MR imaging of syringohydromyelia and Chiari malformations in myelomeningocele patients with scoliosis

The brain and spinal cord were examined with MR imaging in 30 myelomeningocele patients 3-32 years old to study the prevalence of syringohydromyelia and Chiari malformations and to correlate these conditions with developmental scoliosis and spontaneously arrested hydrocephalus. Twelve patients had neurologic deficits above the level of the myelomeningocele and 10 had spontaneously arrested hydrocephalus. MR visualized syringohydromyelia in four patients with widened or focally bulging spinal cords and in eight patients with atrophic spinal cords. All patients had Chiari malformations, 28 of type II and two of type I. Syringohydromyelia was not correlated with type of scoliosis, result of ventriculoperitoneal shunting procedures, radiologic level of the myelomeningocele, or extent of the Chiari malformation. The two patients with the most rapid progression of thoracic scoliosis had the most extensive syringohydromyelia and radiologically low lumbar levels. Neurologic deterioration due to syringohydromyelia and Chiari malformations is probably more common in myelomeningocele than has been recognized previously and may cause developmental scoliosis, loss of ambulation, impaired extremity function, and progressive cranial nerve paralysis.     

Upper body movement during walking in children with lumbo-sacral myelomeningocele

Eight children with lumbo-sacral myelomeningocele (MMC) underwent three-dimensional movement analysis to determine whether or not differing levels of lower extremity strength affected the extent of shoulder, trunk and pelvis movement during independent walking when wearing orthoses. Fourteen control children were also investigated. The patterns of upper body movements in all MMC children were well defined and consistent, showing small standard deviations from the mean. In the frontal and transverse planes, segment displacements of the MMC children assigned into Group II (hip extensor and abductor muscle strength grade 0-2) were almost twice that of the MMC children in Group I (hip extensor and abductor muscle strength grade 3-4). All segment displacements in the frontal, transverse and sagittal planes for Group I and Group II children were significantly greater than those for the controls. In the frontal plane these differences were approximately 4-10 times greater, with the Group II children having the largest peak-to-peak displacements. These results indicate that the motion amplitudes of the upper body segments are related to the degree of muscle weakness of the lower limbs. No significant differences were found when comparing segment motions during walking with either the Ferrari type knee-ankle-foot or ankle-foot orthoses.     

Influence of dual tasks on sitting postural sway in children and adolescents with myelomeningocele

Performing dual tasks, e.g. cognitive and motor tasks simultaneously, may be especially challenging to children with motor disorders. Changes in postural sway have been used to evaluate the effect of dual tasks. Increases in postural sway frequency and concurrent decrease in sway amplitude have been interpreted as tighter control of postural sway. The purpose of this study was to analyze postural sway under single and dual task conditions, while sitting. Thirteen children and adolescents with myelomeningocele and a matched control group were included in the study. The participants performed two single and two dual tasks each. The single task was a sitting still task. The dual tasks were one visual-spatial task and one executive task while simultaneously sitting still. Amplitude, velocity and frequency of center of pressure displacement of postural sway were analyzed between tasks and between groups. The results of the cognitive tasks were analyzed as well. The results revealed different patterns in the groups. During single tasks, the MMC group displayed significantly lower frequencies and velocities of center of pressure displacement compared to the control group. Adding the visual-spatial task influenced postural sway significantly in the control group, while adding the executive task influence the postural sway significantly in the MMC group. The myelomeningocele group confirmed our clinical experience by performing the cognitive tasks slowly but accurately. Further studies are needed to evaluate whether motor function, sensory function, structural anomalies in the brain or any other causes, separately or together, may explain the observed differences in the groups.     

Osteopoikilosis: A radiological and pathological study

Anatomico-pathological and radiological studies of osteopoikilosis were performed in two cases, one involving a femoral head, excised after a fracture of a femoral neck, in an elderly man and the other following biopsy of an iliac crest in a young woman. In both patients wide-spread radiological evidence of the disorder was present as an incidental finding. The radiological appearance of rounded and linear densities corresponded to old and inactive remodelling of spongy trabeculae in epiphyseal and metaphyseal locations. The distribution and appearance of these osteopoikilotic densities suggested them to have been related intimately to mechanical strain on spongy bone trabeculae. The diffuse nature of the lesions, their hereditary character, and their possible association with abnormalities of the skin suggest the existence of a particular terrain in which general metabolic conditions of connective tissue may interact with mechanical stresses in bone. Careful analysis of the findings in osteopoikilosis is desirable in order to provide data concerning the physiopathology of the skeleton and to permit more definitive interpretation of localised areas of bone condensation, including those observed not only in solitary bone islands and such conditions as osteopathia striata, but also those associated with infections and tumours.     

Influence of dual tasks on sitting postural sway in children and adolescents with myelomeningocele

Performing dual tasks, e.g. cognitive and motor tasks simultaneously, may be especially challenging to children with motor disorders. Changes in postural sway have been used to evaluate the effect of dual tasks. Increases in postural sway frequency and concurrent decrease in sway amplitude have been interpreted as tighter control of postural sway. The purpose of this study was to analyze postural sway under single and dual task conditions, while sitting. Thirteen children and adolescents with myelomeningocele and a matched control group were included in the study. The participants performed two single and two dual tasks each. The single task was a sitting still task. The dual tasks were one visual-spatial task and one executive task while simultaneously sitting still. Amplitude, velocity and frequency of center of pressure displacement of postural sway were analyzed between tasks and between groups. The results of the cognitive tasks were analyzed as well. The results revealed different patterns in the groups. During single tasks, the MMC group displayed significantly lower frequencies and velocities of center of pressure displacement compared to the control group. Adding the visual-spatial task influenced postural sway significantly in the control group, while adding the executive task influence the postural sway significantly in the MMC group. The myelomeningocele group confirmed our clinical experience by performing the cognitive tasks slowly but accurately. Further studies are needed to evaluate whether motor function, sensory function, structural anomalies in the brain or any other causes, separately or together, may explain the observed differences in the groups.     

Calvarial masses of infants and children. A radiological approach

Children frequently present with asymptomatic head lumps that have been discovered by their parents or by their hairdressers. Other children present with painful lumps or symptoms of intra-cranial masses with calvarial involvement. Imaging plays an important role in the diagnosis of such masses and in subsequent surgical planning. We present a review of the types of lesion that may present in these ways.     

Cerebellar arteriovenous malformations in children

We review the presentation, imaging findings and outcome in 18 children with cerebellar arteriovenous malformations (AVM). This group is of particular interest because of the reported poor outcome despite modern imaging and neurosurgical techniques. All children had CT and 15 underwent catheter angiography at presentation. Several of the children in the latter part of the study had MRI. Of the 18 children, 17 presented with a ruptured AVM producing intracranial haemorrhage. The remaining child presented with temporal lobe epilepsy and was shown to have temporal, vermian and cerebellar hemisphere AVM. This child had other stigmata of Osler-Weber-Rendu syndrome. Three other children had pre-existing abnormalities of possible relevance. One had a vascular malformation of the cheek and mandible, one a documented chromosomal abnormality and another a midline cleft upper lip and palate. Six of the 17 children with a ruptured cerebellar AVM died within 7 days of the ictus. Vascular pathology other than an AVM was found in 10 of the 14 children with a ruptured cerebellar AVM who had angiography: 4 intranidal aneurysms, 5 venous aneurysms and 2 cases of venous outflow obstruction (one child having both an aneurysm and obstruction). The severity of clinical presentation was directly related to the size of the acute haematoma, which was a reasonable predictor of outcome. Received: 14 October 1996 Accepted: 10 October 1997