Children with FASD-Evolving Patterns of Developmental Problems and Intervention Costs in Ages 0 through 16 in Finland.

This is a retrospective chart review of 55 persons (mean age 11 years, range 2-28 years) diagnosed with fetal alcohol spectrum disorder (FASD) in one Finnish central hospital. The aim was to determine typical problems and interventions and estimate their costs during different periods of childhood between ages 0 and 16. During the first year, 29/38 (76.3%) were treated in the neonatal intensive care unit, 29/43 (67.4%) received physiotherapy, 15/43 (34.9%) were diagnosed with congenital malformation, 8/43 (18.6%) had heart defects. Between 1 and 6 years, 39/45 (86.7%) received occupational therapy, 25/45 (55.6%) speech therapy, and 12/45 (26.7%) were diagnosed with strabismus. Between 7 and 12 years, 25/37 (67.6%) were diagnosed with ADHD and special education was recommended for 30/37 (81.1%). Learning disorders and the need for psychiatric care increased with age. Between 13 and 16 years, 12/15 (80%) were treated in the psychiatric unit, and by this age, 8/15 (53.3%) were diagnosed with intellectual disability. Before 16 years, 44/55 (80%) were placed out of home, which caused 78.5% of the estimated cumulative mean extra costs of EUR 1,077,000 in 2022 currency. Except for psychiatric costs, health care costs were highest during early years. Charting typical patterns of problems may help in identifying children with FASD and planning follow-ups, content of assessments, and interventions.

Challenges and Neuropsychological Functioning in Children and Adolescents with Borderline Intellectual Functioning.

This retrospective chart review study sought to explore neuropsychological profiles, neuropsychiatric and psychiatric comorbidity, changes in diagnoses, support at daycare and school, medication use, psychiatric referrals, and progression into further education in a cohort of participants with borderline intellectual functioning (BIF). Additionally, developmental factors connected to BIF were studied. Delays in language and gross motor development were the initial reasons for the parents to seek health care. Comorbid neuropsychiatric and psychiatric diagnoses were frequent, a total of 41% of participants were referred to psychiatric services, and 45% used medication. Educational support was needed by 92% of the study participants. The majority of those graduating elementary school continued their studies at ordinary or special vocational schools. The risk of dropping out during secondary studies appeared to increase. The results in most of the neuropsychological subdomains declined over time, and 23% of the participants were later diagnosed with an intellectual disability (ID). The early developmental signs pointing towards BIF and the need for prompt support were a delay in language and motor development, difficulties in executive function, a delay in learning the activities of daily living among children under school age, and difficulties in reading and arithmetic skills and abstract reasoning at school age. It is important to follow up and support individuals with BIF as their risk for being left behind in the society is increased. Also, it would be important to repeat the neuropsychological testing of cognitive and adaptive functions before graduating elementary school as to capture those who meet the ID criteria.

Children, Adolescents, and Young Adults with Borderline Intellectual Functioning: Etiological, Neurophysiological, and Mri Findings in a Cohort of 651 Patients.

This retrospective chart review study explored the etiology, use, and yield of the etiological investigations of 651 children and adolescents diagnosed with borderline intellectual functioning (BIF). Neurological, neurodevelopmental, or neuropsychiatric comorbidities were frequent, and in 23%, the BIF diagnosis evolved into an intellectual disability (ID) by the time of discharge. A primary etiological cause was found in 37.6%, the most prevalent causes being pre- or perinatal conditions, genetic syndromes/chromosomal abnormalities, fetal exposure to maternal substance use, cerebral dysgenesis, and neurological diseases. In total, 79.1% of patients went through one or more investigations during their follow-up. The best etiologic yield leading to a diagnosis in this study population was with exome sequencing, a specific gene panel, microarrays, electroneuromyography, and brain magnetic resonance imaging (MRI). Etiological investigations were performed more frequently among those children receiving an ID diagnosis. Yet, there was no statistically significant difference in the proportion of abnormal findings between the BIF and ID groups. This may mean that the current strategy for determining the need for etiological investigations or current means to gain an etiology is still indecisive. Considering that BIF is defined to include individuals performing between normal cognitive functioning and mild ID, this implies that the prevalence would be anywhere between 7 and 14%. Thus, it could be argued whether in-depth etiological investigations may be justified in cases other than ID in this age group of children over five. With these children and adolescents, the clinicians have to discern between those with a normal variation and those having major difficulties in adaptive behavior affecting everyday life in order to specify and prescribe the rehabilitation or other measures needed. We advocate for a targeted etiological search after careful history-taking and neurological examination. National guidelines that take into account the severity of developmental delay are warranted.

Over 25 Years of Pediatric Botulinum Toxin Treatments: What Have We Learned from Injection Techniques, Doses, Dilutions, and Recovery of Repeated Injections?

Botulinum toxin type A (BTXA) has been used for over 25 years in the management of pediatric lower and upper limb hypertonia, with the first reports in 1993. The most common indication is the injection of the triceps surae muscle for the correction of spastic equinus gait in children with cerebral palsy. The upper limb injection goals include improvements in function, better positioning of the arm, and facilitating the ease of care. Neurotoxin type A is the most widely used serotype in the pediatric population. After being injected into muscle, the release of acetylcholine at cholinergic nerve endings is blocked, and a temporary denervation and atrophy ensues. Targeting the correct muscle close to the neuromuscular junctions is considered essential and localization techniques have developed over time. However, each technique has its own limitations. The role of BTXA is flexible, but limited by the temporary mode of action as a focal spasticity treatment and the restrictions on the total dose deliverable per visit. As a mode of treatment, repeated BTXA injections are needed. This literature reviewed BTXA injection techniques, doses and dilutions, the recovery of muscles and the impact of repeated injections, with a focus on the pediatric population. Suggestions for future studies are also discussed.

[Riding therapy in the rehabilitation of mobility-impaired children]. / Ratsastusterapia liikuntavammaisten lasten kuntoutuksessa.

Riding therapy is a comprehensive and functional form of rehabilitation, in which the rehabilitee, the horse and the riding therapist collaborate in order to achieve individually assigned goals that support rehabilitation. In Finland, riding therapy is therapeutic rehabilitation carried out by riding therapists who have undergone approved training. The therapy is mainly implemented in an individual form, but small group working is also applied, e.g. in the form of pair therapy and therapeutic vaulting. In Europe, this form of rehabilitation has been divided into hippotherapy supporting motor functions and heilpedagogical riding therapy functioning in support of upbringing.

Does Botulinum Toxin A Treatment Enhance the Walking Pattern in Idiopathic Toe-Walking?

Objective We conducted a randomized controlled trial to evaluate whether a combination of repeated botulinum toxin A (BTX-A) and conservative treatment is more effective in decreasing toe-walking than conservative treatment alone at 24 months follow-up. Patients and Methods Children between 2 and 9 years of age were randomized either into the conservative (CO) or botulinum treatment (BTX) group. The treatment in the CO group consisted of firm shoes, night splints, a home stretching program and physiotherapy. The BTX arm had all the same conservative treatments added with calf muscle BTX-A injections repeated in 6 months intervals if needed. Change in toe-walking pattern, ankle range of movement (ROM), and overall function were assessed at baseline and 6, 12, 18, and 24 months posttreatment. Results A total of 30 toe-walkers participated: 14 in CO and 16 in BTX group. At 24 months, all children in the BTX group and 85% in the CO group evaluated by the blinded physiotherapist (p = 0.065), 75% in the BTX group and 70% in the CO group graded by the research physiotherapist (p = 0.730), and 50% in the BTX group and 54% in the CO group reported by the parents ceased toe-walking (p = 0.837). The most prominent change was noted during the 1st year. The BTX group seemed to reach the goal earlier. No significant differences between the treatment groups in function or in ankle ROM ensued. Conclusion Adding BTX injections did not significantly enhance the goal to walk either flat foot or with heel strike at 24 months posttreatment.

[Congenital disorder of glycosylation type Ia (CDG Ia) - underdiagnosed entity?]. / Synnynnäinen tyypin Ia glykosylaation häiriö.

Congenital disorders of glycosylation (CDG) are a relatively recently identified group of multisystem disorders caused by defective glycosylation of N-glycosylated proteins. They mainly involve the central and peripheral nervous system, but other organ systems are involved as well. Type CDG Ia accounts for over 80% of cases, characterized by decreased activity of the enzyme phosphomannomutase caused by mutations in chromosome 16 PMM2 gene. Treatment of CDG Ia remains symptomatic.

[Update on Current Care Guideline: Headache (children)]. / Päänärky (lapset).

The majority of children with recurrent headaches can be effectively treated in the primary health care. Paracetamol and ibuprofen are the recommended first-line pain medications. Limited evidence is available on the effectiveness of triptans in children and adolescents. However, nasal sumatriptan and possibly oral rizatriptan and nasal zolmitriptan can be considered for children and adolescents, as well as oral almotriptan for adolescents. Propranolol is the first-line prophylactic medication for migraine.

Hirayama disease.

Hirayama is a form of cervical myelopathy affecting mainly young men. The cardinal features include progressive, either symmetrical or asymmetrical muscular weakness and atrophy of muscles innervated by C7-Th1 motoneurons. The application of soft daytime collar during the early stage of illness can halt the progression of illness.

[Toe walking--follow or treat?]. / Varvaskävely--seurata vai hoitaa?

Idiopathic toe walking refers to continual or intermittent symmetrical gait pattern, in which the normal heel strike is lacking and the weight of the gait is on the ball of the foot. Many toe walkers are able to walk normally if they so wish. Diagnosis is established by excluding any neurological, neuromuscular, structural and orthopedic causes. Toe walkers often exhibit problems in sensory integration. Idiopathic toe walking is considered a benign phenomenon, usually self-limiting before school age, but upon becoming prolonged it may cause physical and social handicap for the child.

[The route of botulinum toxin from cause of food poisoning to medical remedy]. / Botuliinin tie ruokamyrkytyksen aiheuttajasta lääkkeeksi.

Botulinum toxins are amongst the most poisonous substances known in nature. The discovery and development of this toxin into a medical remedy is one of the most fascinating stories in the history of medicine. German physician Justinus Kerner founded the theory of treating hyperactive disorders with botulinum toxin and Alan Scott was the one to make this happen successfully. Nowadays the toxin is widely used in different indications, and the research is still going on for discovering novel tools for treating e.g. pain.

Increased incidence and clinical picture of childhood narcolepsy following the 2009 H1N1 pandemic vaccination campaign in Finland.

BACKGROUND: Narcolepsy is a rare neurological sleep disorder especially in children who are younger than 10 years. In the beginning of 2010, an exceptionally large number of Finnish children suffered from an abrupt onset of excessive daytime sleepiness (EDS) and cataplexy. Therefore, we carried out a systematic analysis of the incidence of narcolepsy in Finland between the years 2002-2010. METHODS: All Finnish hospitals and sleep clinics were contacted to find out the incidence of narcolepsy in 2010. The national hospital discharge register from 2002 to 2009 was used as a reference. FINDINGS: Altogether 335 cases (all ages) of narcolepsy were diagnosed in Finland during 2002-2009 giving an annual incidence of 0.79 per 100,000 inhabitants (95% confidence interval 0.62-0.96). The average annual incidence among subjects under 17 years of age was 0.31 (0.12-0.51) per 100,000 inhabitants. In 2010, 54 children under age 17 were diagnosed with narcolepsy (5.3/100,000; 17-fold increase). Among adults ≥20 years of age the incidence rate in 2010 was 0.87/100,000, which equals that in 2002-2009. Thirty-four of the 54 children were HLA-typed, and they were all positive for narcolepsy risk allele DQB1*0602/DRB1*15. 50/54 children had received Pandemrix vaccination 0 to 242 days (median 42) before onset. All 50 had EDS with abnormal multiple sleep latency test (sleep latency <8 min and ≥2 sleep onset REM periods). The symptoms started abruptly. Forty-seven (94%) had cataplexy, which started at the same time or soon after the onset of EDS. Psychiatric symptoms were common. Otherwise the clinical picture was similar to that described in childhood narcolepsy. INTERPRETATION: A sudden increase in the incidence of abrupt childhood narcolepsy was observed in Finland in 2010. We consider it likely that Pandemrix vaccination contributed, perhaps together with other environmental factors, to this increase in genetically susceptible children.

[Botulinum toxin treatment in children]. / Mitä annettavaa botuliinilla on lapsille?

Botulinum toxin type A treatments in children were started nearly 20 years ago. The first and still most common indication is spastic equinus gait in cerebral palsy, but other indications have emerged, such as idiopathic toe-walking, peri- and post-operative pain, drooling and idiopathic congenital torticollis. The official indication for botulinum toxin treatment is spastic equinus gait in children over 2 years of age. Botulinum toxin is known as the most potent toxin. However, it has proved to be safe and well tolerated among paediatric patients. Adverse events are infrequent, mostly bruising and limited, temporary muscle weakness. With higher doses the risk for generalized adverse events increases.

Botulinum toxin type A injections for treatment of spastic equinus in cerebral palsy: a secondary analysis of factors predictive of favorable response.

OBJECTIVE: To investigate markers predicting favorable botulinum toxin injection outcomes in treating spastic equinus gait in children with cerebral palsy. DESIGN: Thirty-six children (21 boys and 15 girls, aged 1.5-9.6 yrs; 20 with unilateral and 16 with bilateral cerebral palsy levels I to IV with the Gross Motor Function Classification System) with 49 treated lower limbs were included. Passive range of movement, selective dorsiflexion, dynamic muscle length (modified Tardieu Scale), calf tone (modified Ashworth Scale), attainment of anticipated gait pattern (Goal Attainment Scale), and video gait analysis (Observational Gait Scale) were assessed before and 1, 2, and 4 mos after intervention. Participants were classified as poor or good responders, according to the Goal Attainment Scale response at 2 mos. RESULTS: Good response was noted in 23 (64%) children and 29 (59%) legs, whereas 13 (36%) children and 20 (41%) legs were defined as poor responders. In a multivariate regression analysis, the best predictors of good response per child were higher initial Observational Gait Scale total scores, one-level treatment, and normal cognition; and the best predictors per leg were good initial selective motor control in the ankle and capability of occasional flatfoot contact at pretreatment. CONCLUSIONS: These results suggest that children with less severe functional deficit, fair to good selective motor control in the ankle, and mild equinus gait respond best to focal botulinum toxin type A treatment, with an eye to flatfoot or heel strike contact. Likewise, children with higher cognitive level seem to derive functional benefit from the treatment.

Botulinum toxin type A injections into the calf muscles for treatment of spastic equinus in cerebral palsy: a randomized trial comparing single and multiple injection sites.

OBJECTIVE: To investigate the hypothesis that the multiple-site injection technique is associated with better outcomes than the single-point injection method in children with cerebral palsy and spastic equinus gait. DESIGN: A total of 17 children (nine boys, eight girls ages 1.8-9.4 yrs; eight hemiplegics, eight diplegics, one quadriplegic; levels I-IV with the Gross Motor Function Classification System) with 25 treated lower limbs were randomized into two groups: a single-point group receiving a standard dose of botulinum toxin A injection into one site and a multiple-points group into two sites on both heads of the gastrocnemius. Active and passive range of movement, selective dorsiflexion, dynamic muscle length (modified Tardieu scale), calf tone (modified Ashworth scale), attainment of anticipated gait pattern (Goal Attainment Scale), and video gait analysis (Observational Gait Scale [OGS]) were assessed before and 1, 2, and 4 mos after intervention. RESULTS: Both groups improved in dynamic muscle length, muscle tone, OGS-total scores and initial foot contact scores and a similar number of children attained their goals on the Goal Attainment Scale. The only significant difference between the groups was observed at 2 mos in passive dorsiflexion with the knee flexed, favoring the single-point group. Though not significantly, the incidence of adverse effects was higher in the multiple-points group. CONCLUSIONS: Using the methods described, no major changes in main outcome measures were associated with the number of injection sites. Issues other than efficacy guide the decision on whether to inject in single or multiple sites when treating spastic equinus with botulinum toxin.

Upper limb function after botulinum toxin A treatment in cerebral palsy: two years follow-up of six cases.

The objective of this study was to investigate the effects of botulinum toxin A (BTXA) treatment on impairment and function of the upper limb during a 2-year follow-up period. A prospective longitudinal study design with assessments before and after intervention was utilized, involving six patients with cerebral palsy (three boys and three girls) aged 3 years 4 months to 11 years 11 months at commencement of study. The outcome measures were spasticity (modified Ashworth, MAS), active and passive range of movement (ROM), grips (pinch, key grip, 3-finger grip, narrow cylinder grip, wide cylinder grip, pen grip and diagonal grip; grasping, releasing; pronation-supination), bimanual functions, fine motor functions (Melbourne Assessment of Unilateral Upper Limb Function), movement pattern (Upper Limb Physician's Rating Scale, ULPRS), functional skills and self-care capability (Paediatric Evaluation of Disability Inventory, PEDI), upper extremity use (House Classification) and cosmetic appearance. The assessments were repeated by the same examiners at baseline and at 1, 3 and 6 months after each BTXA treatment and then every 6 months until 24 months. One subject received a total of four injections (at 0, 6, 12 and 18 months), one two injections (at 0 and 12 months) and four one injection at the beginning of the study period. Upper extremity surgery was performed on two subjects during the study and one was operated on 2 months after completion of the study. All children benefited from the BTXA treatment in terms of reduction in muscle tone and increase in active and passive ROM. By 6 months, spasticity returned, but in four children passive and especially active ROM remained better than at baseline. No significant changes in grips, bimanual tasks or Melbourne Assessment scores were detected. The change in movement pattern (ULPRS) was maintained for 3 months in two children and beyond this in four, thus extending beyond the pharmacologic effects of botulinum toxin A. All but one child showed improvement in PEDI functional skill and caregiver assistance scale scores during the 2-year period. The House classification showed a one-grade improvement in one child at 1 month and in one child at 3 months and a three-grade improvement in one child at 3 months after BTXA treatment. After each treatment, the parents reported at least a one-grade improvement in cosmetic appearance in all children at 1 month and in four children maintained at least until 6 months. In two subjects operated during the study period, a distinct improvement in active and passive ROM and a two-grade improvement in the House classification were observed after the operation. In this limited series, the reduction in muscle tone after BTXA treatment did not translate into better gripping or quality of fine motor functions (Melbourne Assessment) of the affected hand, but seemed to have a positive effect on upper limb movement pattern (ULPRS), upper extremity use (House Classification) and cosmetic appearance. Assessment of upper limb function in a child with cerebral palsy demands a variety of measures.

Low- and high-dose botulinum toxin A treatment: a retrospective analysis.

Upper limb botulinum toxin A doses in children are empirical, determined by the size of the muscle, seeking to avoid excessive weakness and deterioration of function. This study reports the effects and side effects of botulinum toxin treatment on upper limb impairment and function in 18 children with spastic or dystonic hyperactivity. A total of 54 treatments were divided into low-dose or high-dose groups according to the dose used for the target muscles. The outcome measurements included modified Ashworth Scale, passive range of movement, various grips, bimanual functions, movement pattern, House classification of upper extremity use, and subjective ratings of function and cosmetic appearance. In the functional goal group, children benefited in terms of reduction in muscle tone at elbow and wrist, and increase in passive wrist extension and House classification scores. A significant difference between the groups was observed in the House classification, favoring the low-dose group. In the nonfunctional goal group, a significant difference was detected in subjective parental cosmetic ratings, favoring the high dosage. Side effects were few and occurred mostly in the high-dose group. In conclusion, the use of higher doses in the spastic upper limb does not necessarily yield superior results compared with lower doses but increases the incidence of side effects.

Botulinum toxin treatment of spastic equinus in cerebral palsy: a randomized trial comparing two injection sites.

OBJECTIVE: To explore the clinical relevance of injection site by comparing two different injection techniques in children with cerebral palsy who have spastic equinus gait. DESIGN: A total of 19 children (13 boys, 6 girls; range, 1 yr 6 mos to 7 yrs; nine hemiplegics, eight diplegics, two quadriplegics; levels I to IV with the Gross Motor Function Classification System) participated in the study. The children were randomized into two groups: the proximal group received a botulinum toxin A injection into the proximal part of both heads of the gastrocnemius, and the distal group received a botulinum toxin A injection into the mid-belly of the muscle bulks. A single-point injection of BOTOX, 3 units/kg per site, was used. Assessments of active and passive range of motion, dynamic muscle length (modified Tardieu scale), calf tone (modified Ashworth scale), and video gait analysis (Observational Gait Scale) were performed before treatment and 3, 8, and 16 wks posttreatment. RESULTS: Active and passive dorsiflexion and calf tone in both groups and Observational Gait Scale total scores in the distal group improved at all time points. The median change from baseline values in Observational Gait Scale initial foot contact and total scores at 8 wks showed a significant difference favoring the distal group, but the clinical relevance remained tenuous. CONCLUSIONS: Using the methods described, no major changes in main outcome measures were associated with changing the injection site.