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Kawasaki disease presented with status epilepticus and diffusion MRI abnormalities in the subcortical white matter
Dai Keino1, Yuki Koto1, Chisato Inuo1
1Department of Pediatrics, St. Marianna University School of Medicine, Kawasaki, Kanagawa, Japan.
Insights
Kawasaki disease (KD) can rarely cause acute encephalopathy with biphasic seizures and late reduced diffusion (AESD). Early MRI diagnosis and treatment in infants with KD are crucial for managing this rare complication.
Area of Science:
- Pediatric Neurology
- Infectious Diseases
- Vascular Biology
Background:
- Kawasaki disease (KD) is an acute, self-limited vasculitis primarily affecting infants and young children.
- Encephalitis or encephalopathy is an exceptionally rare complication of KD.
Observation:
- An 8-month-old boy presented with prolonged seizures following a brief febrile illness.
- Clinical signs consistent with incomplete KD were observed, alongside cluster seizures and MRI findings indicative of acute encephalopathy with biphasic seizures and late reduced diffusion (AESD).
Findings:
- The patient received immunoglobulin therapy for incomplete KD and subsequent treatment for AESD, including normothermia, methylprednisolone, midazolam, and edaravone.
- Brain MRI revealed reversible diffusion restriction in the white matter, which resolved after treatment.
- The patient demonstrated normal neurodevelopmental progress at 11 months of age.
Implications:
- Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) should be considered in the differential diagnosis of prolonged seizures in infants with Kawasaki disease.
- Early diagnosis via brain MRI and prompt intervention are vital for managing this rare KD complication.
- Further research and case accumulation are necessary to understand the long-term neurological prognosis of Kawasaki disease associated with AESD.
Background:
Kawasaki disease (KD) is an acute, self-limited vasculitis of unknown etiology that occurs predominantly in infants and young children. Encephalitis/encephalopathy is an extremely rare complication of KD.
Case:
A previously healthy 8-month-old Japanese boy had a prolonged seizure after febrile illness for one day. On the fourth day, he had bilateral nonexudative conjunctivitis, changes in the extremities, rash and induration at the Bacillus Calmette-Guerin inoculation site. He was diagnosed with incomplete KD and treated with immunoglobulin. On the fifth day, he had cluster seizures. Brain magnetic resonance imaging (MRI) showed restricted diffusion in the left subcortical white matter, which was consistent with acute encephalopathy with biphasic seizures and late reduced diffusion (AESD). He was treated with controlled normothermia, pulseddose methylprednisolone, continuous infusion of midazolam, and edaravone. On the tenth day, he had a recurrent fever and was treated with a second course of immunoglobulin. Subsequently, he had defervescence, and the abnormal signal detected in the MRI disappeared. At the age of 11 months, he had normal growth and development for his age by the Denver Developmental Screening Test.
Conclusion:
It is necessary to consider AESD as the differential diagnosis of prolonged seizure in infants with KD. Brain MRI led to early diagnosis and intervention in our patient. The neurological prognosis of our patient was relatively good, but the prognosis of KD with AESD is unknown. To clarify this, further case accumulation is warranted.
