Clinical Management
GAYATHRI C V, MBBS, MD
Senior Resident
Pediatric Allergy Immunology Unit, Department of Pediatrics, PGIMER, Chandigarh
Chandigarh, Chandigarh, India
Background: Central nervous system manifestations reported in KD include extreme irritability, aseptic meningitis, sensorineural hearing loss and rarely cranial nerve palsies. We report an unusual clinical finding: a toddler with complete KD and abducens nerve palsy (ANP).
Methods: A 3-year-old boy came with 9 days of persistent fever and 7 days of inward deviation of the left eye. He also had redness of the eyes without any discharge, a diffuse rash over the trunk and cracking of lips with red tongue. Physical examination showed sheet-like periungual peeling of skin, left cervical lymphadenopathy and irritability. There was restriction of abduction in the left eye on attempted left gaze suggesting left lateral rectus palsy. Fundus examination did not show any papilledema. Investigations revealed neutrophilic leukocytosis, thrombocytosis (WBC: 17.2x109/L; 59% neutrophils, platelet count: 820x109/L); raised ESR (60mm/hour) and CRP (84 mg/L). 2D ECHO revealed normal coronary arteries. Lumbar cerebrospinal fluid(CSF) analysis showed lymphocytic pleocytosis. CSF infective workup was normal. Magnetic Resonance Imaging (MRI) brain was normal. Based on the above features diagnosis of complete KD was with an atypical manifestation of left ANP was proffered. He was treated with IVIG 2g/kg along with aspirin at antiplatelet doses at day 9 of illness. Though he had a defervescence of fever, there was persistence of abducens palsy. Hence, oral prednisolone started with gradual tapering. He had complete resolution of lateral rectus palsy at 6 weeks follow-up.
Results: Neurological manifestations of KD can occur in up to 30% of patients. Abducens cranial nerve palsy is a rare manifestation of KD with only 4 case reports of abducens cranial nerve palsy. The pathogenesis of cranial nerve palsy in Kawasaki remains unclear. It is postulated to be due to peri neuritis associated with aseptic meningitis seen in KD [3,4]. Including the index child, 80% of cases with abducens nerve palsy required steroids to reverse the clinical features.
Conclusion: This case highlights that cranial nerve palsy, including ANP, in KD may require treatment intensification with corticosteroid. It also underscores the need to identify the atypical manifestations of KD promptly to offset delays in diagnosis and unnecessary investigations.