22.: The pseudo-poliomyelitis of the 21st century, enterovirus 71

Between January 2013 and June 2013, 35 children presented with neurologic symptoms and enterovirus positivity. Twenty-six of 35 were genotyped and found to have enterovirus 71 (EV17). There were four deaths statewide. One of the difficulties is that neurologic symptoms of these children are variable, as are laboratory and medical imaging results. There do not appear to be robust predictors to indicate which children may have an adverse outcome. This research aims to develop a further understanding of the disease course and potential therapies in EV71. A severity score index will be developed for the first time, incorporating clinical, neuroimaging and laboratory data from EV71 patients. Clinical presentation, investigation results, imaging findings, treatment and short-term outcome of these 35 patients were collected. A score of one point was given for each the following clinical symptoms: myoclonic jerks, seizures, ataxia, vomiting, urinary retention, limb weakness, truncal weakness, cranial neuropathy, and autonomic instability (respiratory distress, tachycardia/bradycardia, hypertension/hypotension). When available, a score was also given for positive MRI findings of intracranial lesion and/or spinal cord lesion. Maximum severity score for each patient was 14. Mild severity score was 1–3, moderate was 4–10 and severe 10–14. Symptoms of these patients were variable, most common being myoclonic jerks and ataxia. Twenty-one patients had an MRI, two-thirds of which showed an abnormality. Treatment choice was variable with some patients receiving both intravenous immunoglobulin (IVIG) and high dose methylprednisolone and some no special treatment. Patients who had higher severity score were frequently treated. Three patients with severe scores needed full cardio-respiratory support and received both IVIG and methylprednisolone. Patients with moderate severity score who were treated had better outcome (p = 0.011). This finding is in keeping with World Health Organization comment that IVIG should be considered for the autonomic dysfunction group and possibly in the central nervous system group. EV71 outbreaks can have devastating consequences. In the context of an epidemic, fever, lethargy and myoclonus are pathognomonic. Other features include ataxia and flaccid weakness. There is a role for IG therapy in patients who are at risk of severe disease. Using a severity score is helpful in detecting patients at risk. MRI is a useful tool in diagnosis, with diffusion restriction in the brainstem associated with a poor outcome. Maintaining awareness for the potential for cardiopulmonary collapse is essential.