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Case Reports Indian Pediatrics 2000;37: 1266-1268. |
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Kawasaki Disease with Palatal Palsy |
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Kawasaki disease was first described by Tomisaku Kawasaki in Japan in 1967(1,2). It is a form of acute multisystemic necrotizing vasculitis involving small and medium sized arteries. This is now said to be the commonest cause of acquired heart disease in children in developed countries(3-5), surpassing the incidence of rheumatic heart disease. In the developing countries it is now being recognized more frequently. The disease primarily affects children below 4 years of age(2,4,5). In India, Kawasaki disease (KD) has not been reported as frequently as it should be, only 25 cases have been reported so far(6-14). In our institution we have managed 5 cases of KD during the period from November ’98 to October ’99. Here we present one such case having coronary artery disease as well as neurological complication in the form of palatal palsy.
A 7-year-old boy belonging to low socio-economic group presented with the complaints of continuous fever for 8 days not responding to antimicrobials (ceftriaxone and amikacin), mild cough and rhinorrhea with bilateral conjunctival injection and pain over the left knee joint and also over the periumbilical and epigastric region. The patient was extremely irritable. On the third day of illness his parents noticed nasal intonation of voice and nasal regurgitation of fluids. Examination revealed a febrile, irritable child with bilateral nonpurulent conjunctivitis with cracked and fissured lips. Oropharynx appeared red and raw with strawberry tongue. There was significant lymphadenopathy of the posterior cervical groups along with splenohepato-megaly (spleen 6 cm below costal margin in (Lt) midclavicular line, firm inconsistency, liver 4 cm below costal margin in (Rt) mid- clavicular line, firm with sharp border, smooth surface, mildly tender and the upper border of liver dullness was within normal limits). Neurological examination revealed neck rigidity and positive Kernig’s sign. All cranial nerves were normal except the IX nerve which showed right sided palatal palsy with asymmetrical curtain like movement of the palate. Jerks, plantar, motor and sensory functions were normal. Subsequently, the patient also developed periungual des-quamation. Laboratory investigations showed Hb of 9.4 g/dl, PCV of 30% and the total leukocyte count was 11200/cumm (Polymorphs 60%, Lymphocytes 38%, and Eosinophils 2%). The reticulocyte count was 1.0%. Platelet count was 2,30,000/cumm and erythrocyte sedimentation rate was 26 mm in 1st hour (Wintrobe). No malarial parasites were found in the peripheral film and Widal test was also negative. Tests for antinuclear factor and rheumatoid factor were also negative. Urine examination showed albumin in traces, microscopic examination revealed 8 to 10 pus cells per high power field and occasional red blood cells. Culture of urine (at 48 and 72 hours) did not show any growth of organisms. Chest X-ray was non contributory. CSF examination revealed 5 leukocytes per cumm, with following biochemistry: sugar 78 mg/100 ml, protein 22 mg/100 ml and chloride 124 mEq/L. Echocardiographic report revealed coronary artery aneurysm involving left main coronary artery (2.8 mm) and right coronary artery (2.8 mm). Aneurysms of 2.8 mm at 7 years of age may be normal (JKDRC-criteria 1984, up to 3.5 mm diameter in children >5 years old). This case best fits as an atypical case of Kawasaki disease. But coronary arteritis which occur during this period evolves into coronary aneurysms which are first seen from 10-28 days after the onset of the illness(2-5). So a diameter of 2.8 mm at around 15 days of illness could be a sign of evolving aneurysms or dilatation.
Kawasaki disease is now being recognized more and more. Neurological complications of KD are well recognized in the form of hemiplegia, seizures, myositis(15), cranial nerve involvement, peripheral neuropathy, cerebral infarctions and aseptic meningitis(16). In one large series(17), neurological complications arose in 1.1% of cases. There have been 18 previous cases of facial nerve palsy in Kawasaki disease (15,18). Review of these cases(18) noted 6 of the 10 children, i.e., 60% in whom cerebrospinal fluid was sampled had pleocytosis. At the time of diagnosis coronary artery involvement was found in 55% compared to 20-40% of all cases of Kawasaki disease(19), but with treatment it occurs in <5% patients(2-5). Cases of unilateral palatal palsy have not been reported in the literature. This case showed complete recovery of the paralysis after 3 weeks from the onset of the disease. This child at present is doing well and is on low dose aspirin (5 mg/kg/day) and is under follow-up for his cardiac status. In this child, a 6 cm splenomegaly could not be explained by Kawasaki disease alone. Since the child lives in a malaria endemic zone, this splenomegaly could be due to previous malarial infections. Timely (particularly within first ten days) administration of immunoglobulin can reduce the occurrence of coronary aneurysms, but whether this can prevent neurological complications is not known. In this case, with aspirin administration (100 mg/kg/day) alone, the child showed marked clinical improvement. Immunoglobulins could not be given because of financial constrains. Contributors: AG was the overall co-ordinator and guide; he will act as a guarantor for this paper. SC guided the manuscript writing, PN compiled the data and drafted the manscript. AB helped in drafting and compiling data. Funding: None.
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