Abstract
INTRODUCTION: Presence of a encephalopathy following a febrile illness in the past week in the absence of encephalitis and other causes of encephalopathy with distinct clinicoradiological features is defined as Infection Triggered Encephalopathy Syndrome (ITES). It's an emerging clinical entity and the current study describes the varying manifestations of the disorder OBJECTIVES: 1. Describe the clinical spectrum of patients with ITES. 2. Describe the radiological changes in patients with ITES. 3. Therapeutic modalities used in the patients. 4. Outcome-duration of hospital stay, neurological status at discharge, mortality. MATERIALS AND METHODS: Children <18 years presenting with encephalopathy following a febrile illness and satisfying criteria for ITES were included and their clinical, radiological and treatment data with outcome measures were studied. They were classified into various ITES based on classic radiological description and clinical presentation. RESULTS: 14 children were included during the study period.42.8% had Acute Necrotizing Encephalopathy(ANE),42.8% had Acute leukoencephalopathy with restricted diffusion(ALERD),14.2% had mild encephalopathy with a reversible splenial lesion (MERS). Average age of presentation was 82.3 months and 57.2% were male and 42.8% were female. All patients presented with fever except 2 and average day pf presentation was 4 days after symptom onset.71.4% had seizures at presentation and 14.3% had focal neurological deficit. Among 6/14 patients with ANEC-trigger was identified on all patients.50% had influenza A,33% had dengue and 16.6% had pneumococcal infection. 83.3% had multiorgan dysfunction and had required mechanical ventilation,66% died and among survivors the average Glasgow Outcome Scale (GOSE) was 3.5. All had received immunomodulation with pulse steroids,33.3% received anakinra,83.3% received tocilizumab. Length of stay was 2.5 days in non survivors vs 13.5 days in survivors. The children who survived had received a combination of pulse steroids and tocilizumab. Among 6/14 patients who had ALERD, trigger was identified on half of them-Influenza A and pneumococcal infection. 16.6% had multiorgan dysfunction and 33.3% had transient hemodynamic instability. Average hospital stay was 8.35 days.83.3% had received immunomodulation and all among them received pulse steroids. 16.6% had received IV immunoglobulin(IVIG) alone,16.6% received Ivig+anakinra,16.6% received Ivig+anakinra+tocilizumab.83.3% needed ventilation with an average ventilation duration of 4days and 1/6 child required tracheostomy. Average GOSE at discharge was 6.5. All children survived. 2/14 patients had MERS and parainfluenza was identified as trigger in one.50% required mechanical ventilation for 3 days. 50% received immunomodulation with pulse steroid and IVig combination. None of the patients had multiorgan dysfunction. Average length of stay was a week and GOSE at discharge was 7.5. CONCLUSIONS: In ANEC population, patients treated with immunomodulation within 72 hours of fever onset/24 hours of encephalopathy onset, had survived. Poor neurological outcome was noted in all surviving patients and a 83.3% had multiorgan failure & hyperferritinemia. Diffuse ALERD in MRI had a poor neurological outcome despite immunomodulation, though there was no mortality in any of them. Focal neurological deficits were associated with worse GOSE at discharge. MERS has good prognosis and immunomodulation may not be needed in all the patients.