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Year : 2009  |  Volume : 4  |  Issue : 2  |  Page : 138-139

Kawasaki disease presenting atypically as meningoencephlitis

Department of Pediatrics, Indira Gandhi Medical College, Shimla, HP, India

Date of Web Publication29-Oct-2009

Correspondence Address:
Parveen Bhardwaj
HIG-8, Housing Board Colony, Jakhoo, Shimla - 171 002, HP
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1817-1745.57335

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How to cite this article:
Bhardwaj P, Kaushal RK, Gupta H. Kawasaki disease presenting atypically as meningoencephlitis. J Pediatr Neurosci 2009;4:138-9

How to cite this URL:
Bhardwaj P, Kaushal RK, Gupta H. Kawasaki disease presenting atypically as meningoencephlitis. J Pediatr Neurosci [serial online] 2009 [cited 2023 Dec 5];4:138-9. Available from: https://www.pediatricneurosciences.com/text.asp?2009/4/2/138/57335


Kawasaki disease (KD) is a generalized vasculitis of unknown etiology and a leading cause of acquired heart disease among children living in developed countries, but is still enigmatic in India. There is no laboratory test which can help the clinician in arriving at or confirming a diagnosis of KD. The difficulty is further compounded by the fact that the clinical features gradually evolve over a period of days to a few weeks, and the entire clinical spectrum is not seen at any one particular point of time and sometimes KD may present with atypical presentation. Moreover, it may be difficult to distinguish KD from other common febrile illnesses of childhood and is usually misdiagnosed as viral exanthem more so due to lack of awareness among physicians. Since untreated cases may have coronary abnormalities, including coronary aneurysms about one fourth of the cases and may be fatal if not treated. Hence we are reporting a case of Kawasaki disease presenting as meningoencephlitis.

A 9 month old male child presented with history of high grade fever (1040F) for seven days with an erythematous rash over the face and trunk for three days and abnormal movements and unconsciousness for one day. On examination child was comatose responding only to deep painful stimuli, erythematous rash was present over the face, neck and abdomen, Throat was hyperemic with tonsillar enlargement and had strawberry tongue, puffiness of hand and feet was noted. On systemic examination child was comatose responding only to deep painful stimuli, Neck rigidity was present, there were no other meningeal signs and rest of the CNS examination was normal. Spleen was just palpable. Respiratory and cardiovascular system examination was unremarkable except for tachycardia. No family history of seizures or febrile convulsion was present. Possibility of viral encephalitis was kept and child was managed symptomatically. Child started improving on symptomatic management and was conscious on second day of admission but was irritable and febrile and on fourth day of admission had massive peeling of skin all over the body. On Investigations ESR was 90 mm, CRP was increased, normocytic normochromic anemia, Polymorphonuclear leucocytosis with polymorphs showing toxic granulation and dohle bodies, platelet count initially were 380000/cmm and increased to 5,99000/cmm after 11 days of illness, CSF examination revealed lymphocytic pleocytosis with mildly elevated proteins and sugar was normal, ASO was negative, Throat culture, urine culture and blood culture were negative, CXR was normal .ECG showed tachycardia and non specific ST changes. Echocardiography was normal. After analyzing the clinical course of the disease and investigation revised diagnosis of atypical Kawasaki disease was kept and child was started on intravenous immunoglobulin infusion (2gm/kg) as single infusion and high dose aspirin (100mg/kg) for two weeks and then dose of aspirin was tapered to 5mg/kg/day for next 6 weeks. Child improved dramatically on starting the treatment. Repeat echo done after 2 weeks and 6 weeks were normal. Child is on follow up and is well.

After Henoch-Schonlein purpura, KD is the commonest vasculitic disorder of children [1],[2]. Albeit the number of cases reported from India remains minuscule and is largely confined to typical KD. It is likely that many cases of KD and more so of Incomplete Kawasaki Disease (IKD) go unnoticed, especially so in the absence of a precise diagnostic test. KD is a clinical diagnosis based on the characteristic history and physical findings. The diagnosis of classic Kawasaki disease (KD) requires fever of at least 5 days duration and the presence of 4 of the following. [1] Non exudative conjunctival infection [2] Oral involvement including strawberry tongue, mucosal hyperemia and cracked or erythematous lips [3] Changes in peripheral extremities, including edema or desquamation in convalescence [4] polymorphous rash and [5] acute cervical Lymphadenopathy > 1.5 cms in diameter. Marked irritability out of proportion to the degree of fever is usually present which responds dramatically to treatment. There is no laboratory test which can help the clinician in arriving at or confirming a diagnosis of KD . The difficulty is further compounded by the fact that the clinical features gradually evolve over a period of days to a few weeks, and the entire clinical spectrum is not seen at any one particular point of time.

However, "atypical" or "incomplete" cases of Kawasaki disease, in which patients have fewer than four of the five principal features and presence of atypical features like aseptic meningitis, hepatitis, arthritis which are not described in the classical presentation of KD are difficult to diagnose and untreated may lead to cardiac morbidity. The present case presented as meningoencephlities and was eventually diagnosed as Kawasaki disease only after ruling out other causes and presence of strawberry tongue, edema hand and feet, rash and desquamation of skin and presence of increased acute phase reactants and thrombocytosis. Neurological complications in Kawasaki disease are found in 1.1-3.7% of cases, It is considered that meningoencephlitis in KD may develop in cases having more severe and prolonged inflammatory changes; the clinical findings revealed a serious form of KD. This might be due to vasculitis of small arteries, arterioles, capillaries, and venules, which consists of infiltration of lymphocytes and large mononuclear cells, and edema [3],[4]. Diagnosing classical KD is difficult enough. Recognizing cases that do not fully meet the diagnostic criteria and present with atypical manifestation like meningoencephlitis offers an even greater challenge. Nevertheless it is important to be aware of this entity, as an unaware clinician may face a diagnostic dilemma. On one hand, there is just not enough clinical evidence for diagnosing KD. On the other hand, waiting endlessly could lead to irreversible complications such as coronary artery aneurysms and subsequent propensity to develop cardiac problems.

To help clinicians to arrive at the diagnosis, Japanese and North American groups have evolved diagnostic criteria [5],[6]. The Japanese workers deem presence of any four of the six clinical criteria sufficient for the diagnosis of IKD while the North American workers consider the presence of three criteria to be sufficient for the diagnosis of IKD provided echocardiography or angiography reveals coronary artery changes due to arteritis. As coronary lesions are often present by day 9 or 10 of illness, various workers have attempted to find out if any of the laboratory criteria could help in diagnosing atypical or incomplete forms of KD. Levy et al found that thrombocytosis was present consistently in atypical forms with peak thrombocytosis occurring at 13.5±5.9 days of illness and concluded that presence of thrombocytosis should be considered compatible with the diagnosis of IKD[7]. Albeit waiting for detecting thrombocytosis and coronary lesion to confirm the diagnosis of IKD can adversely affect the prognosis. For prevention of the complications, the physician has to start therapy much earlier. Hence, it is imperative that one diagnoses KD early and institutes treatment promptly. This is true of IKD too as this form has the tendency to develop all the complications of the classic KD [8],[9]. Unless a high index of suspicion is kept the patients with IKD will continue to escape our attention.

   References Top

1.Melish ME, Hicks RV. Kawasaki syndrome: clinical features, pathophysiology, etiology and therapy. J Rheumatol 1990;17:2-10.  Back to cited text no. 1      
2.Dhillon R, Newton L, Rudd PT, Hall SM. Management of Kawasaki disease in the British Isles. Arch Dis Child 1993; 69:631-38.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]  
3.Terasawa K, Ichinose E, Matsuishi T, Kato H. Neurological complications in Kawasaki disease. Brain Dev. 1983; 5(4):371-4.  Back to cited text no. 3      
4.Takagi K, Umezawa T, Saji T, Morooka K, Matsuo N. Meningoencephalitis in Kawasaki disease (Article in Japanese). No To Hattatsu. 1990 Sep; 22(5):429-35.  Back to cited text no. 4      
5.Japan Kawasaki Disease Research Committee. Diagnostic guidelines of Kawasaki disease, 4th Revision. Tokyo: Kawasaki Disease Research Committee; 1984.  Back to cited text no. 5      
6.Witt MT, Luann ML, Bohnsack JF, Young PC. Kawasaki disease: more patients are being diagnosed who do not meet American Heart Association criteria. Pediatrics 1999;104:e10.  Back to cited text no. 6      
7.Levy M, Koren G. Atypical Kawasaki disease: analysis of clinical presentation and diagnostic clues. Pediatr Infect Dis J 1990; 9:122-6.  Back to cited text no. 7  [PUBMED]  [FULLTEXT]  
8.McMaster P, Cooper S, Isaacs D. Is it Kawasaki disease? J Pediatr Child Health 2000;36:506-8.  Back to cited text no. 8      
9.Rowley AH, Goonzalez-Crussi F, Gidding SS, Duffy CE, Shulman ST. Incomplete Kawasaki disease with coronary artery involvement. J Pediatr 1987;110:409-13  Back to cited text no. 9      


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