded from the 1DImm indicated increased excitability of the motor cortex ipsilateral to the 1DIvol during active index finger abduction compared with the 1DIvol relaxed. Transcranial magnetic stimulation, using the Bistim technique, indicated that the transcallosal inhibitory pathway in children may not operate in the same way as in the adult. Cross-correlation analysis did not detect shared synaptic input to motoneuron pools innervating homologous left and right hand muscles. We conclude that the mirrored movements/activity observed in healthy adults and children are produced by simultaneous activation of crossed corticospinal pathways originating from both left and right motor cortices.

Abnormal motor unit synchronization of antagonist muscles underlies pathological co-contraction in upper limb dystonia.

The aim of this study was to examine the pathophysiological mechanisms underlying co-contraction in patients with dystonia (n = 6) and writer's cramp (n = 5). Multi-unit needle and surface EMGs were recorded from extensor carpi radialis (ECR) and flexor carpi radialis (FCR) muscles during motor tasks that elicited dystonia or writer's cramp. The EMGs from ECR and FCR were recorded simultaneously and analysed using cross-correlation analysis. Similar recordings were obtained from healthy age- and sex-matched control subjects (n = 8). Despite co-contraction of the muscles, cross-correlograms from the healthy subjects did not reveal evidence of motor unit synchronization. Cross-correlograms from the dystonic subjects revealed a central peak with a median duration of 37 ms, indicating broad-peak motor unit synchronization. Cross-correlograms from patients with writer's cramp were either flat or modulated by a 11-12-Hz tremor. Frequency-domain analysis of ECR and FCR EMGs demonstrated significant coherence in the patients with dystonia and writer's cramp. These results indicate that co-contraction in dystonia is neurophysiologically distinct from voluntary co-contraction and is produced by abnormal synchronization of presynaptic inputs to antagonist motor neuron pools. ECR and FCR co-contraction in writer's cramp may be a compensatory process under voluntary control.

Mirror movements in X-linked Kallmann's syndrome. I. A neurophysiological study.

Possible mechanisms underlying the pathological mirror movements that are seen in the majority of patients with X-linked Kallmann's syndrome have been investigated using neurophysiological techniques. An EMG was recorded from the first dorsal interosseous muscle (1DI) during voluntary self-paced abduction of one indexed finger; EMG activity could also be recorded simultaneously from the contralateral 1DI. There was no significant difference between the time of onset of the bursts of voluntary and involuntary mirroring EMG. Focal magnetic stimulation of the hand area of the motor cortex revealed the presence of fast conducting bilateral corticospinal projections from each motor cortex in all subjects. However, both inter- and intra-subject differences exist when considering the ratio of ipsilaterally to contralaterally projecting axons. Cross-correlation analysis of multi-unit EMGs recorded during simultaneous voluntary sustained activation of homologous left and right pairs of distal upper limb muscles was performed. A short duration central peak was seen in the cross-correlograms indicating the presence of a common drive to left and right homologous motor neuron pools. This common drive may result from the synchronous activation of intermingled ipsilaterally and contralaterally projecting corticospinal neurons in the motor cortex. Cutaneomuscular reflexes were recorded from the 1DI following stimulation of the digital nerves of the index finger. Typically each reflex comprises spinal and longer latency trans-cortical components. In these subjects, the long latency components of the reflex response could, in addition, be recorded from the 1DI of the non-stimulated side. We conclude that these subject have a novel ipsilateral at least in part, for the pathological mirroring.

Mirror movements in X-linked Kallmann's syndrome. II. A PET study.

To investigate the mechanism of mirror movements seen in X-linked Kallmann's syndrome, we measured changes of regional cerebral blood flow with H2 15O-PET. We studied six right-handed Kallmann male subjects and six matched, right-handed control subjects during an externally paced finger opposition task. The analyses were done both on a single subject and a group basis. The Kallmann group showed a strong primary motor cortex (M1) activation contralateral to the voluntarily moved hand, but there was also a significant degree of M1 activation ipsilateral to the voluntarily moved hand, i.e. contralateral to the mirroring hand. However, when comparing contralateral to ipsilateral M1 activation, the M1 activation contralateral to the voluntarily moved hand was significantly stronger. In the controls, significant increases in rCBF were seen in the contralateral M1 during voluntary movement of either hand; a small ipsilateral M1 activation was found in two out of six normal subjects when they moved their left hand. In a second experiment it was shown that, in two out of two Kallmann subjects, passive movements of the right hand resulted in left M1 activation that was similar to the activation in the left M1 when subjects made mirror movements with their right hand. This suggests, but does not prove, that the small but significant activation of the ipsilateral M1 in Kallmann's subjects may be due to sensory feedback from the involuntarily mirroring hand.

Electrical and mechanical output of the knee muscles during isometric and isokinetic activity in stroke and healthy adults.

Surface electromyography (EMG) and torque were measured from knee flexors and extensors in 12 control subjects (CS) aged 25-59 years (10 female) and bilaterally in 12 stroke subjects (SS) aged 27-75 years (four female) with hemiparesis and mild clinical spasticity. They performed isometric and isokinetic maximal voluntary contractions (MVC) and also isokinetic passive movements at angular velocities from 30 to 300 degrees/s. The time taken to walk 10 m was documented. Greater torque was recorded during passive extension in the paretic legs when compared with both non-paretic and control limbs (p < 0.01). No EMG activity was measured in any subject. Isometric MVC torque of both muscles in the paretic leg was less (p < 0.01) than both the non-paretic and control limbs. The SS generated relatively less torque bilaterally at the lower velocities than CS. Not all SS reached the higher velocities and none of the paretic limbs achieved 300 degrees/s during flexion. Gait speed correlated with maximal paretic knee extension velocity (p < 0.001). The extent of co-contraction during MVCs was generally low or absent and similar in all three groups. These results suggest a mechanical rather than reflex cause for the restraint detected clinically. Low force generation by the paretic agonists appeared to be the major cause of reduced torque, rather than antagonist opposition.