Clinical, Laboratory, and Radiological Findings in Acute Necrotising Encephalopathy of Childhood: A Case Series

Abstract

Acute Necrotising Encephalopathy of Childhood (ANEC) is a fatal disease diagnosed on basis of clinical and typical Magnetic Resonance Imaging (MRI) findings. The prognosis of this encephalopathy is generally poor, with fewer than 10% of cases recovering completely. Patients with a favourable outcome had reversible neuroimaging findings. Long-term sequelae commonly include neurologic deficits such as spasticity, epilepsy, and involuntary movements. In this case series, five cases of ANEC were reported. The children included in this series ranged in age from nine months to 16 years. All the children were female, and their presenting symptoms included seizures (n=5), altered sensorium (n=5), features of raised intracranial pressure (n=5), and posturing (n=1). Neuroimaging revealed characteristic lesions in the thalamus with varied involvement of other parts of the brain in all cases (n=5). Cerebrospinal Fluid (CSF) analysis was normal in the majority of children. Four out of five cases survived and responded to early steroids, intravenous immunoglobulin, physiotherapy, and rehabilitation. One child had complete recovery, with three children experiencing disabilities and one child succumbing to the disease.

Keywords

• intravenous immunoglobulin
• thalamic lesions
• spasticity