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.