A child case presenting with diffuse dural thickening due to a coma after unsuccessful treatment for a first-episode neuromyelitis optica spectrum disorder

Introduction

Fulminant demyelinating diseases, including neuromyelitis optica spectrum disorder (NMOSD), progress rapidly with severe outcomes. We report a pediatric case of NMOSD with diffuse brain edema and a poor neurological prognosis.

Case presentation

A 6-year-old boy received an influenza vaccination 12 days prior to onset, and subsequently developed fever, headache, decreased urination frequency, and constipation. He presented with seizures and a disturbance of consciousness accompanied by hyperthermia (> 41 °C). Physical examination revealed tachycardia, neck stiffness, and papilledema. Magnetic resonance imaging (MRI) revealed scattered white matter lesions on T2-weighted images (T2WI) and longitudinally extensive spinal cord lesions from C4 to Th9. A cerebrospinal fluid (CSF) examination revealed elevated pressure and pleocytosis. Despite performing methylprednisolone pulse therapy following the diagnosis of NMOSD, the patient developed distributive shock. Intensive care was introduced, but the patient developed severe brain edema, and his consciousness did not recover after the cessation of intensive care. On day 30, an EEG revealed a flat trace. On day 38, contrast-enhanced MRI revealed diffuse dural thickening. Serum anti-aquaporin-4 antibodies and anti-myelin oligodendrocyte glycoprotein antibodies were negative.Discussion: We speculate that the combination of hyperthermia, intracranial hypertension, and distributive shock led to severe cerebral ischemia in this case. Even in the absence of overt brain edema the initial MRI, a severe clinical course may still occur.

Conclusion

In cases of acute encephalopathy or encephalitis presenting with spinal cord symptoms, hyperthermia, and intracranial hypertension, aggressive temperature and circulatory management under intensive care is essential to prevent severe brain edema.