A distinct neurological syndrome is being seen in some children with the Kawasaki disease-like COVID-19-related paediatric multisystem inflammatory syndrome (MIS-C), according to neurologists in the UK.
Clinicians from the UCL Institute of Neurology in London found serious neurological symptoms in four of 27 children with COVID-19-related paediatric multisystem inflammatory syndrome.
All four required intensive care unit admission despite not suffering from respiratory problems. Their neurological symptoms included encephalopathy, headache, brainstem signs with dysarthria or dysphagia, meningism and cerebellar ataxia. Peripheral nervous system involvement was seen in all patients, with global proximal muscle weakness and reduced reflexes.
Reporting their findings in JAMA Neurology they say the previously healthy children aged 8 to 15 years old showed a clinical phenotype involving both the CNS and the peripheral nervous system and with imaging showing lesions in the splenium of the corpus callosum (SCC).
Investigations ruled out other infective causes of the neurological symptoms.
The children were treated with immunomodulatory therapies such as steroids, immunoglobulin, anakinra and rituximab as part of the MIS-C management.
Two of the patients recovered and were discharged after 11-18 days, but the other two remain inpatients and although improving are wheelchair bound as a result of proximal lower-limb muscle weakness.
The report authors say negative cerebrospinal fluid results and the childrens’ response to immunosuppression, and the clinical overlap with hemophagocytic lymphohistiocytosis suggest that their condition is likely to be immune mediated.
They note that the SCC lesion on imaging are usually as sign of focal intramyelin oedema secondary to inflammation.
“The phenotype of our cohort raises the possibility of a virus-specific immunological syndrome. A plausible mechanism would be exposure of the immune system to new CNS antigens as a result of blood-brain barrier damage from SARS-CoV-2, which causes endotheliopathy and leads to an immunedirected attack on the CNS.
They suggest that since COVID-19 infection is often mild and asymptomatic in children, the virus should also be considered in the diagnosis of paediatric patients presenting with primary neurologic symptoms without systemic involvement.
“Close neurodevelopmental surveillance is required to assess the neurological and cognitive outcomes in these patients,” they write.