A teenager arrives in ED with fatigue and headaches. He is diagnosed with primary CNS vasculitis.
THE CASE
You are the resident on call in the pediatric emergency department when you observe a father carrying in his teenage son. The teenager, who is a 16-year-old Caucasian, complains of a bilateral occipital headache that he has had for two weeks. He also complains of numbness and paresthesias in the right arm and right leg that occurred over the last three days, and an acute onset of weakness that started today in the right lower extremity.
The headaches are located focally in both the occipital and frontal regions bilaterally, and are intermittent. They have been partially alleviated with acetaminophen, ibuprofen, and sumatriptan. The teen describes the pain as a twelve on a scale of one to ten. He reports associated symptoms of nausea and vomiting. The headaches are not alleviated with vomiting, and they do not occur at any specific time of day. The headaches are not present upon waking in the morning, and do not wake him from sleep.
The parents relate that one week ago, he had a negative head computed tomography (CT) at an outlying ED. It was noted that he had an increased blood pressure of 160/110 mm Hg, and he was referred by the ED physician to a pediatric nephrologist who started him on hydralazine. His blood pressures normalized over the next few days, and the hydralazine was discontinued on a follow-up visit with his pediatrician. At that time a renal ultrasound was obtained, but was found to be within normal limits.
His parents report he is fatigued and has been sleeping for longer periods of time. He also complains of blurry vision in his peripheral fields bilaterally, and photophobia. He has had an eight- to ten-pound weight loss over the past two weeks. The parents deny trauma, loss of consciousness, fevers, seizures, or recent travel history.
His immunizations are current. His past medical history includes leg fractures from sports-related injuries in 2001 and 2002. In October 2005, his left eye was injured by a magnet dart.
You obtain a family history, which is significant for a 22-year-old brother who suffered from a "stroke." A paternal uncle died of a ruptured aneurysm. The patient's father suffers from migraines. There is no family history of autoimmune disease, multiple sclerosis, or seizures.
He is currently in eleventh grade, and has been growing and developing normally. He plays hockey. He admits to occasional marijuana use. Mom is concerned that his grades dropped last month.
Filling in the blanks
You obtain vitals, which are significant for a pulse in the 40s to 50s. However, an electrocardiogram (EKG) is normal, and he has normal blood pressure for height and age. On your exam you note that he is confused with possible aphasia. Generally, he is cooperative, but very tired. He has nonblanching erythema over both cheeks. He also appears to have photophobia. You check his fundi and see no papilledema.
He also has no oral lesions. On neck exam he has no lymphadenopathy and negative Kernig and Brudzinski signs. Auscultation of his heart reveals no murmur. His distal pulses are strong. Musculoskeletal exam reveals joints within full range of motion. None of the joints are swollen or tender. Examination of the chest and abdomen is unremarkable.
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