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506 PART 3
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GROWTH AND DEVELOPMENT OF THE NERVOUS SYSTEM AND THE NEUROLOGY OF AGING
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ally, the legs are rmly exed, but in either instance relatively few movements are made When hypotonia is a forerunner of an extrapyramidal motor disorder (eg, so-called double athetosis, another type of cerebral palsy), the rst hint of abnormality may be an opisthotonic posturing of the head and neck However, involuntary choreic movements usually do not appear in the upper limbs before 5 to 6 months of age and often are so slight as to be overlooked They worsen as the infant matures and by 12 months assume a more athetotic character, often combined with tremor Tone in the affected limbs is by then increased but may be interrupted during passive manipulation When hypotonia is a prelude to a cerebellar motor defect, the ataxia becomes apparent when the infant makes the rst reaching movements Tremulous, irregular movements of the trunk and head are seen when the infant attempts to sit without support Still later, as the infant attempts to stand, there is unsteadiness of the entire body In distinction to the gross de cits in motor development described above, there is a relatively small but distinct group of young children who exhibit only mild abnormalities of muscle tone, clumsiness or unusual postures or rhythmic movements of the hands, tremor, and ataxia ( ne motor de cit ) Such awkwardness in the somewhat older child are referred to as soft signs and have been extensively described by Gubbay and colleagues in what they called the clumsy child Like speech delay and dyslexia, ne motor de cits of this sort are more frequent in males Tirosh found that intranatal problems were more prevalent among children with ne motor de cits (compared to those with gross motor de cits), as were minor physical anomalies and seizures Systemic diseases in infancy pose special problems in evaluation of the motor system The achievement of motor milestones is delayed by illnesses such as congenital heart disease (especially cyanotic forms), cystic brosis, renal and hepatic diseases, infections, and surgical procedures Under such conditions one does well to deal with the immediate illnesses and defer pronouncements about the status of cerebral function The brain proves to be simultaneously affected in 25 percent of patients with serious forms of congenital heart disease and an even higher proportion of patients with rubella and Coxsackie B viral infections In a disease such as cystic brosis, where the brain is not affected, it is advisable to depend more on the analysis of language development than on assessment of motor function, because muscular activity may be generally enfeebled
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funduscopy Faulty vision becomes increasingly apparent in older infants when the normal sequences of hand inspection and visuomanual coordination fail to emerge Retention of pupillary light re exes in a sightless child signi es a defect in the geniculocalcarine tracts or occipital lobes conditions that may be con rmed by MRI and testing of visual evoked responses With respect to hearing, again there is the dif culty in evaluating this function in an infant Normally, after a few weeks of life, alert parents notice a brisk startle to loud noises and a response to other sounds A tinkling bell brought from behind the infant usually results in hearkening or head turning and visual searching, but a lack of these responses warns only of the most severe hearing defects The detection of slight degrees of deafness, enough to interfere with auditory learning, requires special testing To make the problem even more dif cult, both a peripheral and a central disorder may be present in some conditions, such as kernicterus Brainstem auditory-evoked responses (BAERs) are particularly helpful in con rming peripheral (cochlear and eighth nerve) abnormalities in the infant and young child After the rst few months, impaired hearing becomes more obvious and interferes with language development, as described further on
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