Eye-movement disorders and visual-perceptual impairment in diplegic children born preterm: a clinical evaluation.

The visual-motor behaviour of 15 preterm diplegic children and 50 control children (age range 4 to 7 years) was recorded on video as they performed a visual-perceptual task (an adaptation of the Animal House subtest of the Wechsler Preschool Primary Scale of Intelligence). The following parameters were analysed and scored: time to perform task; omissions; figure-colour association; sequence direction; sequential scanning order; accuracy of fitting target; and number of anticipatory saccadic movements to next target. The ability of the control children to perform the task improved significantly with age, as measured by performance time, mistakes in sequence direction and scanning order, accuracy of target fitting, and number of anticipatory saccadic movements. The scores of children with diplegia were not related to age and were poorer overall than those of the control group. Children with diplegia made significantly more mistakes of sequence direction and scanning order, and significantly fewer anticipatory saccadic movements than the control group. These results indicate that visual-perceptual impairment in diplegic children born preterm is not attributable only to sensory visual loss and to fine manipulation difficulties but is also related to difficulties in eye movements and in using anticipatory control to process information.

MRI features of cerebral lesions and cognitive functions in preterm spastic diplegic children.

The aim of this study is to further clarify the relation between the pattern of cognitive impairment in spastic diplegic children born preterm and MRI features of cerebral lesions. The cognitive profile by Wechsler Scale of a sample of 30 children aged 6 years, 8 months to 14 years, 7 months was assessed, and the correlations between the Full Scale, Verbal, and Performance IQ and periventricular leukomalacia features on MRI were investigated. A significant difference was observed between the mean Verbal and Performance IQ, indicating a specific failure in the visuoperceptual functions of spastic diplegic children born preterm. Periventricular leukomalacia was detected in all children. The severity of ventricular dilatation, the degree and extent of white matter reduction, optic radiation involvement, and the thinning of the posterior corpus callosum correlated significantly with the Full Scale and Performance IQ: no correlation was observed between the Verbal IQ and any of the MRI features analyzed. In spastic diplegic children, an MRI examination between the ages of 1 and 2 years may be helpful in predicting a specific neuropsychological pattern of dysfunction and in defining an early intervention program.

Prospective study of gross motor development in children with SMA type II.

The natural history of SMA and the identification of predictive criteria of functional development are still a matter of discussion. This prospective study involved 20 children with SMA, aged between 3.10 and 15.7 years. The patients were followed from 1979 to 1992 in order to try to develop a greater understanding of the natural history of SMA at a very early age. A standardized protocol was used at regular intervals to assess parameters such as joint contractures, scoliosis and the milestones of gross motor functions. Our findings agree with the data in the literature concerning the early and generalized onset of joint contractures and scoliosis. A significant correlation was found between the level of acquired gross motor functions and walking with support. The acquisition of rolling by 5 years of age was the milestone that best correlated with the acquisition of walking with crutches and braces; furthermore the inability to roll seemed to correlate with the severity of the disease. These findings should be useful in planning a more rational rehabilitation program.

The cognitive development of children born preterm and affected by spastic diplegia.

In a prospective study the intellectual development of 20 premature children affected by spastic diplegia was compared with that of 10 preterm low-risk children. The assessment was carried out with the Griffiths scale at the age of 3 years and with the WPPSI scale at the age of 6 years. The analysis of data collected in the 2 psychometric evaluations of the preterm-born diplegic children showed a disharmonic profile of neuropsychological functions, already present at the age of 3 years and confirmed at the age of 6 years. The average scores in diplegic children were poorest in the subscales locomotor, eye-hand coordination, and performance on Griffiths scale, and in the performance subtests of the WPPSI scale. The mean scores of subscales for hearing and speech, and practical reasoning on the Griffiths scale and of the verbal subscale of the WPPSI were near to the lower range of the normal distribution. Significant differences in performance subtests were found between the groups of preterm diplegic children and the group of low-risk preterm children, both at 3 and 6 years of age.

Clinical features and MR imaging in children with repaired myelomeningocele.

The aim of the study is to define the role of associated malformations in the clinical evolution of children affected by myelomeningocele. MRI investigation of the spinal cord was carried out on 25 patients between the age of 7.3 and 18.10 with MMC repaired and followed up for at least 7 years. The relation between associated malformations demonstrated by MRI and clinical trend was analysed. The results are the following: 1) presence of asymptomatic tethered cord in all cases; 2) high frequency (92%) of Chiari malformation; 3) presence of syringomyelia in 20% of patients, symptomatic in 1; 4) presence of ventricular enlargement in 72% of cases without increased intracranial pressure syndrome. For a better therapeutic approach prospective MRI studies are needed in order to follow up associated malformations.

Neurogenic arthrogryposis multiplex congenita: clinical and MRI findings.

A clinical and magnetic resonance imaging (MRI) study on a selected group of 11 children, with a diagnosis of neurogenic arthrogryposis multiplex congenita (AMC) based on clinical, electromyographic, and muscle biopsy findings, is presented to determine the extent of central nervous system involvement in AMC. Family history, pregnancy, perinatal problems, other abnormalities, and epileptic seizures were reviewed. Neurologic examination, electroencephalography, intellectual assessment, and MRI study both of spinal cord and brain were performed. The clinical and laboratory findings disclosed evidence of spinal cord lesions with involvement of anterior horn cell function in all patients, and impairment of cerebral function in 5 patients. MRI revealed spinal cord atrophy in 3 patients, diffuse atrophy in 2 patients, and involved thoraco-lumbar segments in 1 patient. Cranial MRI studies demonstrated features of developmental brain abnormalities in 3 patients, cortical frontal atrophy in 2, and was normal in 4. In neurogenic AMC patients, MRI examination of the spinal cord and brain may help to clarify the pathogenesis of the disease and is helpful for prognostic and therapeutic purposes.

Nosology of spastic tetraplegic cerebral palsy: clinical review of fifty cases.

The aim of this study is to contribute to the definition of tetraplegic cerebral palsy (TCP) and to verify the classification criteria currently used by reviewing the clinical and neurological aspects of 50 children having non-progressive encephalopathy with neurological involvement of 4 limbs (symmetric 4-limb type, side-asymmetric type, upper-limb dominated type with or without dystonic traits). All severe diplegic patients, i.e. less upper than lower limb involvement and patients with dystonic hyperkinetic syndrome without spastic features were excluded. The data were supplied by the hospital records, evolution of motor performance and the presence or absence of epilepsy, visual problems, language disorders, intellectual impairment. All the children underwent: neurological examination, functional assessment, cognitive evaluation. Severe motor impairment was found in 80% of the patients, whose clinical characteristics led to a diagnosis of severe TCP or "true TCP" according to Hagberg, whereas 20% of them showed mild to moderate impairment with a more favorable prognosis. This group of patients complied with the classification of Michaelis who defined the quadrispastic CP types more broadly and delineated five subgroups also including cases with milder involvement (side-dominated, three-limb dominated tetraparesis). The intellectual assessment showed that, in the severely affected patients, only a small percentage had severe intellectual impairment (IQ less than 50, 37.5%), which contrasts with published data on this pathology, while the majority of the subjects showed moderate (25%) or mild intellectual impairment (32.5%) or normal cognitive function (5%).(ABSTRACT TRUNCATED AT 250 WORDS)