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Published on: June 21, 2019
Persistent preceding focal neurologic deficits in children with chronic Epstein-Barr virus encephalitis
J M Caruso1, G A Tung, G G Gascon
1Department of Pediatric Neurology, Children's National Medical Center, Washington, DC 20010, USA. jcaruso@cnmc.org
Insights
Chronic Epstein-Barr virus encephalitis can cause lasting cognitive and neurological issues in children, contrary to its usual self-limiting nature. This study highlights persistent deficits and MRI abnormalities in five pediatric cases.
Area of Science:
- Pediatric Neurology
- Infectious Diseases
- Neurovirology
Background:
- Epstein-Barr virus (EBV) encephalitis is typically self-limiting with minimal long-term effects.
- Persistent neurological deficits following EBV encephalitis in children remain poorly described.
Observation:
- Five children presented with persistent cognitive and focal neurological deficits.
- Clinical manifestations included aphasia, apraxia, behavioral changes, cognitive decline, seizures, and obsessive-compulsive disorder.
- Magnetic resonance imaging revealed various T2-weighted abnormalities in affected children.
Findings:
- All patients exhibited elevated EBV serum titers, with four testing positive for EBV in cerebrospinal fluid via PCR.
- Methylprednisolone treatment yielded minimal improvement and no MRI changes in three patients.
- EBV encephalitis can manifest insidiously with chronic neurological symptoms.
Implications:
- EBV encephalitis should be considered in the differential diagnosis of acute or chronic neurological illnesses in children.
- This study expands the understanding of EBV encephalitis beyond its typical self-limiting course.
- Recognizing chronic EBV encephalitis is crucial for timely diagnosis and management of persistent neurological deficits in pediatric populations.
Abstract:
Epstein-Barr virus encephalitis is a self-limiting disease with few sequelae. Persistence of neurologic deficits prior to and after the acute illness has yet to be described in children. We describe five children with persistent cognitive and focal neurologic deficits due to chronic Epstein-Barr virus encephalitis with various T2-weighted magnetic resonance imaging abnormalities. Clinical features were a 9-year-old boy with aphasia and apraxia, an 11-year-old girl with impulsivity and inappropriate behavior, a 17-year-old boy with deterioration of cognitive skills and judgment, a 5-year-old boy with complex-partial seizures, and a 6-year-old girl with obsessive-compulsive behavior. All patients had elevated serum Epstein-Barr virus titers for acute infection, with cerebrospinal fluid polymerase chain reaction positive for Epstein-Barr virus in four patients. Three children were treated with methylprednisolone with minimal improvement without changes on magnetic resonance imaging. Epstein-Barr virus encephalitis can present with chronic and insidious neurologic symptoms and should be considered in the differential diagnosis of children with acute or chronic neurologic illness of unknown etiology.
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