Kawasaki Disease

Kawasaki disease, also known as acute infantile febrile mucocutaneous lymph-node syndrome, is a syndrome of unknown etiology affecting children. Children typically present with fever, irritability, strawberry tongue, conjunctival congestion, cervical lymphadeno-pathy, and desquamating rash on the palms and soles. In the United States, approximately 3000 cases are diagnosed annually. Neurologic manifestations of the syndrome include aseptic meningitis, irritability, meningeal signs, seizures, and encephalopathy (113).

Facial nerve paralysis was first noted in 1974 by Murayama, with 28 cases documented by the year 2000 (114). Facial palsy is usually transient, ranging from two days to three months. Complete recovery was the rule for all infants who survived the syndrome. Facial nerve palsy is likely due to a vasculitis involving the facial nerve. Vasculitis typically involves the coronary arteries in Kawasaki's syndrome and is a significant determinant of morbidity and mortality occurring in 25% of untreated cases. When facial nerve involvement is present, the incidence of coronary artery aneurysms is increased to 54%. Aggressive treatment for the systemic syndrome is warranted to prevent coronary aneurysms. Intravenous immunoglobulin and aspirin therapy are the current treatments employed (115).

Kawasaki disease is discussed in further detail in Chapter 9.

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