The clinical utility of MRI in patients with neurodevelopmental disorders of unknown origin.

INTRODUCTION: Neuroimaging of the brain in the diagnostic work-up of patients with neurodevelopmental disorders is a matter of continuing debate. Recommendations range from performing brain imaging in all patients with neurodevelopmental disorders to performing an MRI only in those with indication on clinical examinations. Important indications for neuroimaging are head size abnormalities and focal neurological findings. METHODS: Patients with neurodevelopmental disorders of unknown origin (n = 410), referred to a specialized tertiary diagnostic center for neurodevelopmental disorders were included in a retrospective analysis. A 1-day work-up, including an MRI of the brain was performed. Studied were the: (i) yield of MRI scans of the brain and (ii) associations of specific clinical symptoms/signs with abnormal and diagnostic MRI scans. RESULTS: (i) In 30.7% of the 410 patients with neurodevelopmental disorders (n = 126), abnormal MRI scans were observed, leading to an etiological diagnosis in 5.4% of the patients (n = 22). (ii) Pyramidal disorders (P = 0.001), epilepsy (P = 0.04) and an abnormal head circumference (P = 0.02) were associated with an abnormal MRI scan. The presence of one of the following neurological symptoms/signs: movement disorders, pyramidal disorders, epilepsy, or an abnormal head circumference was associated with a diagnostic MRI scan (P < 0.001) (diagnostic MRI % in neurological versus no neurological symptoms/signs, 13.0% versus 1.9%). CONCLUSION: Neuroimaging of the brain in a tertiary care center for patients with neurodevelopmental disorders of unknown origin is useful, especially in case of neurological symptoms/signs.

Epilepsy surgery does not harm motor performance of children and adolescents.

The impact of epilepsy surgery on motor performance, activities of daily life (ADL) and caregiver assistance was assessed in 37 children (age range 0.1-15.4 years) with pharmacologically untreatable epilepsy, 17 of whom were also diagnosed as having spasticity of cerebral origin. All patients underwent epilepsy surgery between 1996 and 2001 at the Wilhelmina University Children's Hospital and were assessed using a standard protocol at fixed intervals: before surgery and 6 months, 1 year and 2 years after surgery. The type of surgery was hemispherectomy (n = 14) and temporal (n = 14), frontal (n = 4), parietal (n = 2) and central (n = 2) resection. One child underwent callosotomy. Engel's classification was used to determine seizure outcome. Impairments were measured in terms of muscle strength, range of motion and muscle tone. Motor performance of infants and children without spasticity was measured using the Movement Assessment Battery for Children (M-ABC). The Gross Motor Function Measure (GMFM-88) was used in children with spasticity, the severity of motor disability in this group being determined by means of the Gross Motor Function Classification System (GMFCS). Daily activities and caregiver's assistance were measured in all children using the Pediatric Evaluation of Disability Inventory (PEDI). Twenty-four months after surgery 74% of the children could be classified as Engel class 1, indicating a significant seizure reduction. Impairments revealed some decrease in muscle strength and range of motion in the group with spasticity. Scores improved statistically significantly at group level on M-ABC and GMFM (P < 0.05). Improvement in activities of daily life and caregiver's assistance could not be measured in children without spasticity because of the ceiling effect of the PEDI, but children with spasticity improved significantly with respect to these parameters (PEDI) (P < 0.05). Hence, epilepsy surgery does not harm motor performance in children with or without spasticity.

Functional consequences of hemispherectomy.

Using the International Classification of Functioning Disability and Health (ICF) (WHO, 2001), impairments, activities and social participation are reported in 12 children (mean age at surgery 5.9 years) who were investigated before and three times over a 2-year period after hemispherectomy. Impairments were assessed (i) in terms of seizure frequency (Engel classification) and seizure severity (HASS) and (ii) with respect to muscle strength (MRC), range of motion (JAM score) and muscle tone (modified Ashworth scale). Activities were assessed in terms of gross motor functioning (GMFM) and self-care, mobility and social function (PEDI). Participation was assessed in terms of epilepsy-related restrictions and quantified by means of the Hague Restrictions in Childhood Epilepsy Scale (HARCES). Nine out of 12 children could be classified as free of seizures (Engel class I), and in the remaining three seizure frequency was Engel class III. HASS scores showed maximum improvement in 10 out of 12 children and near-maximum improvement in the two remaining children. Muscle strength and muscle tone on the side of the body contralateral to the hemispherectomy, which were already decreased preoperatively, decreased even further in the first 6 months after surgery, but returned to the presurgical baseline thereafter, except for the distal part of the arm. Range of motion was abnormal prior to operation and remained so after operation. Mean GMFM increase was 20% after 2 years (95% confidence interval 10-33); all five dimensions improved statistically significantly (P < 0.05). Mean PEDI increase was more than 20 scale points (95% confidence interval 10-35); again, all domains improved significantly (P < 0.05). In nearly all children, HARCES scores had normalized 2 years after surgery. In conclusion, decrease of seizure frequency and severity widens the scope of motor and social functioning, which overrides the effects of remaining motor impairments.

Influence of infant-walkers on motor development: mimicking spastic diplegia?

We discuss two patients, who used an infant walker during the period in which they learned to walk. The influence on qualitative and quantitative motor development is illustrated in this report. A disharmonic and delayed motor development, contractures of the calf-muscles and motor development mimicking spastic diplegia are considered to be caused by the early use of infant walkers. As illustrated by the literature, infant walkers do not have any positive effect on improving motor development. Vulnerability of infants with regards to accidents is increased. In our opinion, the use of infant-walkers should be discouraged.

Osteogenesis imperfecta in childhood: impairment and disability.

OBJECTIVE: To determine clinical characteristics in children with osteogenesis imperfecta (OI) regarding impairment (range of joint motion and muscle strength) and disability (functional skills) in relation to the different types of the disease, and to study the correlation between characteristics of impairment and disability. METHODS: In a cross-sectional study 54 children with OI (OI type I: 24; OI type III: 15; OI type IV: 15), the range of joint motion, muscle strength, and functional ability were measured in a standardized way and analyzed statistically. RESULTS: The range of joint motion in almost all joints differed significantly with respect to the different disease types. In OI type I patients, generalized hypermobility of the joints was present, without decrease in joint motion. In OI type III the extremities were severely maligned, especially the lower limbs. In type IV the upper and lower extremities were equally maligned. Muscle strength differed significantly with respect to the different types of OI. In type I patients, muscle strength was normal except for the periarticular hip muscles. In type III, especially in the lower extremities, muscle strength was severely decreased, with a muscular imbalance around the hip joint. In type IV, muscle strength was mainly decreased in the proximal muscles of the upper and lower extremities. In children .6). CONCLUSION: In OI, severity-related profiles exist, within the different subtypes of the disease, regarding range of joint motion, muscle strength, and functional skills. In younger children, impairment parameters do not sufficiently correlate for disability. Rehabilitation strategies in younger children should therefore focus on improvement of functional skills and not only on impairment parameters.

[Sensorimotor training in children with motor retardation]. / Sensomotorische training bij kinderen met een motorische achterstand.

Sensory motor training is often prescribed for children with a delayed motor development. This training is preceded by a determination of their motory level by a test. The Oseretsky test is the one we mostly use for this purpose. If the test is performed before and after the training, the effect of the training can be evaluated. In theory, sensomotory development is often looked upon as a foundation for further development, especially for the cognitive development. The effect of this training is not the same for all children; 54% of the children, who have been trained by us, scored a motory profit on ony year or more.

[Plasmapheresis in 2 children with Guillain-Barré syndrome]. / Plasmaferese bij twee kinderen met het Guillain-Barré syndroom.

The clinical course in two young patients, suffering from acute progressive idiopathic polyradiculoneuropathy, the Guillain Barré Syndrome, is described. In view of the progressive course, plasmapheresis was applied. After plasmapheresis we noted a remarkable clinical recovery. Those observations are compatible with several case reports in the literature. In our view plasmapheresis deserves also in children serious consideration in the treatment of rapidly progressive cases of GBS.