A 22-month-old girl with behavioral changes and hypertension, and a 16-year-old boy with generalized weakness, lower-extremity pain, and fasciculations.
Dr. Kish is a third-year resident in the department of pediatrics, Johns Hopkins Hospital, Baltimore, Md.
Dr. Myers is a staff pediatrician at the Waco regional clinic of Scott & White Memorial Hospital and Clinic, where she was a resident at Texas A&M University Health Science Center College of Medicine, Temple, Texas., where this article was written.
Saturday afternoon has been slow on the units of your children's hospital-until you get a call from a harried emergency physician who wants to admit a 22-month-old girl being transferred from a community hospital. She's been labeled a "diagnostic dilemma"-and, he regrets to say, it's been so busy in the emergency room that the staff hasn't even been able to review the chart from the other hospital. All he can tell you is that the child has a one-month history of behavioral changes and hypertension with, so far, a negative work-up. Her blood pressure in the ER is 131/83 mm Hg, equal in all four extremities, and she appears to be in no distress. She does cling tightly to her mother, however, he tells you, and refuses to look at anyone who enters the room.
"No problem," you reply, and await the toddler's arrival on the floor.
Five weeks ago, you learn, this adolescent was examined for intermittent headaches by his primary care physician, who started acetaminophen and aspirin, with little relief. After four weeks, the boy was hospitalized for dehydration and complaints of nausea, vomiting, and diminished appetite co-incident with the headaches. He also began to report mild shortness of breath, increasing weakness, and lower-extremity pain. A diagnosis of a viral illness was made, and he was rehydrated with intravenous fluids.
After two days of hospitalization, the boy's mother requested that he be transferred to another hospital for further evaluation because his symptoms persisted. There, pneumonia was diagnosed and he was started on ceftriaxone (Rocephin). Cerebrospinal fluid was obtained for analysis by lumbar puncture because of increasing irritability. Intermittent headaches and lower-extremity pain persisted, and onset of bilateral lower-extremity fasciculations was noted. After a brief stay, the adolescent has been moved again, with a transfer diagnosis of "possible encephalitis/encephalopathy." You promise to be back to see him shortly.
The toddler and her mother now arrive on the floor, and immediately you notice that the patient is sweating and extremely fractious. Throughout your interview with her mother, the child requires constant attention and stroking; when her mother tries to remove her arms from around her daughter or stops rubbing her back or hands, she screams. She keeps her face buried in her mother's chest and cries during the physical exam. She doesn't speak when asked direct questions and you don't observe her communicating with her mother.
The mother, who does not live with the patient's father, tells you that the girl's behavior began approximately one month ago and is fully uncharacteristic: She has always been pleasant and outgoing, spoke in sentences, and loved meeting new people. Over the past month, she became increasingly "needy and whiny" and even began to refuse to walk. She regressed to speaking in one- and two-word phrases only. In fact, the mother tells you that she began to feel as though her daughter "has been going backwards, becoming an infant again." It was at that point that she decided to seek help from the girl's pediatrician.
By the mother's report, the patient's BP was found to be significantly elevated when measured at the pediatrician's office. She reports that the physician acknowledged that he was concerned about the behavioral changes. He also noted a rash on the girl's trunk.
The girl was seen by the pediatrician several times over the next three weeks to follow her BP; ultimately, she was admitted to a community hospital, where a number of laboratory tests and imaging studies were performed-although her mother is unable to remember exactly what they were or what was found. She does note that the nursing staff and physicians at the other hospital turned their attention to the girl's night sweats, intermittent hypertension, and "rapid heartbeats."