History

A detailed history is the cornerstone of any neurologic assessment. Although parents may be the primary informants, most children older than 3-4 yr are capable of contributing to their history and should be questioned directly.

The history should begin with the chief complaint, as well as a determination of the complaint’s relative significance within the context of normal development (Chapters 7–14). The latter step is critical because a 13 mo old who cannot walk may be perfectly normal, whereas a 4 yr old who cannot walk might have a serious pathology.

Next, the history of present illness should provide a chronological outline of the patient’s symptoms, with attention paid to location, quality, intensity, duration, associated features, and alleviating or exacerbating factors. It is essential to perform a review of systems, because abnormalities of the central nervous system (CNS) often manifest with vague, nonfocal symptoms that may be misattributed to other organ systems (e.g., vomiting, constipation, urinary incontinence). A detailed history might suggest that vomiting is due to increased intracranial pressure (ICP) rather than gastritis or that constipation and urinary incontinence are due to a spinal cord tumor rather than behavioral stool withholding.

Following the chief complaint and history of present illness, the physician should obtain a complete birth history, particularly if a congenital disorder is suspected. The birth history should begin with a review of the pregnancy, including specific questions about common complications, such as pregnancy-induced hypertension, preeclampsia, gestational diabetes, vaginal bleeding, infections, and falls. It is important to quantify any cigarette, alcohol, or drug (prescription, herbal, illicit) use. Inquiring about fetal movement might provide clues to an underlying diagnosis, because decreased or absent fetal activity can be associated with chromosomal anomalies and CNS or neuromuscular disorders. Finally, any abnormal ultrasound or amniocentesis results should be noted.

A labor history should address the gestational age at delivery and mode of delivery (spontaneous vaginal, vacuum- or forceps-assisted, cesarean section) and should comment on the presence or absence of fetal distress. If delivery was by cesarean section, it is essential to record the indication for surgery.

The birth weight, length, and head circumference provide useful information about the duration of a given problem, as well as insights into the uterine environment. Parents can usually provide a reliable history of their child’s postnatal course; however, if the patient was resuscitated or had a complicated hospital stay, it is often helpful to obtain the hospital records. The physician should inquire about the infant’s general well-being, feeding and sleeping patterns, activity level, and the nature of his or her cry. If the infant had jaundice, it is important to determine both the degree of jaundice and how it was managed. Historical markers of neurologic dysfunction include full-term infants who are unable to breathe spontaneously; have poor, uncoordinated sucks; need an inordinate amount of time to feed; or require gavage feeding. Again, it is important to consider the developmental context, because all of these issues would be expected in premature infants, particularly those with very low birth weights.

The most important component of a neurologic history is the developmental assessment (Chapters 6 and 14). Careful evaluation of a child’s social, cognitive, language, fine motor, and gross motor skills is required to distinguish normal development from either isolated or global (i.e., in two or more domains) developmental delay. An abnormality in development from birth suggests an intrauterine or perinatal cause, but a loss of skills (regression) over time strongly suggests an underlying degenerative disease of the CNS, such as an inborn error of metabolism. The ability of parents to recall the precise timing of their child’s developmental milestones is extremely variable. It is often helpful to request old photographs of the child or to review the baby book, where the milestones may have been dutifully recorded. In general, parents are aware when their child has a developmental problem, and the physician should show appropriate concern. Table 584-1 outlines the upper limits of normal for attaining specific developmental milestones. A comprehensive review of developmental screening tests and their interpretation is listed in Chapter 14.

Table 584-1 SCREENING SCHEME FOR DEVELOPMENTAL DELAY: UPPER RANGE

Family history is extremely important in the neurologic evaluation of a child. Most parents are extremely cooperative in securing medical information about family members, particularly if it might have relevance for their child. The history should document the age and history of neurologic disease, including developmental delay, epilepsy, migraine, stroke, and inherited disorders, for all first- and second-degree relatives. It is important to inquire directly about miscarriages or fetal deaths in utero and to document the sex of the embryo or fetus, as well as the gestational age at the time of demise. When available, the results of postmortem examinations should be obtained, as they can have a direct bearing on the patient’s condition. The parents should be questioned about their ethnic backgrounds, because some genetic disorders occur more commonly within specific populations (e.g., Tay-Sachs in the Ashkenazi Jewish population). They should also be asked if there is any chance that they could be related to each other, because the incidence of metabolic and degenerative disorders of the CNS is increased significantly in children of consanguineous marriages.

The social history should detail the child’s current living environment, as well as his or her relationship with other family members. It is important to inquire about recent stressors, such as divorce, remarriage, birth of a sibling, or death of a loved one, because they can affect the child’s behavior. If the child is in daycare or school, one should document his or her academic and social performance, paying particular attention to any abrupt changes. Academic performance can be assessed by asking about the child’s latest report card, and peer relationships can be evaluated by having the child name his or her “best friends.” Any child who is unable to name at least 2 or 3 playmates might have abnormal social development. In some cases, discussions with the daycare worker or teacher provide useful ancillary data.

Neurologic Examination

The neurologic examination begins at the outset of the interview. Indirect observation of the child’s appearance and movements can yield valuable information about the presence of an underlying disorder (Chapters 6 and 14). For instance, it may be obvious that the child has dysmorphic facies, an unusual posture, or an abnormality of motor function manifested by a hemiparesis or gait disturbance. The child’s behavior while playing and interacting with his or her parents may also be telling. A normal child usually plays independently early in the visit but will rapidly engage in the interview process. A child with attention-deficit/hyperactivity disorder might display impulsive behavior in the examining room, and a child with neurologic impairment might exhibit complete lack of awareness of the environment. Finally, note should be made of any unusual odors about the patient, because some metabolic disorders produce characteristic scents (e.g., the “musty” smell of phenylketonuria or the “sweaty feet” smell of isovaleric acidemia). If such an odor is present, it is important to determine whether it is persistent or transient, occurring only with illnesses.

The examination should be conducted in a nonthreatening, child-friendly setting. The child should be allowed to sit where he or she is most comfortable, whether it be on a parent’s lap or on the floor of the examination room. The physician should approach the child slowly, reserving any invasive or painful tests (e.g., measurement of head circumference, gag reflex) for the end of the examination. In the end, the more that the examination seems like a game, the better the child will cooperate. Because the neurologic examination of an infant requires a somewhat modified approach from that of an older child, the two groups are considered separately (Chapters 7, 8, and 88).