X-linked microcephaly, microphthalmia, microcornea, congenital cataract, hypogenitalism, mental deficiency, growth retardation, spasticity: possible new syndrome.

We describe a male and his sister's son with microcephaly, microphthalmia, microcornea, congenital cataract, hypogenitalism, severe mental deficiency, progressive spasticity and growth retardation. Both affected males have brachycephaly, upslanting palpebral fissures, epicanthal folds, highly arched palate, small mouth, and retrognathia. Two maternal cousins of the propositus's mother may also have been affected.

Sensory disorders in cerebral palsy: two-point discrimination.

Two-point discrimination (TPD) was measured on eight points of the upper extremities of 220 children with cerebral palsy aged between seven and 14 years. 46 had classical diplegia, 23 had mildly spastic diplegia (without adductor spasms), 86 had hemiplegia, 26 had generalized dyskinesia, 10 had right- and four had left-sided hemiathetosis and 25 had quadriplegia. TPD was decreased in all cases compared with normal controls: slightly more for the classical forms of diplegia and on the paretic side of those with hemiplegia, slightly less in athetoid children.

[Neurologic findings in mild mental retardation].

The author subjected to neurological examination all children from a special school for retarded children (39 children, 22 boys, 17 girls). All children had a positive neurological finding. Central hypotonic syndrome was revealed most frequently--31 children; static tremor of the upper extremities--11 children and static tremor of the lower extremities--8 children.

[Determination of inhibition habituation in the alpha rhythm (eyes open)].

Luria used the term habituation for the phenomenon that the so-called orientation reflex to a certain stimulus is lost when the stimulus is repeated. In the present investigation habituation was tested by opening the eyes during conventional EEG. During the examination the patient was asked twelve times to open and close the eyes.

Disorders of memory of motor sequences in cerebral palsied children.

In 74 children (32 boys, 42 girls) between 7 and 14 years of age with different forms of cerebral palsy (mainly spastic ones) the motor memory was tested with a sequence of movements which had to be remembered at once, after 24 hours and after 5 days. The normal levels were determined by testing 21 healthy children matched as to age as control as (10 boys, 11 girls). Each exercise was evaluated separately as follows: the movement is performed correctly in the right sequence, 2 points; the movement is performed in a wrong sequence, but in the correct group of three exercises, 1 point; the sequence of movements is entirely wrong or the child does not perform the movements at all, 0 points.

[Central infantile hypotonia syndrome].

Czechoslovak child neurologists devoted much attention to central infantile hypotonic syndrome (CIHS) in a series of investigations conducted in 1959-1986. They found that it is a developmental syndrome caused by affection of the immature brain, and later, at the age of 3-5 years, it disappears or transforms into other syndromes: most frequently cerebellar syndromes and developmental disintegrations (disintegration of the development of the CNS and medium-grade mental retardation). These groups overlap only little.

Development of flexor spastic signs in preterm infants.

Fifty-five preterm infants with birthweights between 800 and 2000g and gestational ages between 25 and 33 weeks were repeatedly examined for the Rossolimo and Mendel-Bechterew lower-limb reflexes. Up to 34 weeks conceptional age (CA) an abduction-extension pattern was characteristic. Between 34 and 36 weeks CA the response changed to one implying flexor spasticity, which in some cases lasted throughout the first postnatal trimester.