Neurologic disorders in children are encountered commonly by pediatricians and family physicians. Although many neurologic illnesses that affect infants and children also affect adults (such as infection, epilepsy, inflammatory and demyelinating diseases, peripheral neuropathies, and myopathies), some, including developmental disorders, malformations, and many genetically determined conditions, are especially characteristic of the pediatric population.

The history is the most important component of the evaluation of a child with a neurologic problem. It shares the same principles as described for the adult history but also requires a complete review of the pregnancy, labor, and delivery (especially in cases of perinatal injury), congenital infection, and family history.

DEVELOPMENT AND MATURATION

One of the most important elements of the neurologic history is a developmental assessment of the child. The Denver Developmental Screening Test is an efficient and reliable method to assess achievement of developmental milestones. It evaluates four components of development: gross motor skills, fine motor adaptive skills, language, and personal–social interaction. Table 25-1 summarizes developmental milestones by age. This is based on averages and therefore can be used only with an understanding of the variability among children. Table 25-2 gives a brief description of primitive reflexes and their significance.

CEREBRAL PALSY

Cerebral palsy (CP) is a static (nonprogressive) disorder due to pre- or perinatal damage to cerebromotor pathways. It is the most common motor disability in childhood, affecting about 2.7 per 1,000 live births. Risk factors for CP include hypoxic–ischemic insult to the brain in the perinatal period, prematurity, low birth weight, chorioamnionitis, prenatal viral infections, and prenatal strokes.

Classification

The most commonly used classification of CP is based on the distribution of the affected motor dysfunction:

•Hemiparetic: Weakness and spasticity are seen on one side of the body. Signs include fisting on the affected side, early hand preference, and increased reflexes on the affected side.

•Diparetic: There is spasticity of all four limbs but affecting the legs much more than the arms. The children are often of normal intelligence and are less likely to have seizures than children with other forms of CP.

•Spastic quadriplegic: All four limbs are affected. Seizures often occur within the first 48 hours of life. The infant may show signs of cerebral hypotonia (see later discussion).

Clinical Manifestation

CP may be diagnosed as early as the first week of life: infants may have flaccid weakness, asymmetric limb movements, or seizures. In older children, spasticity, dystonia, and developmental delay are common presentations.

Diagnostic Evaluation

The diagnosis of CP is based on the clinical symptoms and signs. The time course of symptoms should be static, rather than progressive. Other entities that may present with dystonia, ataxia, or spasticity but that progress with time (e.g., metabolic disorders and leukodystrophies) must be excluded. Magnetic resonance imaging (MRI) is indicated to exclude the structural causes of the symptoms and signs, such as tumor, stroke, or vascular malformations.

Treatment

In general, a multidisciplinary approach is necessary, with early infant stimulation, physical and occupational therapy, orthopedic and psychologic evaluation, and speech therapy.

INTELLECTUAL DISABILITY AND DEVELOPMENTAL DELAY

Intellectual disability (ID) is the term used to describe impairment in the ability to achieve an expected level of cognitive function. The disorder can vary significantly in severity but manifests prior to adulthood. It can be classified by the results of standard intelligence tests such as the Stanford–Binet IQ and the Wechsler Preschool and Primary Scale of Intelligence—Revised. Normal IQ is 100 with a standard deviation of 15. ID is categorized as follows:

•Mild: IQ between 55 and 70

•Moderate: IQ between 40 and 55

•Severe: 25 to 40

•Profound: less than 25

For most children with ID, the cause is not known. There are many known causes of ID, including prenatal and postnatal trauma (e.g., intracerebral hemorrhage and hypoxic–anoxic encephalopathy); congenital and postnatal infection (e.g., congenital rubella, syphilis, cytomegalovirus, toxoplasmosis, and HIV infection); chromosomal abnormalities (e.g., Down syndrome, fragile X syndrome, Angelman syndrome, Prader–Willi syndrome); chromosomal translocations (e.g., cri du chat syndrome); inherited metabolic disorders (e.g., hypothyroidism, galactosemia, Tay–Sachs disease); and toxic, nutritional, and environmental causes. Table 25-3 summarizes some important chromosomal abnormalities associated with ID.

Developmental delay is the failure to acquire age-appropriate cognitive, language, fine or gross motor skills, or social skills. The Denver Developmental Assessment is a standard test that can help establish the diagnosis. Many of the etiologies of developmental delay are similar to those responsible for ID and include intrauterine toxins and infections, genetic abnormalities, neuronal migrational disorders, hypoxic–ischemic encephalopathy, and inborn errors of metabolism. Most often, though, no cause for developmental delay is found, in which case it is labeled “idiopathic” (or more appropriately, cryptogenic).

The treatment for both ID and developmental delay includes referral to early intervention programs for special education and training.

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The Sensory System The Approach to Coma and Altered Consciousness Head Trauma Neuro-Ophthalmology Demyelinating Diseases of the Central Nervous System Disorders of the Spinal Cord

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