Rare cases of leukoencephalitis have been reported in infants with documented enterovirus (EV) central nervous system (CNS) infections. A case of fatal encephalitis with white matter lesions caused by echovirus 18 is described, and it highlights the role of EV CNS infection as a potential cause of leukoencephalitis in infants.
CASE REPORTThe patient described herein was an 18-month-old male infant who was the eldest child in a three-child family and had no particular medical history. Twelve hours before his hospital admission, his parents reported fever, vomiting, and a general tonic and clonic seizure. On hospital admission, he was afebrile (36.5°C), conscious, feverish, and without any particular symptoms on physical and neurological examination. Lumbar puncture revealed the presence of 184 white cells per l of cerebrospinal fluid (CSF) with 98% lymphocytes. CSF protein and glucose concentrations were 2.08 g/liter and 3.77 mmol/liter, respectively, and the blood concentration was 5.74 mmol/liter. No bacterial counts were observed after classical staining of CSF. Moreover, electroencephalogram findings revealed diffuse and excessive slow-wave activity. Initial treatment included ceftriaxone (100 mg/kg/day), amoxicillin (100 mg/kg/ day), and acyclovir (250 mg/m 2 ). Following a new episode of febrile general seizure, the infant was transferred to the pediatric department of the regional university hospital (Champagne Ardenne, France). On admission, his child-modified Glasgow score was 13/15, with eyes opening in response to a voice and consolable crying; he was afebrile, tachycardic, and normotensive. A second lumbar puncture revealed the presence of 81 white cells per l of CSF with 100% mononuclear cells. CSF protein and glucose concentrations were 3.2 g/liter and 3.1 mmol/liter, respectively, associated with an alpha interferon (IFN-␣) level of 12 IU/ml (the IFN-␣ level was Ͻ2 IU/ml of blood plasma). Three days after the beginning of febrile illness, the Glasgow score remained the same (13/15) and was not associated with high levels of C-reactive protein (Ͻ3 mg/liter). Classical biochemical parameters reflecting metabolic disorders were examined and found to be normal, indicating the absence of an inborn metabolic disorder.Two days after the admission of this infant to the university hospital, brain magnetic resonance imaging (MRI) studies with and without gadolinium revealed a diffuse hyperintense signal in the white matter. MRI T2 showed modifications, symmetrically involving the white matter of both cerebral hemispheres, that were predominantly located in the periventricular and subcortical white matter ( Fig. 1A and C). Seven days after his initial admission to the university hospital, a second brain MRI study revealed an extensive and diffuse hyperintense signal in the subcortical and deep white matter of both cerebral hemispheres ( Fig. 1B and D). At that time, several images showed extension of the hyperintense signal into the cortical gray matter (not shown).Testing of CSF, peripheral blood, throat, an...