Presence of delayed myelination and macrocephaly in the sister of a patient with vacuolating leukoencephalopathy with subcortical cysts.

Leukoencephalopathy with swelling and a discrepantly mild clinical course ("van der Knaap disease") is a recently identified syndrome. It is characterised by macrocephaly occurring during the first year of life, initially normal or nearly normal development, and slowly progressive ataxia and spasticity with initial preservation of intellectual functions. MRI shows diffuse abnormality in signal intensity, as well as swelling of the hemispheral white matter with subcortical cyst-like spaces in the fronto-parietal and anterior temporal areas.

[Leukoencephalopathy with swelling, megalencephalopathy and surprisingly mild course. A case report].

Leukoencephalopathy with swelling and a discrepantly mild clinical course is a recently identified syndrome described by van der Knaap et al. It is characterised by macrocephaly occurring during the first year of life, initially normal or nearly normal development, slowly progressive ataxia and spasticity with initial preservation of intellectual functions. MRI shows diffuse abnormality of signal intensity in the hemisphere white matter with subcortical cyst-like spaces in the fronto-parietal and anterior temporal areas.

Anxiety of the 8th month in the preterm child.

Background: Aim of this paper was to verify whether the condition described by Spitz as anxiety of the 8th months occurs earlier in the preterm than in the child born at term.

Methods: A prospective study was carried out on 27 subjects subdivided into three groups of 9, 8, 10 patients, respectively, according to gestational age (group I < or = 33 weeks; group II > or = 34 < or = 37 weeks; group III > or = 38 < or = 41 weeks). The cases were selected at random from the patients admitted to the Neuropsychomotor Unit of the Gaslini Institute of Genoa (Italy), from May 1, 1993 to May 22, 1998.

[Hypotonic cerebral palsy. Which treatment?].

Background: Aim of this study is to verify whether tonus neurophysiological and communicative-relational aspects in hypotonic subjects can be recovered by psychomotor therapy.

Methods: This case series includes two randomized groups of children with central hypotonia observed at the Department of Child Neuropsychiatry of the Gaslini Institute of Genova from February 1992 to December 1996. The 1st group includes 6 subjects treated in a local rehabilitation service only by kinesitherapy.

[Recovery of the "instrument-hand" and the "communication-hand" in the hemiparetic child].

Background: Rehabilitation techniques have always attached great importance to the recovery of hand functions in the different forms of child cerebral palsy. The human hand has complex structure and functions, and it can be defined as "instrument-hand", "expression-hand", "relation-hand" and "constant challenge to rehabilitation programmes. Among the most common techniques used today, neuromuscular facilitation allows the recovery only of the instrumental function, and cortical facilitation allows only the awareness of the motor learning.

[Aplasia and hypoplasia of the optic nerve. Comparison of 2 cases].

Hypoplasia and aplasia of the optic nerve are congenital anomalies characterized respectively by a marked volume reduction (very small papilla, often identifiable only as a rosy-yellowish area from which the retinal vessels emerge) and by the absence of the optic disk (absence of the nerve and mainly of its vessels) and of the visual functions. These anomalies are often associated with malformations of the central nervous system and of the ocular structures. The defects originate in the embryonal period due to the arrested development of the mesodermal component towards the head of the optic nerve.

[From movement to gesture. Evaluation in the treatment of neuro- psychomotor disability].

Aim of this study is to evaluate whether the subjects with neuromotor deficits can achieve both the restoration of the motor function and of the intentional and communicative components of gesture. Our case series includes 20 children (10 males, 10 females) aged 8 months to 14 years. Ten had neuromotor impairment (9 cases with different features of C.

[Interference of anticonvulsants in the rehabilitation treatment].

Aim of this study is to investigate whether the adverse neuropsychiatric effects of anticonvulsant drugs can affect the results of physical therapy. The influence of these drugs on neurological and/or psychological functions is considered an important factor in conditioning the active psycho-perceptuo-motor learning of child during physical therapy. Our case series included 30 children aged 4; 20 to 23 months.

[Psychomotor treatment of cranial injuries in childhood. Two cases of apallic syndrome].

In this paper the authors aim to assess the results of psychomotor treatment of 5 patients with cranial injuries admitted to the Division of Infantile Neuropsychiatry at Gaslini Hospital from 1983 to 1985 and to evaluate the role of this treatment in a pathology with multiple, complex functional disorders. Evolution was excellent in 3 out of 5 patients who presented level 1-3 coma with limited post-traumatic sequelae (epilepsy and dysfunction of the left side in A.D.

[Object permanence in children with neurological and psychomotor disorders].

The aim of this research was to investigate whether the development of object permanence is an available sign of the cognitive development in infants with psychomotor handicaps. Subjects consisted of 5 males, ranging in age from 12 to 33 months, with handicaps as a result of perinatal brain injury. Four were preterm infants.

[Cognitive and affective aspects of psychomotor assessment of myodystrophic children with the aim of specific treatment].

The aim of this study was to evaluate the incidence of cognitive and affective problems in the treatment of children suffering from myodystrophy. It was hypothesised that psychomotory assessment taking appropriate account of data regarding the evaluation of cognitive and affective development might allow an adequate and realistic therapeutic protocol to be defined. For this purpose the authors examined 55 myodystrophic patients aged between 6 and 10 years old during the period January 1980 and June 1993 using traditional psychomotory tests (Stanford-Binet and/or WISC in relation to the various age groups), projective tests (CAT, TAT) and graphic tests.

[The timing of the start of rehabilitative intervention with the newborn infant at neurological risk].

The few studies related to the effect of the timing of intervention of high-risk infants, ie. early vs late, have produced equivocal results. The aim of this study was to investigate whether "critical periods" for intervention exist.

[Psychomotor management in the rehabilitation of patients with myopathy in infancy. Case studies].

The salient features are described of the psychomotor treatment given to two sisters: SA aged 11 yrs 5 months and MGA aged 9 yrs 9 months both affected with aspecific congenital myopathy and under medical treatment since they were 3 months old. These two cases were selected as examples for the following reasons: (1) they may offer the opportunity to highlight what should be the focal objective of all treatment of myopathic children whatever the syndrome involved, namely to ensure that the children retain capacities required for normal social life as long as possible and to help them retain the essential physical capabilities required for everyday life; (2) because the slower course of this type of myopathy permits the gradual implementation of a diversified treatment protocol in the various social contexts (family, school, play groups) in which these patients are able to lead a life whose limitations are scarcely visible for a long time. Experience in the treatment of these two cases confirms that the role of Psychomotor Education in the treatment of myopathy cases is essentially that of teaching the child to be aware of and responsible for its own movements as the disease progresses, while showing it how touse all its available sensomotorial instruments to counteract its muscular inadequacies which progressively damage the child's body image.

[Rehabilitative treatment of severe sensory disorders. Description of 2 cases].

Unlabelled: The aim of the work has been to verify the efficacy of a psychomotor rehabilitative treatment undertaken at a late age in two children with multiple handicaps: sensorial (visual and hearing impairment), motor (apostural-akinetic stage) and relational (autistic behavior).

Methods: Salvatore C, and Ivan S., admitted to the "G.

[Effects of early rehabilitative treatment on neurological development and cognitive and perceptual-motor functions of preterm infants at risk].

Longterm effects of early rehabilitative treatment are evaluated in 37 premature infants at neurological risk. The late neurological and psychomotor development of this group of children (experimental group) is compared with that of another 35 at risk premature infants (control group), who received only conventional follow-up care. The two groups do not differ in birth-weight, gestational age, sex and neonatal disorders.

[Collateral transient behavioural changes during ACTH therapy in infantile generalized epilepsy (author's transl)].

ACTH therapy induces transient behaviour disturbances (irritability, restlessness or drowsiness and apathy), parallel to the EEG. changes. These behaviour abnormalities have a positive relationship with cortisolemic levels and clinical results.