Crying and agitation for no apparent reason in children with severe neurologic impairment is a common and frustrating problem for parents and providers alike.1 It is not unusual for families to make frequent trips to their primary care provider or to the emergency department (ED) for this problem. The patients referred to in this article are nonverbal, nonambulatory, and dependent on others for care. Such patients are unable to provide obvious clues as to what is making them uncomfortable, and their episodes of persistent crying or screaming cause distress to their family.
A methodical approach is helpful in dealing with this problem, yet sometimes the cause is not apparent, even after a thorough search. It is helpful to explain to parents and caretakers that the cause of the distress may take some time to figure out, and that diagnosis is often difficult. However, in most cases, a satisfactory treatment can be found.
Initial approach and differential diagnosis
The first step, as with any medical complaint, is to obtain a thorough history, followed by a physical examination looking for potential causes of pain. How long has the pain or irritability been present? Are there any triggers or precipitating events? Does anything make the crying better or worse? Was the onset associated with any changes at home, trauma, medication regimen, or other significant changes in care or treatment? Are there any signs of an acute illness or infection?
Consider the following list of problems while performing a history and physical exam, and deciding on laboratory tests. The differential diagnosis discussed below focuses on problems to which neurologically impaired children are particularly susceptible, and excludes acute, easily diagnosed conditions that might be found on the initial evaluation, such as an ear infection or another illness causing pain or distress. As a start, consider all the diagnoses listed in Table 1 and obtain the laboratory tests in Table 2. Of course, one can alter the laboratory workup according to the clinical situation.
The more common diagnoses discussed here are grouped by organ system, followed by less common but equally important conditions listed in an arbitrary order based on experience rather than by any published studies, as none exist.