Kawazaki disease and the cns
Kawasaki disease, or the mucocutaneous lymph node syndrome, is characterized by fever lasting longer than 5 days, polymorphous exanthem, reddening and swelling of the hands and feet, lymphadenopathy, conjunctival injection and changes in the lips and oropharynx which include reddening, fissuring and ulceration. Desquamation of the skin occurs in the second and third weeks of the illness (Kawasaki 1967). Although most cases recover spontaneously, coronary artery involvement occurs in 30-40% of cases, with the development of coronary artery aneur-ysms. Myocardial infarction, which may be fatal, occurs in 1-2% of cases. The disorder is associated with a variety of other symptoms, including arthritis, hepatitis, sterile pyuria, hydrops of the gall-bladder and CNS involvement. Aseptic meningitis is common, with mild increases in CSF lymphocytes, and occasionally with elevation of protein. Cerebral arthritis has been reported but is very rare. Irritability and lethargy are almost always present. Characteristic haematological findings include neutrophil leucocytosis, anaemia and thrombocytosis (Nihill et al 1987).
Epidemiological features suggest that Kawasaki disease is due to an infectious agent (Rawley & Shulman 1987). The disorder occurs in epidemics, and a wavelike spread of the epidemic has been documented in Japan. Treatment with intravenous immunoglobulin and aspirin appears to reduce the incidence of coronary aneurysms (Bierman & Gersony 1987).
- Kawasaki T 1967 Acute febrile mucotaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes. Japanese journal of Allergy 16: 178-222
- Nihill M R, Feigin R D, Gruber R et al 1987 Kawasaki disease. In: Feigin R D, Cherry J D (eds) Pediatric infectious diseases, 2nd edn. Saunders, Philadelphia, pp 2137-2165
- Rawley A H, Shulman S T 1987 The search for the etiology of Kawasaki disease. Pediatric Infectious Disease journal 6: 506-508
- Bierman F Z, Gersony W M 1987 Kawasaki disease, clinical perspective. Journal of Pediatrics II 1: 789-792