Long-Term Outcomes in Ventriculoatrial Shunt Surgery in Patients with Pediatric Hydrocephalus: Retrospective Single-Center Study.

OBJECTIVE: Long-term outcomes are rarely reported for patients with pediatric hydrocephalus. Ventriculoperitoneal shunting is the surgical standard; nevertheless, in selected patients, a ventriculoatrial shunt (VAS) remains an important alternative. This study aimed to analyze the causes of VAS revisions and complications. METHODS: Pediatric patients who underwent their first shunt operation between 1982 and 1992 were included. The timing, cause, and modality of VAS revisions were retrospectively determined. RESULTS: Overall, 138 patients were treated for hydrocephalus and 61 patients received a VAS during the follow-up period. A primary VAS was the first shunt type in 42 (68.85%) patients. In 19 (31.15%) patients, conversions to second-line VAS were carried out. The rates of VAS revisions performed for dysfunction or elective lengthening of a short atrial catheter were 52.2% and 22.9%, respectively. There was no difference in the number of VAS revisions between patients with primary VASs and second-line VASs. Age at VAS and etiology of hydrocephalus had no effect on the number of revisions. Specific VAS complications were observed in 2 patients. Deep positioning of the distal catheter led to asymptomatic tricuspid regurgitation that was reversible after shortening of the atrial catheter. Another patient presented with shunt nephritis and completely recovered after the atrial catheter was replaced with a peritoneal catheter. CONCLUSIONS: VAS remains an appropriate second-line alternative in selected patients. Specific VAS complications were rarely observed and completely reversible after treatment. However, regular and specific follow-up examinations are strongly recommended to avoid cardiopulmonary or renal complications.

Adult Outcome in Shunted Pediatric Hydrocephalus: Long-Term Functional, Social, and Neurocognitive Results.

OBJECTIVE: Very long-term outcomes are rarely reported for patients with shunted pediatric hydrocephalus. This study aimed to determine the functional, social, and neurocognitive outcomes of such patients after transition to adulthood. METHODS: Adult patients with pediatric hydrocephalus who underwent their first shunt operation between 1982 and 1992 were included. Functional, social, educational, working aspects, and verbal intelligence were evaluated. In patients with average or above average verbal intelligence, detailed neuropsychological testing was performed and memory, executive functioning, selective attention, and concentration were assessed. RESULTS: Overall, 137 patients underwent primary surgery because of pediatric hydrocephalus, 53 (38.7%) of whom died during the follow-up period. Of the 84 long-term survivors, 65 (77.4%) agreed to participate and were included for further analysis. Forty-five patients (69.2%) had completed secondary school, but only 34 (52.3%) were integrated in the open labor market. Although the verbal intelligence of 31 patients (47.7%) was within the normal range, 19 (29.2%) had a severe mental handicap. Shunt infections (P = 0.0025), epilepsy (P < 0.0001), and the number of shunt operations (P = 0.0082) were associated with reduced verbal intelligence. Most patients with average or above average verbal intelligence had deficits in detailed neuropsychological testing. In 23 patients, detailed neuropsychological testing was performed. CONCLUSIONS: The overall long-term outcome of patients with shunted pediatric hydrocephalus is poor. These results highlight the importance of lifelong routine controls to avoid later complications. Further, repeated neuropsychological examinations might be important to understand the patient's special needs to optimize professional support.

Intrathecal baclofen in metachromatic leukodystrophy.

Metachromatic leukodystrophy (MLD) is a rare progressive neurological disorder, often accompanied by motor impairments that are challenging to treat. In this case series, we report the course of treatment with intrathecal baclofen (ITB), aimed at improving daily care and comfort in children and young adults with MLD. All patients with MLD in our centre on ITB treatment for a minimum of 6 months were included (n=10; 4 males, 6 females; mean age 10y 8mo [range 6-24y]). Eight patients had MLD with a predominant spastic movement disorder (sMLD) and two were mainly dyskinetic. Patients with sMLD were compared with matched patients with spastic cerebral palsy (CP). Complication rates related to ITB treatment were similar in both groups. ITB treatment course in the first 6 months after pump implantation appears to show more dose increase in most patients MLD, compared to patients with spastic CP. This may be due to the progressive disease in MLD. ITB is a feasible therapy to improve daily care and comfort in patients with MLD and should therefore be considered early. WHAT THIS PAPER ADDS: Intrathecal baclofen (ITB) is a feasible therapy to improve comfort and daily care in children and young people with metachromatic leukodystrophy (MLD). In the first 6 months of ITB treatment, MLD seems to show more dose increase compared to spastic cerebral palsy.

Factors Associated With Long-Term Improvement of Gait After Selective Dorsal Rhizotomy.

OBJECTIVE: To identify factors associated with long-term improvement in gait in children after selective dorsal rhizotomy (SDR). DESIGN: Retrospective cohort study. SETTING: University medical center. PARTICIPANTS: Children (N=36) (age 4-13y) with spastic diplegia of Gross Motor Function Classification System (GMFCS) level I (n=14), II (n=15), and III (n=7) were included retrospectively from the database of our hospital. Children underwent SDR between January 1999 and May 2011. Patients were included if they received clinical gait analysis before and 5 years post-SDR, age >4 years at time of SDR and if brain magnetic resonance imaging (MRI) scan was available. INTERVENTION: Selective dorsal rhizotomy. MAIN OUTCOME MEASURES: Overall gait quality was assessed with Edinburgh visual gait score (EVGS), before and 5 years after SDR. In addition, knee and ankle angles at initial contact and midstance were evaluated. To identify predictors for gait improvement, several factors were evaluated including functional mobility level GMFCS, presence of white matter abnormalities on brain MRI, and selective motor control during gait (synergy analysis). RESULTS: Overall gait quality improved after SDR, with a large variation between patients. Multiple linear regression analysis revealed that worse score on EVGS and better GMFCS were independently related to gait improvement. Gait improved more in children with GMFCS I and II compared to III. No differences were observed between children with or without white matter abnormalities on brain MRI. Selective motor control during gait was predictive for improvement of knee angle at initial contact and midstance, but not for EVGS. CONCLUSION: Functional mobility level and baseline gait quality are both important factors to predict gait outcomes after SDR. If candidates are well selected, SDR can be a successful intervention to improve gait both in children with brain MRI abnormalities as well as other causes of spastic diplegia.

Evolution of gait in adolescents and young adults with spastic diplegia after selective dorsal rhizotomy in childhood: A 10 year follow-up study.

BACKGROUND: Short-term benefit on gait of selective dorsal rhizotomy (SDR) surgery, which relieves spasticity of the lower extremities has been demonstrated in children with cerebral palsy (CP). However very little is known of the evolution of gait when patients become adolescents and young adults. RESEARCH QUESTION: How does the gait pattern evolve in adolescents and young adults who underwent SDR during childhood? METHODS: A longitudinal study was performed including 19 ambulant patients with spastic diplegia due to CP or other causes (mean age at SDR: 6.6 ±â€¯1.6 years) who were assessed four times: pre-SDR, 2 years post- SDR, 5 years post-SDR and at least 10 years post-SDR. From 2D video recordings, Edinburgh Visual Gait Score and lower limb joint kinematic parameters were calculated. RESULTS: Our data show that the improvement in the gait pattern obtained short-term after SDR continues during into adolescence and adulthood. Ten years after SDR all patients improved compared to baseline. Considering the lower limb joint kinematics, most notable improvements were found at knee and ankle joints. Compared to the evaluation before SDR, the range of motion of the knee increased: the knee was more extended at initial contact and knee flexion in midswing improved. Excessive ankle plantar flexion was reduced during the entire gait cycle. Only minor changes were found at hip and pelvis. Eight patients underwent additional orthopaedic surgery in the years after SDR, and the present findings should be considered as a combination of SDR, development and additional treatment. SIGNIFICANCE: We demonstrate lasting improvement of gait quality in ambulant patients with spastic diplegia who underwent SDR during childhood when they become adolescents and young adults.

Abdominal Pseudocysts and Peritoneal Catheter Revisions: Surgical Long-Term Results in Pediatric Hydrocephalus.

OBJECTIVE: An abdominal pseudocyst (APC) is a distal catheter site-specific failure in patients treated with ventriculoperitoneal shunts. Few studies with more than 10 patients have been reported. The aim of this study was to analyze causes of peritoneal catheter revisions with special emphasis on revisions because of an APC. METHODS: Pediatric patients with first shunt operation between 1982 and 1992 were included, and time, cause, and modality of peritoneal catheter revision were determined retrospectively. RESULTS: One hundred thirty-eight patients were treated for hydrocephalus, and 112 patients received a peritoneal catheter during the follow-up. An APC was diagnosed in 14 (12.5%) patients, and 28 revisions were needed for its treatment. The rate of shunt infection in patients with APC was 50%, but bacterial examination of the pseudofluid culture revealed infection in only 3 patients. Age at first surgical procedure, type of first surgical procedure, and etiology of hydrocephalus were not associated with APC diagnosis. APC recurred in 4 patients. These patients had a catheter repositioning directly into the peritoneum as first surgical treatment. No recurrences were observed in patients with shunt externalization or replacement of the peritoneal catheter. CONCLUSIONS: An APC is a major long-term complication after ventriculoperitoneal shunt treatment. Although a sterile inflammatory response cannot be excluded completely, our results favor the hypothesis of low-level shunt infection. In both cases, the surgical consequences are the same. An infected APC should be treated as a shunt infection. Uninfected patients can be treated with shunt externalization and replacement of only the peritoneal catheter.

Long-term mortality rates in pediatric hydrocephalus-a retrospective single-center study.

PURPOSE: Very long-term follow-up and outcome are rare for pediatric patients with hydrocephalus and shunt operations. The aim of this study was to determine the long-term mortality rates in these patients. METHODS: Pediatric patients with first shunt operation between 1982 and 1992 were included. For each patient, time and cause of death were determined. Further, patients with first operation from 1982 to 1987 were compared to those first operated from 1988 to 1992. RESULTS: One-hundred thirty-seven patients were included. Etiologies of hydrocephalus were intraventricular hemorrhage (31.4 %), meningomyelocele (25.5 %), postinfectious (11.7 %), congenital (10.2 %), posterior fossa cyst (8.8 %), aqueductal stenosis (8 %), and others (4.4 %). Overall, 53 patients (38.7 %) died. The percentage of patients surviving 1, 2, 10, and 20 years after first operation were 82.6, 73.6, 69.4, and 65.3 %, respectively. In 23 patients, the cause of death was related to shunt treatment: shunt infection was diagnosed in 18 and acute shunt dysfunction in 5 patients. Mortality was considerably higher for patients with their first operation in time period 1982-1987 compared to time period 1988-1992 (51 versus 25 %). The reduction of mortality was mainly due to an increased survival after shunt infection. Eighty-seven patients survived more than 20 years after initial shunt operation. Of those long-term survivors, three (3.4 %) patients died 22-24 years after first operation. CONCLUSION: Mortality in hydrocephalic pediatric patients is high especially in the first postoperative years but is even significant in adult patients with pediatric hydrocephalus. As deaths occur even after 20 years, routine follow-up of long-term survivors remains necessary.

Sudden falls as a persistent complication of selective dorsal rhizotomy surgery in children with bilateral spasticity: report of 3 cases.

Selective dorsal rhizotomy (SDR) surgery is a well-established treatment for ambulatory children with bilateral spastic paresis and is performed to eliminate spasticity and improve walking. The objective of this case report is to describe sudden falls as a persistent complication of SDR. The authors report on 3 patients with bilateral spastic paresis, aged 12, 6, and 7 years at the time of surgery. The percentage of transected dorsal rootlets was around 40% at the L2-S1 levels. Sudden falls were reported with a frequency of several a day, continuing for years after SDR. The falls were often triggered by performing dual tasks as well as occurring in the transition from sitting to standing, during running, after strenuous exercise, or following a fright. Patients also had residual hyperesthesia and dysesthesia of the foot sole. The authors hypothesize that the sudden falls are caused by a muscle inhibition reflex of the muscles in the legs, as an abnormal reaction to a sensory stimulus that is perceived with increased intensity by a patient with hyperesthesia. A favorable effect of gabapentin medication supports this hypothesis.

The effect of intrathecal baclofen treatment on activities of daily life in children and young adults with cerebral palsy and progressive neurological disorders.

INTRODUCTION: Intrathecal baclofen (ITB) treatment is applied in patients with spastic cerebral palsy (SCP), dystonic cerebral palsy (DCP) and progressive neurological disease (PND). Our aim was to investigate whether ITB treatment has a different effect on activities of daily life (ADL) in these groups. METHOD: A retrospective and cross-sectional survey was conducted using a questionnaire to assess the qualitative effect of ITB (Likert scale) on different domains of functioning (mobility, personal care, communication, comfort) and satisfaction with the results. Groups were compared using non-parametric statistics. RESULTS: Questionnaires were completed for 68 patients (39 SCP, 13 DCP, 16 PND). Satisfaction scores were relatively high in all groups (7-8) and the positive effect on personal care and communication was similar in all groups. The PND group had the shortest follow-up and scored significantly less favourably for the effect on mobility and comfort. DISCUSSION: This is the first study to show that ITB treatment has similar effects on personal care and communication in stable and progressive neurological disease. The decrease in mobility in the PND group is likely due to the progressive nature of the disease. The different effect on comfort between groups is mainly due to the smaller effect on startles in the PND group.

Symptomatic popliteal venous aneurysm causing a footdrop.

Symptomatic aneurysms of the popliteal vein are uncommon, with the majority resulting in thromboembolic complications. Neurologic symptoms are extremely rare. We present a case of a 53-year-old man with a footdrop resulting from a saccular popliteal venous aneurysm. Compression of the peroneal nerve caused the neurologic deficit. After aneurysmectomy and lateral venorrhaphy, the patient regained full strength of his lower leg muscles. Neurologic complications caused by a popliteal venous aneurysm should be considered in patients with a swelling in the popliteal fossa and a neurologic deficit of the lower extremity.

Long-term outcome of accessory nerve to suprascapular nerve transfer in obstetric brachial plexus lesion: functional, morphological, and electrophysiological results.

PURPOSE: An accessory to suprascapular nerve (XIN-SSN) transfer is considered in patients with obstetric brachial plexus lesion who fail to recover active shoulder external rotation. The aim of this study was to evaluate the quality of extraplexal suprascapular nerve neurotization and to perform a detailed analysis of the infraspinatus muscle (IM) and shoulder external rotation. METHODS: A XIN-SSN transfer was performed in 14 patients between 2000 and 2007. Patients had been operated at the age of 3.7 ± 2.8 years. Follow-up examinations were conducted up to 8.5 ± 2.5 years. Magnetic resonance imaging was performed to investigate muscle trophism. Fatty muscle degeneration of the IM was classified according to the Goutallier classification. We conducted nerve conduction velocity studies of the suprascapular nerve and needle electromyography of the IM to assess pathologic spontaneous activity and interference patterns. Active glenohumeral shoulder external rotation and global shoulder function were evaluated using the Mallet score. RESULTS: Postoperatively, growth of the IM increased equally on the affected and unaffected sides, although significant differences of muscle thickness persisted over time. There was only grade 1 or 2 fatty degeneration pre- and postoperatively. Electromyography of the IM revealed a full interference pattern in all except one patient, and there was no pathological spontaneous activity. Glenohumeral external rotation as well as global shoulder function increased significantly. CONCLUSION: Our results indicate that the anastomosis after XIN-SSN transfer is functional and that successful reinnervation of the infraspinatus muscle may enable true glenohumeral active external rotation.

Elbow Flexion Contractures in Childhood in Obstetric Brachial Plexus Lesions: A Longitudinal Study of 20 Neurosurgically Reconstructed Infants with 8-Year Follow-up.

OBJECTIVE: Little knowledge exists on the development of elbow flexion contractures in children with obstetrical brachial plexus lesion (OBPL). This study aims to evaluate the prognostic significance of several neuromuscular parameters in infants with OBPL regarding the later development of elbow flexion contractures. METHODS: Twenty infants with OBPL with insufficient signs of recovery in the first months of life who were neurosurgically reconstructed were included. At a mean age of 4.6 months, the following neuromuscular parameters were assessed: existence of flexion contractures, cross-sectional area (CSA) of upper arm muscles on MRI, Narakas classification, EMG results, and elbow muscle function using the Gilbert score. In childhood at follow-up at mean age of 7.7 years, we measured the amount of flexion contractures and the upper arm peak force (Newton). Statistical analysis is used to assess relations between these parameters. RESULTS: Flexion contractures of greater than 10 degrees occurred in 55% of our patient group. The relation between the parameters in infancy and the flexion contractures in childhood is almost nonexistent. Only the Narakas classification was related to the development of flexion contractures in childhood (p = 0.006). Infant muscle CSA is related to childhood peak muscle force. CONCLUSION: The role of infancy upper arm muscle hypotrophy/hypertrophy, reinnervation, and early elbow muscle function in the development of childhood elbow contractures remains unclear. In this cohort prediction of childhood flexion, contractures were not possible using infancy neuromuscular parameters. We suggest that contractures might be an adaptive process to optimize residual muscle function.

Intrathecal baclofen treatment in dystonic cerebral palsy: a randomized clinical trial: the IDYS trial.

BACKGROUND: Dystonic cerebral palsy is primarily caused by damage to the basal ganglia and central cortex. The daily care of these patients can be difficult due to dystonic movements. Intrathecal baclofen treatment is a potential treatment option for dystonia and has become common practice. Despite this widespread adoption, high quality evidence on the effects of intrathecal baclofen treatment on daily activities is lacking and prospective data are needed to judge the usefulness and indications for dystonic cerebral palsy. The primary aim of this study is to provide level one clinical evidence for the effects of intrathecal baclofen treatment on the level of activities and participation in dystonic cerebral palsy patients. Furthermore, we hope to identify clinical characteristics that will predict a beneficial effect of intrathecal baclofen in an individual patient. METHODS/DESIGN: A double blind placebo-controlled multi-center randomized clinical trial will be performed in 30 children with dystonic cerebral palsy. Patients aged between 4 and 25 years old with a confirmed diagnosis of dystonic cerebral palsy, Gross Motor Functioning Classification System level IV or V, with lesions in the cerebral white matter, basal ganglia or central cortex and who are eligible for intrathecal baclofen treatment will be included. Group A will receive three months of continuous intrathecal baclofen treatment and group B will receive three months of placebo treatment, both via an implanted pump. After this three month period, all patients will receive intrathecal baclofen treatment, with a follow-up after nine months. The primary outcome measurement will be the effect on activities of and participation in daily life measured by Goal Attainment Scaling. Secondary outcome measurements on the level of body functions include dystonia, spasticity, pain, comfort and sleep-related breathing disorders. Side effects will be monitored and we will study whether patient characteristics influence outcome. DISCUSSION: The results of this study will provide data for evidence-based use of intrathecal baclofen in dystonic cerebral palsy.

Long-term effect of selective dorsal rhizotomy on gross motor function in ambulant children with spastic bilateral cerebral palsy, compared with reference centiles.

AIM: The aim of this study was to evaluate the long-term effect of selective dorsal rhizotomy (SDR) on the gross motor function of ambulant children with spastic bilateral cerebral palsy (CP), compared with reference centiles. METHOD: The study used a prospective cohort design and participants comprised 29 children classified using the Gross Motor Function Classification System (GMFCS) in level I (n=7), II (n=4), or III (n=18; 18 males, 11 females; median age at time of surgery 6 y 4 mo; range 2 y 10 mo-12 y 1 mo), who were examined 5 years and 10 years after SDR. We used individual centiles based on Gross Motor Function Measure (GMFM-66) scores and age, corresponding to the GMFCS levels. Individual improvement or deterioration was defined as a change of more than 20 centiles. Side effects experienced and additional treatment received after SDR were also recorded. RESULTS: Five years after SDR, 10 out of 28 children showed improvement, and 10 years after SDR 6 out of 20 children had improved. Spinal side effects were noted in two children and hip subluxation in three. Additional treatments included subtalar arthrodesis (n=13), endorotational osteotomy of the tibia (n=5), and botulinum toxin treatment (n=13). INTERPRETATION: None of the children showed deterioration of gross motor function based on centile ranking. Five and 10 years after SDR, gross motor function in some children had improved more than would have been expected according to the reference centiles. This suggests, taking the limitations of this study into account, that the applied criteria for selection were adequate. However, the children still required additional treatment after SDR.

[A man with severe traumatic brain injury]. / Een man met ernstig traumatisch schedelhersenletsel.

We present a 41-year-old man with severe traumatic brain injury. Cranial imaging studies revealed cerebral contusion and a longitudinal fracture of the temporal bone. Several days later brain herniated into the left external auditory canal. Imaging studies showed the known skull fracture with a direct connection between the external acoustic meatus and the intracranial structures.

Musculoskeletal growth in the upper arm in infants after obstetric brachial plexus lesions and its relation with residual muscle function.

AIM: Denervation after obstetric brachial plexus lesion (OBPL) is associated with reduced musculoskeletal growth in the upper arm. The aim of this study was to investigate whether reduced growth of upper arm flexor and extensor muscles is related to active elbow function and humeral length. METHOD: In this study, 31 infants age less than 6 months (mean age 4.3mo; range 2.1-5.9mo; 17 males; 14 females;) with unilateral OBPL (Narakas class I, 19; II, 3; III, 2; and IV, 7) treated at the VU medical centre, in whom neurosurgical reconstruction was considered were prospectively studied using magnetic resonance imaging of both arms at a mean age of 4.3 months. Humeral length and the cross-sectional area (CSA) of elbow flexor and extensor muscles were measured in both upper arms. Paresis of elbow function was estimated when the infants were a mean age of 4.5 months using the Gilbert score. RESULTS: Both flexor and extensor CSAs were significantly smaller on the affected side than on the unaffected side (88% [SD 32%], p=0.020, and 88% [SD 24%], p=0.001 respectively), as was humeral length (96% [SD 7%], p=0.005) (unaffected side 100% in all cases). There was no relation between the reduction in flexor and extensor CSA and residual muscle function. In 17 out of 31 patients, hypertrophy of flexor and/or extensor muscles was observed. Humeral length was not related to muscle parameters. INTERPRETATION: Denervation has different effects on muscle growth and function as well as bone growth. In young infants with an OBPL, muscle size is not a predictor of muscle function. Flexion contractures of the elbow later in childhood may not be explained by a dominance of flexor muscle mass in infants.

Pediatric meningiomas in The Netherlands 1974-2010: a descriptive epidemiological case study.

OBJECTIVE: The purpose of this study was to review the epidemiology and the clinical, radiological, pathological, and follow-up data of all surgically treated pediatric meningiomas during the last 35 years in The Netherlands. METHODS: Patients were identified in the Pathological and Anatomical Nationwide Computerized Archive database, the nationwide network and registry of histopathology and cytopathology in The Netherlands. Pediatric patients of 18 years or younger at first operation in 1974-2009 with the diagnosis meningioma were included. Clinical records, follow-up data, radiological findings, operative reports, and pathological examinations were reviewed. RESULTS: In total, 72 patients (39 boys) were identified. The incidence of operated meningiomas in the Dutch pediatric population is 1:1,767,715 children per year. Median age at diagnosis was 13 years (range 0-18 years). Raised intracranial pressure and seizures were the most frequent signs at presentation. Thirteen (18 %) patients had neurofibromatosis type 2 (NF2). Fifty-three (74 %) patients had a meningioma World Health Organization grade I. Total resection was achieved in 35 of 64 patients. Fifteen patients received radiotherapy postoperatively. Mean follow-up was 4.8 years (range 0-27.8 years). Three patients died as a direct result of their meningioma within 3 years. Four patients with NF2 died as a result of multiple tumors. Nineteen patients had disease progression, requiring additional treatment. CONCLUSION: Meningiomas are extremely rare in the pediatric population; 25 % of all described meningiomas show biological aggressive behavior in terms of disease progression, requiring additional treatment. The 5-year survival is 83.9 %, suggesting that the biological behavior of pediatric menigiomas is more aggressive than that of its adult counterparts.

Intrathecal baclofen for progressive neurological disease in childhood: a systematic review of literature.

BACKGROUND: Intrathecal baclofen (ITB) treatment is frequently used for individuals with severe, but non-progressive, spasticity refractory to oral treatment. However, experiences with ITB in patients with progressive neurological disorders of childhood causing spasticity are limited. AIM: To investigate whether ITB is an option in patients with progressive neurological disorders causing spasticity in childhood. DESIGN: A systematic literature search in Embase, Pubmed and the Cochrane Library was performed. RESULTS: We identified six eligible studies considering patients with progressive neurological disease in childhood and receiving ITB treatment. The studies included a total of seven paediatric patients and four adult patients. Improvement was reported in spasticity, spasms, pain, gait, activities of daily life and providing care. High satisfaction is described. CONCLUSIONS: ITB has beneficial effects in paediatric patients with progressive neurological disease. However, the level of evidence is limited due to the small number of available studies and due to the poor quality of these studies.

Effects of intrathecal baclofen on daily care in children with secondary generalized dystonia: a pilot study.

AIM: Treatment options for dystonic cerebral palsy (CP) are limited. Our aims were to determine whether intrathecal baclofen (ITB) improves daily care, decreases dystonia and decreases pain in patients with dystonic CP. METHODS: Patients received randomized blinded treatment with ITB or placebo. Scores on problems of daily care were recorded and dystonia, pain and comfort were assessed. RESULTS: Four patients (three males, average age 12 years 6 months) were included (all Gross Motor Function Classification System level V). During the trial period problem scores and dystonia scores decreased in all four patients. CONCLUSION: In this pilot study we report positive functional effects of ITB trial treatment in four patients with dystonic CP. A randomized trial with a larger cohort is needed to verify these results.

Short- and long-term effects of selective dorsal rhizotomy on gross motor function in ambulatory children with spastic diplegia.

OBJECT: The primary aim of this prospective cohort study was to evaluate the short-term (1 year) and long-term (mean 6 years) effects of selective dorsal rhizotomy (SDR) on gross motor function and spasticity in ambulatory children with spastic diplegia. Secondary aims were to investigate side effects, additional treatment during follow-up (botulinum toxin type A injections or orthopedic surgery), and parental satisfaction. METHODS: Thirty-three children who had undergone SDR at a mean age of 6 years and 7 months (± 2 years) were included. There were 7 children at Gross Motor Function Classification System (GMFCS) Level I, 7 at Level II, and 19 at Level III. Gross motor function was assessed with the Gross Motor Function Measure-66 (GMFM-66). Spasticity was measured according to a modified Tardieu scale. Side effects, additional treatment, and parental satisfaction were recorded using a parental questionnaire and medical records. RESULTS: At 1-year follow-up, mean GMFM-66 scores improved significantly by 4.3 ± 4.1 points. Children at GMFCS Levels I and II showed significantly more improvement (7.2 points) on the GMFM-66 compared with children at GMFCS Level III (2.9 points). On long-term follow-up (mean 6 years ± 22 months), mean GMFM-66 scores improved significantly by 6.5 ± 5.9 points, without a difference between children at GMFCS Levels I and II and Level III. No relapse of spasticity was noted. Ten children (30%) needed orthopedic surgery and 13 children (39%) received botulinum toxin type A treatment after SDR. Twenty (91%) of the 22 parents who answered the questionnaire at long-term follow-up believed that their child's functioning had improved after SDR. CONCLUSIONS: Selective dorsal rhizotomy resulted in short- and long-term improvements in gross motor function, without relapse of spasticity. However, the majority of the children still needed additional surgery or botulinum toxin A treatment.