In late September 2000, a nine-year-old boy with no significant past medical history complained of pain over the upper third of his left arm, associated with a fever of 38.2°C. His pain was associated with numbness and progressed over the subsequent three days, radiating to the left wrist, left shoulder and lateral aspect of his neck. The pain was worse on palpation, with active movement and at night, causing insomnia. Four days after the onset of symptoms, the patient developed uncontrolled tremors of his left arm, increasing with voluntary movements and stress. The next day, the child presented for medical assessment and was hospitalized.
While his pain remained localized over the left arm, the tremor progressed to the right arm in the next 12 h. He also started complaining of a sore throat and difficulty swallowing cold liquids. The subsequent night was complicated by an intense pruritus over the scalp, neck and torso, with minimal response to diphenhydramine. On day 1 after admission, still conscious and oriented, he exhibited bilateral arm-beating tremors with choreiform and myoclonic movements of the fingers, toes and calves. Deep tendon reflexes were symmetrical, and strength and sensory examinations were normal. At that time, an electroencephalogram revealed a diffuse slowing of cerebral activity with rapid dysrythmia but no epileptic activity. An electromyogram of the left deltoid muscle and computed tomography of the head were normal. Later that day, the patient developed hydrophobia and aerophobia, and he became dysarthric. An evanescent macular rash was noted over the posterior aspect of the neck and back, associated with intense pruritis. He became agitated and experienced transient visual hallucinations. He had significant difficulty drinking but was still able to swallow his saliva.
The following day, the patient developed severe tremor and myoclonus of the face and all extremities, priapism, drooling, pharyngeal spasm, and a feeling of suffocation. He was intubated, heavily sedated and transferred to a tertiary care facility with a presumptive diagnosis of rabies.
Procedures to confirm diagnosis were undertaken, and human rabies immune globulin, rabies vaccine and acyclovir were administered immediately. During the following 24 h, extreme agitation and constant tremors required aggressive analgesia and sedation. On day 3, high fever, intense keratoconjunctivitis and swollen hyperemic lips were noted; these signs remained present for the next six days. A nonpurpuric maculopapular rash appeared over the face, neck and upper torso, and was confirmed by skin biopsy to be erythema multiforme. A possible right coronary aneurysm was identified by echocardiography, and the patient received intravenous immunoglobulin infusion for a Kawasaki-like syndrome. Over the ensuing two days, his neurological status progressively deteriorated. On day 5, he developed decerebrate posturing as well as a marked pharyngolaryngeal spasm. The following day, his pupils became dilated and unequal, and he presented transient hemodynamic instability. On day 7, his pupils became fixed and dilated, priapism disappeared and gasping movements were noted. Sedation was gradually decreased as the patient lost bulbar reflexes and progressed to a profound flaccid coma. Evoked potentials revealed abnormal conduction in the left suprabulbar tract. On day 9, 14 days after the onset of his initial symptoms, the patient presented a clinical picture compatible with brain death, was extubated and died.
