The most important parts of the evaluation of a child with neurologic symptoms are the history and physical examination. Principles used during the general evaluation are applicable to the child with a neurologic problem. Slight modification of the approach can increase the amount of information obtained. The purpose of taking the history is to elicit information that enables a tentative diagnosis to be made. With a tentative diagnosis in mind, the physician performs the neurologic examination to see whether the findings are consistent with the postulated diagnosis. For example, if a patient is thought to have idiopathic epilepsy but has an abnormal neurologic examination, the possibility that a structural lesion is causing the seizures should be considered. In the case of a child with a complaint of weakness, cerebellar dysfunction should be considered, as well as disease of nerve or muscle, because unsteadiness may be interpreted as weakness by the family. Tests of coordination, power of individual muscle groups, reflexes, and sensation can help to differentiate the cause of the symptom.

Information obtained from the history should allow the tempo of the illness to be assessed and the findings to be interpreted in accord with neuroanatomic and neurophysiologic principles. The physician should determine whether the disease process is acute or chronic and whether the onset was abrupt or insidious. Occasionally, an event such as intercurrent illness or trauma results in closer than usual observation of a child and discovery of a preexisting problem; a history dating the beginning of neurologic symptoms may not always be accurate. A decrease in the rate of acquisition of new developmental skills or loss of previously acquired skills suggests a degenerative process. Specific questions should be asked to help clarify the meaning of terms used by the historian. Dizziness may indicate vertigo or light-headedness. Weakness may refer to loss of muscle power, fatigue, or
unsteadiness. Blurred vision may indicate diplopia, decreased acuity, a visual field defect, or scotomata. Each of these conditions has different importance in terms of localizing the area of dysfunction. The physician must decide whether the symptoms can be explained by dysfunction of one part of the nervous system or whether the process is diffuse or multifocal. Different physical findings correlate with each of these possibilities.

Information obtained during history taking should include details of the mother’s pregnancy, labor, and delivery. Occasionally, insults may affect the nervous system of the fetus or neonate and can produce immediate or subsequent neurologic problems. Particular attention given to the schedule of acquisition of motor and language developmental milestones yields information about when the disorder began and whether the problem involves specific areas of function. A summary of normal developmental milestones is given in Box 394.1.

Many neurologic illnesses are familial, and important clues to the child’s illness may be obtained from careful review of the family history. Family members may not know the specific name of the disease being considered but may be familiar with symptoms and signs, particularly in cases of familial diseases involving the cerebellum, peripheral nerves, or muscle. Subtle manifestations of neurocutaneous syndromes may not have been appreciated previously, and the finding of tuberous sclerosis or neurofibromatosis in the child can result in identification of other affected relatives. Occasionally, diseases are diagnosed erroneously by family members. Any severe headache may be referred to mistakenly as migraine. Eliciting a detailed history in family members to see whether the symptoms do suggest migraine is important.

The physical examination begins when the child enters the examining room; observations continue during the history taking. Significant information about cranial nerves and cerebellar and motor function can be obtained by watching an infant crawl, walk, or play with toys. The general physical examination should include measurement of head size. The presence of dysmorphic features or cutaneous abnormalities may help to establish a diagnosis. Performing a Wood light examination may be necessary to detect the depigmented ash-leaf lesions seen in tuberous sclerosis. Metabolic disorders resulting in the accumulation of excessive amounts of lipids or other materials may result in enlargement of the liver and spleen. Cardiac murmurs may signify abnormalities that predispose to neurologic complications such as embolic strokes and cerebral abscesses. A cranial bruit may suggest a large intracranial vascular malformation. Cutaneous abnormalities, dimples, vascular malformations, and tufts of hair over the lower back may be associated with occult spinal dysraphism. Limb growth asymmetry suggests chronic hemiparesis.

The formal neurologic examination is used to confirm the information obtained by observation and to corroborate the diagnosis suspected from the history. Examination often is easier to perform with the infant seated in the mother’s lap rather than with the infant seated or lying on an examining table. Beginning the examination with the child’s legs and working upward results in better cooperation than if more unpleasant aspects of the examination, such as funduscopy, are performed first.

Cranial nerve testing is easy to perform. Cranial nerve I, the olfactory nerve, often is not tested unless there is a specific indication for doing so. Cranial nerve II, the optic nerve, can be inspected and tested. Vision can be assessed by several methods in children who are too young to cooperate in formal testing with visual acuity charts. Various revolving drums or tapes can be used to elicit optokinetic nystagmus in young infants. With infants older than 6 months of age, various small objects, even a fleck of paper, can be placed in front of them, and their attempts to pick it up can be observed. Each eye can be tested separately. Cranial nerves III, IV, and VI are responsible for eye movements, pupillary responses, and lid opening. They can be tested by observing spontaneous eye movements and by having the child watch toys such as puppets. Facial sensation is supplied by cranial nerve V and can be tested with a wisp of cotton. The motor branch of cranial nerve V supplies the muscles of mastication. These muscles can be tested by having the child open the jaw against resistance and by palpating the masseter muscles as the teeth are clenched. Facial movements, a function of cranial nerve VII, can be assessed when the child smiles and laughs and with volitional movements. Asking the child to smile allows observation of the symmetry of the nasolabial folds; the symmetry of burying the eyelashes can be observed as the eyes are closed. The symmetry of the strength of the eyelid closure can be tested by attempting to open the upper lids while the child tries to keep the eyes tightly closed. Auditory acuity, controlled by cranial nerve VIII, can be tested by using a tuning fork or watch or by giving whispered instructions out of sight of the child. Lower cranial nerves IX, X, XI, and XII can be tested by eliciting a gag reflex, watching the palate contract, and observing movements of the tongue, shoulders, and neck.

In preschool-aged children, motor function is assessed by watching the child play, crawl, climb, or walk. Activities can be designed to test functions of upper or lower extremities. Lifting a child with the examiner’s hands placed in the patient’s axillae tests shoulder girdle function. Lifting a child off the ground while the child holds the examiner’s thumbs tests hand strength. Tone is tested by passively moving the child’s limbs.

Deep tendon reflexes are elicited in children by techniques similar to those used with adults. Infants have developmental reflexes in the newborn period that disappear with normal development. The more common developmental reflexes are listed in Table 394.1. The abnormal persistence of these reflexes usually is accompanied by other abnormalities in the neurologic examination or a lack of appropriate developmental skills. The isolated persistence of one of these reflexes should be interpreted cautiously because of the significant variation in the age at which they normally disappear.

Cerebellar function can be assessed in young infants and preschool children by watching them play; this method is particularly useful for observing upper extremity function as well as balance. The skill with which young children perform fine motor tasks is age-dependent, and the assessment of normal function must take this fact into consideration. Infants often can be coaxed into reaching for small objects, and during these maneuvers, fine motor coordination and hand function are observed. The presence of adventitial or associated movements can be evaluated at this time. Watching a child walk or run is helpful in determining cerebellar function and motor strength, peripheral nerve function, and abnormalities of tone.

The sensory examination often is difficult to perform and interpret accurately in infants and preschool children. When it is performed near the end of a history-taking and examination session, attention and cooperation may be lacking. Under these circumstances, completing the sensory examination at a later time may yield more useful information. Engaging the child in “play games” often enhances effort and interest. Testing of cortical or sensory modalities, such as double simultaneous stimulation, stereognosis, or graphesthesia, requires accurate reporting by the examinee. Reliable information often is difficult to obtain until the child is of school age.

The laboratory tests frequently used in the evaluation of children with neurologic problems are discussed briefly. A few indications are given for each test, but the discussion is neither complete nor comprehensive. Readers can refer to other sections of this book that discuss the use of laboratory tests in the context of specific diseases or problems.