F. Heinen

Development of the corticospinal system and hand motor function: central conduction times and motor performance tests.

Maturation of the corticospinal (CS) tract and hand motor function provide paradigms for central nervous system development. In this study, involving 112 participants (aged from 0.2 to 30 years), we evaluated central motor conduction times (CMCT) obtained with transcranial magnetic stimulation (TMS) during preinnervation conditions of facilitation and relaxation. Auditory reaction time, velocity of a ballistic movement of the arm, finger tapping, diadochokinesis, and fine motor visuomanual tracking were also examined. The maturation profiles for every parameter were calculated. CMCTs for the different preinnervation conditions reached adult values at different times and this could be explained by maturation of excitability at the cortical and spinal level. A stable phase for CMCTs and reaction time was reached during childhood. Parameters which measured motor speed and skill indicated that the development of these continued into adulthood. The maturation of the fast CS tract seems to be completed before the acquisition of the related motor performance has been accomplished. In conclusion, we could demonstrate that data from several neurophysiological methods can be combined and used to study the maturation of the function of the nervous system. This approach could allow appraisal of pathological conditions that show parallels with omissions or lack of developmental progress.

Medium-term functional benefits in children with cerebral palsy treated with botulinum toxin type A: 1-year follow-up using gross motor function measure

One of the main goals when treating spasticity is to relieve pain and improve function. Intramuscular injection of botulinum toxin type A (BTX‐A) has gained widespread acceptance in the treatment of spastic cerebral palsy. Several studies have clearly shown the short‐term functional benefit of BTX‐A treatment. Information is limited, however, on the efficacy of medium and long‐term regimens, using repeated injection of BTX‐A. The aim of the present open‐label, prospective study was to evaluate functional outcome in children with spastic cerebral palsy after 1u2003year of treatment with BTX‐A, using the Gross Motor Function Measure (GMFM) as a validated outcome measure. Patients (n=25, age 1.5–15.5u2003years) were treated with BTX‐A for adductor spasm (n=12) or pes equinus (n=13). The local effect was evaluated using passive range of motion and modified Ashworth Scale. Apart from a significant improvement in joint mobility and reduction of spasticity compared to pretreatment values (Pu2003<u20030.01), we demonstrated a significant improvement of gross motor function after 12u2003months of treatment, with a median gain of 6% in total and goal scores (Pu2003<u20030.001). An increase in GMFM scores was particularly evident in younger and moderately impaired children (Gross Motor Function Classification System level III). Whether the observed improvement in gross motor function in children with cerebral palsy is specifically related to therapy with BTX‐A or represents at least in part the natural course of motor development still needs clarification.

Neurologic varicella complications before routine immunization in Germany.

Varicella is an acute febrile, highly infectious disease. We describe the incidence and types of neurologic complications in children up to 16 years old. Hospitalized varicella cases were prospectively captured by active nationwide surveillance through the German Pediatric Surveillance Unit for Rare Diseases from January 2003 to December 2004. Neurologic complications occurred in 232 (25.4%) of 918 hospitalized children with varicella, and were the most frequent reason for hospitalization. The median age was 4.2 years (interquartile range 2.5-5.9). The median duration of hospital stay was 6 days (interquartile range 3-11). Neurologic complications were more frequent (P=0.054) in immunocompetent (32%) than immunocompromised (4%) children. The most frequent diagnoses comprised acute cerebellar ataxia in 72 (31.0%), febrile convulsion in 69 (29.7%), meningoencephalitis in 52 (22.4%), cerebral convulsions in 21 (9.1%), syncope in 9 (3.9%), and cerebral vasculitis/infarction in 6 (2.6%) of all children with neurologic complications. Twenty-eight (12%) demonstrated sequelae (18 with ataxia, four with epilepsy, two with hemiparesis, three with cerebral nerve palsy, and one with dysesthesia). Three patients died. The yearly incidence of neurologic varicella-associated hospitalizations was estimated at 2.4 neurologic complications per 100,000 children, corresponding to about one neurologic complication in 2000 varicella cases.

Fast corticospinal system and motor performance in children: conduction proceeds skill

Transcranial magnetic stimulation and motor performance tests were used to study the correlation between corticospinal maturation and actual motor performance in a group of young school children (n = 10, mean age = 7 years, age range = 6-9 years). The results were compared with normal adults (n = 10, mean age = 24 years, age range = 22-26 years). In children the central conduction time under the preinnervation condition of facilitation and the postexcitatory silent period was similar to that in adults. However, the central conduction time under relaxation, the latency jump (defined as the difference between the two preinnervation conditions), and the stimulus intensity were statistically different between children and adults (P < 0.01-0.001). Children did not reach the same level of performance as adults in any of the motor performance tasks (simple acoustic reaction time, tapping, ballistic movement, tracking, and diadochokinesis) (P < 0.05-0.01). The results indicate that at an early school age, children already possess mature fast corticospinal pathways able to access spinal motoneurons through the pyramidal tract. However, despite the partially adult-like level of neuronal maturation, young school children were not able to perform deliberate motor actions with the same proficiency as adults.

Botulinum toxin treatment in cerebral palsy: evidence for a new treatment option.

Abstract Intramuscular injections of botulinum toxin type A (BTX-A) have increasingly been used to reduce spasticity in specific muscle groups in children with cerebral palsy. Targets of therapeutic efforts are improvement of gross motor function, alleviation of pain or facilitation of hygienic care. Placebo-controlled studies have shown the local and functional effectiveness of BTX-A for the treatment of dynamic pes equinus. Whether long-term treatment with BTX-A improves motor development and delays contractures is still under investigation.

Adductor spasticity in children with cerebral palsy and treatment with botulinum toxin type A: the parents' view of functional outcome

Botulinum toxin type A (BTX‐A) has been used successfully to manage spasticity in children with cerebral palsy. Little has been done to evaluate treatment outcome and satisfaction from the patients and parents points of view. The aim of this study was to investigate the parents perceptions of the benefits of BTX‐A on movement disorders in children with cerebral palsy. Twenty‐six children with adductor spasticity were enrolled into an open‐label, prospective study. Patients received intramuscular injections of BTX‐A, and assessments of joint mobility (passive range of motion), degree of spasticity (Modified Ashworth Scale) and functional benefit (Gross Motor Function Measure) were made before and 12–18 weeks after treatment. Parents assessment of treatment outcomes were evaluated using a standardised questionnaire. BTX‐A was shown to be effective in reducing muscular hyperactivity and functional limitations. Parents satisfaction with the treatment outcome was high. For non‐ambulatory patients, the reported benefits included facilitation of daily care, ease of positioning and reduction of pain. For patients who were disabled to a lesser extent, improvements in gait and posture included sitting with improved comfort, standing for longer periods of time and/or walking longer distances. The parents responses supported the impressions of the therapists, demonstrating that BTX‐A produced beneficial effects on daily activities, according to both objective measures and parents observation.

Treatment of neuropathic bladder using botulinum toxin A in a 1-year-old child with myelomeningocele

Sirs, Intramuscular injection of botulinum toxin A (BTX/A) is an effective and safe therapy for children and adults with dystonic and spastic movement disorders [1, 2]. Furthermore, clinical trials have been successfully carried out treating neuropathic bladder under various conditions [3, 4, 5, 6]. We report the successful treatment with BTX/A of a neuropathic bladder in a 1-year-old boy with myelomeningocele (MMC), which has not, to our knowledge, been described in the literature before. The 1-year-old male patient showed MMC extending from Th9 to L1. Consolidation of the large wound on his back was complicated by recurrent infections. The child had an Arnold-Chiari malformation with severe hydrocephalus and showed no active lower limb movements. However, there was severe muscular hypertonus of the rectus femoris muscle and the adductor muscles. Rectal palpation suggested a highly active sphincter ani externus muscle, causing severe constipation. Starting at the age of 9 months, the child suffered from recurrent urinary tract infections with multi-resistant Pseudomonas, at a frequency of up to twice a month, although he had been circumcised. Over a period of 12 weeks, repeated sonographic evaluation of the urinary tract revealed post-void residual urine volumes of 75, 100, 150, and 80 ml. Each measurement was performed three times post voiding and the lowest value was taken. Neither reflux nor hydronephrosis was observed. Intermittent catheterization was carried out, but was not accepted as a first-line treatment by the parents. Extensive urodynamic studies were not undertaken because a prolonged supine position was strictly prohibited due to the wound on his back. In order to evaluate the external urethral sphincter, we performed a needle electromyogram (EMG), which revealed an overactive muscle. Treatment with BTX/A was carried out after obtaining approval from the local ethics committee and informed consent from the parents. The external urethral sphincter was treated with 40 units BTX/A (Botox, Merz, Frankfurt, Germany, 100 units diluted in 2 ml), by transperineal injection using EMG guidance. Two injection points were used. The child was sedated with midazolam. Thereafter, we performed a sonographic evaluation of post-void residual urine volume at weekly intervals. The volume before treatment with BTX/A was about 80 ml; during the 3 weeks after treatment, it decreased to below 5 ml (Fig. 1).

Besteht ein Zusammenhang zwischen der verkürzten Gymnasialzeit und Kopfschmerzen und gesundheitlichen Belastungen bei Schülern im Jugendalter

BACKGROUNDnThe reduction of school years in grammar schools from 9 to 8 years (G9 vs. G8) is supposed to exhibit increased impairments of health of the latter group of students. Aim of the present study was to investigate whether G8-students are exposed to more stress and report more headaches and other health complaints than G9-students.nnnPARTICIPANTSn1 260 formers of grammar schools in Munich (10 (th) vs. 11 (th) form).nnnMETHODSnIn a survey, the frequency of headache and other health complaints, experience of chronic stress and health-related quality of life were assessed with a questionnaire and compared between the two groups of different grammar-school durations (G8 vs. G9).nnnRESULTSn83.1% of all formers reported to suffer from headache at least once per month. Further frequently reported health complaints were back pain (47.7%), excessive need for sleep (45.6%) and pain in neck or shoulder (45.0%). 20.4% of the formers reported high exposure to stress. The greatest reductions in quality of life were found with respect to school-related and physical wellbeing. As the only significant differences, formers of G8 reported fewer daily leisure time and that available leisure time was not sufficient for recreation.nnnCONCLUSIONSnThe high prevalence of pain, health complaints and stress indicates high demands to all grammar scholars. High demands due to the reduction of school years in grammar school, however, are not reflected in increased health impairments in these formers, but rather in limited leisure time activities.

Inhibitory conditioning stimulus in transcranial magnetic stimulation reduces the number of excited spinal motor neurons

OBJECTIVEnTo study the mechanisms of amplitude attenuation caused by a transcranial magnetic conditioning stimulus. Both conventional MEPs and the recently described triple stimulation technique (TST) were applied; the latter to improve the quantification of the response size decrease.nnnMETHODSnTST uses a peripheral collision method to eliminate the effects of desynchronization of the transcranial magnetic stimulation (TMS) induced spinal motor neuron discharges. The attenuation of motor evoked potentials (MEPs) and responses to TST was studied in 10 healthy volunteers using the conditioning-test paradigm with 2 ms interstimulus intervals.nnnRESULTSnConventional MEPs and responses to TST demonstrated a marked attenuation by the preceding conditioning stimulus in all subjects. The ratio of MEP to TST amplitudes was the same in conditioned and unconditioned responses.nnnCONCLUSIONSnOur findings suggest that the transcranial conditioning stimulus does not change the degrees of desynchronization of spinal motor neuron discharges, but results in a reduced number of excited alpha motor neurons. This reduction can be estimated by both MEPs and TST.

Concurrent TNFRSF1A R92Q and pyrin E230K mutations in a child with multiple sclerosis

We report a 16-year-old female patient with a severe course of multiple sclerosis and concomitant symptoms suggestive of a hereditary autoinflammatory disease. Genetic analyses revealed that she inherited a TNFRSF1A R92Q mutation from her mother and a pyrin E230K mutation from her father. To our knowledge, this is the first report of a patient with severe childhood multiple sclerosis and mutations in two genes which predispose to hereditary autoinflammatory disorders. We speculate that these mutations contribute to early multiple sclerosis manifestation and enhance the inflammatory damage inflicted by the autoimmune response.