Acute Necrotizing Encephalopathy of Childhood: A Case Report

Abstract

cute necrotizing encephalopathy of childhood (ANEC) is a rare, severe atypical encephalopathy of para infectious etiology usually triggered by viral infections and is associated with rapid deterioration of sensorium and seizures [1]. It was first described by Mizuguchi from Japan in 1995 [1]. It is seen predominantly in previously healthy young children or infants of East Asian countries such as Japan and Taiwan. However, sporadic cases of this illness have been reported from all around the globe [1,2]. Influenza virus, human herpes virus 6 (HHV-6), herpes simplex virus, mycoplasma, and dengue viruses are among the most common infections causing ANEC [3-5]. Recently, acute hemorrhagic necrotizing encephalitis was observed in a young adult due to coronavirus disease-19 infection [6]. The elevation of hepatic enzymes is unique without hyperammonemia [7]. A recurrent or familial form is associated with mutations in the RANBP2 gene and is noted as ANE1 [7,8]. Classical magnetic resonance imaging (MRI) findings include multifocal symmetrical lesions in thalami, brainstem, and cerebellum [3,9]. The physicians should rule out other causes of encephalopathies such as fulminant hepatitis, Reye's syndrome, toxic shock syndrome, Leigh encephalopathy, organic academia, hypoxic brain insult, acute disseminated encephalomyelitis, venous and arterial infarcts in children, and occasionally heatstroke [10]. The prognosis is poor in most cases. We report a case of ANEC following HHV-6 infection in a previously healthy 9-month-old infant. CASE REPORT A previously healthy 9-month-old female infant presented to us with fever for 4 days, loose stools for 2 days, and decreased activity for 1 day. At the time of admission, she was irritable, drowsy and her deep tendon reflexes were brisk with the Glasgow Coma Scale of 13/15. Her pupils were equal and reacting to direct light and the hydration status of the child was good. However, after 1 h of admission in the pediatric intensive care unit (PICU), she developed right-sided focal seizure, which was aborted with short-acting benzodiazepine injection midazolam, and later, she was loaded with inj. levetiracetam 20 mg/kg IV route over 20 min. The seizure got controlled but encephalopathy with signs of meningitis (neck stiffness, Kernig's sign, and Brudzinski signs) persisted. Hence, an initial provisional diagnosis of acute meningoencephalitis was made. Other differential diagnoses kept in mind were metabolic encephalopathy with sepsis, hypoxicischemic encephalopathy (HIE) due to status epilepticus, and Reye's syndrome, respectively. She was given O 2 therapy, intravenous (IV) fluids, IV thirdgeneration cephalosporins, and IV acyclovir initially for 5 days. After stabilizing the airway, breathing, circulation, and neurological status of the child, a lumbar puncture was done after ruling out optic disc edema by fundoscopy. The cerebrospinal fluid (CSF) report revealed total two cells with 100% lymphocytes, CSF glucose was 38 mg/dl, and CSF total protein was 44 mg/dl. Thus, there was neither C...

Adenovirus-induced Acute Necrotizing Encephalopathy in a Previously Healthy Infant- A case report

“…[5] The use of Cidofovir in our case was targeted against the adenovirus in the CSF. The use of foscarnet, valganciclovir, or cidofovir has been recommended by some centers for disease control and prevention for immunodeficient children [12]. ANEC generally has a poor prognosis with high mortality and morbidity rates.…”