Infantile Kawasaki disease presenting as acute meningoencephalitis.

Abstract

Neurological manifestations are seen in only 0.4% of children with Kawasaki disease (KD), manifesting commonly as irritability, and less frequently as severe headache, alteration in consciousness, seizures, isolated or multiple cranial nerve palsies and stroke. We describe a 10 months old infant who presented with fever, generalized rash and acute onset encephalopathy, and subsequently diagnosed as KD. Although uncommon, KD should be considered in the differential diagnosis of children with acute febrile encephalopathy. A 10-month-old girl presented with high-grade fever for 4 days. She had redness of lips and eyes, maculopapular rash involving trunk and proximal parts of extremities on day 2 of illness which improved over 24 h. She developed irritability and deterioration in responsiveness for 24 h prior to presentation. She was completely immunized as per the National Schedule. She had poor fixation to light, was unresponsive to verbal commands of parents and was not indicating daily needs. Her Glasgow Coma Scale score was 10. No rash was noticed at admission, her oral cavity was normal and she had insignificant bilateral submandibular lymphadenopathy. She did not have neck stiffness but had a bulging non-pulsatile anterior fontanelle. Neurological examination revealed hypotonia and paucity of movements of right lower limbs. Clinical diagnosis of pyogenic meningitis and viral meningo-encephalitis was considered and she was started on ceftriaxone, acyclovir along with hypertonic saline and glycerol for osmotherapy. Contrast-enhanced computed tomography scan was normal. Hemogram showed hemoglobin 9 g/dL, total leukocyte count 13 100/mm3 and platelet count 349 000/mm3. Lumbar puncture revealed 20 cells (100% lymphocytes), glucose 54 mg/dL (blood glucose 82 mg/dL) and proteins 28 mg/dL. Cerebrospinal fluid (CSF) analysis was negative for herpes, triple antigen screen for streptococcus, pneumococcus and Haemophilus influenzae. CSF culture, blood culture and urine examinations were sterile. Ultrasonography of the abdomen did not reveal gall bladder hydrops. Blood serology for enteric fever, dengue, scrub typhus and leptospira were negative. Consciousness and neurodeficit improved by day 4; however, irritability persisted. She continued to have low-grade fever till day 11 of illness, progressive thrombocytosis (487 000/mm3), periungual desquamation of fingers (Fig. 1), mild transaminitis (two times the upper normal limit), increasing erythrocyte sedimentation rate (from 18 to 48 mm/first hour) and erythema/edema at site of bacillus Calmette-Guérin (BCG) vaccination (suggestive of BCG reactivation). Diagnosis of Kawasaki disease (KD) was considered as the child satisfied four out of five clinical criteria described by the American Heart Association (all clinical criteria except cervical lymphadenopathy) and after exclusion of alternative diagnoses.1 She was administered intravenous immunoglobulin 2 g/kg. Echocardiography at diagnosis and after 6 weeks did not suggest any involvement of coronary arteries. She was discharged on oral aspirin and has remained well at 24 months follow-up. Kawasaki disease is an uncommon cause of aseptic meningitis and meningo-vasculitis. The diagnosis of KD is based on a constellation of clinical symptoms. Diagnosis of KD in infants is difficult as incomplete and atypical forms of KD are more common than in older age groups. The diagnosis of KD is clinical, based on a constellation of symptoms.1 Involvement of the central nervous system is rare in KD and occurs in 0.4% of children.2 Irritability is a most common manifestation, although other manifestations like seizures, severe alteration of consciousness, focal deficits, isolated cranial nerve palsies, pseudotumor cerebri and subdural effusion have been described only in isolated case reports.3, 4 KD is an arteritis involving small and medium sized arteries. Similarly, cerebral vasculitis of small end-on vessels may cause focal hypoperfusion leading to lacunar stroke and neurodeficits. Such focal perfusion abnormalities can be missed even on magnetic resonance imaging and can be detected by single-photon emission computed tomography.5 Differentiation of KD on the basis of CSF parameters is difficult. Findings are nonspecific and may be indistinguishable from any viral meningitis and encephalitis. CSF may show elevated pleocytosis, mononuclear cells, normal glucose and normal or mildly elevated CSF proteins.6 Higher pleocytosis has been observed in younger children. The index child was also treated in lines of viral meningo-encephalitis until desquamation appeared. Although in isolation, desquamation is non-specific; it was the sequential timing of symptoms, persistent irritability and fever, and the constellation, which suggested KD as the diagnosis.7 Although uncommon, KD should be considered as a differential diagnosis of fever, rash and encephalopathy, especially in infants, as timely detection can prevent severe complications like coronary aneurysms. None. SRD drafted the manuscript; PCV, DS, SS case management and edited the manuscript, PDS edited the manuscript and did critical review. An informed consent form was signed by the parents of the patient to approve the use of patient information or material for scientific purposes. The manuscript and the figure do not reveal the identity of the child.