Session Type: ePosters
Session Title: ePosters
Authors(s): T. Shinha
Authors Affiliations(s): Internal Medicine and Infectious Diseases, Inc, United States
Background:
Epstein-Barr Virus (EBV) is associated with a variety of neurological manifestations. However, its association with rhombencephalitis referring to infectious pathologies involving the brainstem and/or cerebellum is less well recognized. In this study, we discuss the clinical, laboratory, and radiographic features of EBV-associated rhombencephalitis.
Case:A retrospective case review was conducted on the available medical literature. Rhombencephalitis was defined by brainstem and/or cerebellar involvement according to clinical presentation, MRI findings, or cerebrospinal fluid (CSF) abnormalities with pleocytosis (>4 cells/mm3). We analyzed 27 cases of EBV-associated rhombencephalitis reported between 1984 and 2019. In addition, we report a new case.
A 19-year-old woman presented with headache, nuchal rigidity, and altered mental status. CSF analysis revealed total nucleated cells (TNCs) of 127 cells/mm3, protein 229 mg/dL, and glucose 34 mg/dL. Brain MRI revealed hazy brain stem enhancement. CSF EBV DNA PCR and serum EBV IgM tests were positive.
Among the 27 retrospectively reviewed cases of EBV-associated rhombencephalitis, 17 (63%) were male, and 10 were female (37%). The mean age was 29.1 years (range: 8 months to 79 years). The presenting symptoms evaluated were fever (n=19, 70%), altered mental status (n=22, 81%), cranial nerve involvement (n=21, 78%), ataxia (n=16, 59%), and meningeal signs (n=5, 18%). CSF analysis showed mean TNCs of 72 cells/mm3 (range 2–247, n=24), protein levels of 107.8 mg/dL (range 16–300, n=20), and glucose levels of 58.2 mg/dL (range 31-98, n=16). The MRI findings were abnormal in 25 cases (92%); cerebellum involvement was found in 18 cases (67%), and pons was found in 5 cases (19%). Hydrocephalus was reported in 6 cases (22%); 17 cases (63 %) tested positive for CSF EBV PCR; and 19 (70%) tested positive for serum EBV IgM.
Discussion:EBV-associated rhombencephalitis occurs in a wide age distribution; however, it is more commonly reported in younger populations. It frequently presents with fever and altered mental status. Ataxia is a common neurological manifestation. MRI findings involving the cerebellum or pons are valuable for diagnosis. CSF abnormalities are generally nonspecific. Both CSF EBV DNA PCR and serum EBV serology should be ordered to increase the diagnostic yield.
Session Type: ePosters
Session Title: ePosters
Authors(s): T. Shinha
Authors Affiliations(s): Internal Medicine and Infectious Diseases, Inc, United States
Background:
Epstein-Barr Virus (EBV) is associated with a variety of neurological manifestations. However, its association with rhombencephalitis referring to infectious pathologies involving the brainstem and/or cerebellum is less well recognized. In this study, we discuss the clinical, laboratory, and radiographic features of EBV-associated rhombencephalitis.
Case:A retrospective case review was conducted on the available medical literature. Rhombencephalitis was defined by brainstem and/or cerebellar involvement according to clinical presentation, MRI findings, or cerebrospinal fluid (CSF) abnormalities with pleocytosis (>4 cells/mm3). We analyzed 27 cases of EBV-associated rhombencephalitis reported between 1984 and 2019. In addition, we report a new case.
A 19-year-old woman presented with headache, nuchal rigidity, and altered mental status. CSF analysis revealed total nucleated cells (TNCs) of 127 cells/mm3, protein 229 mg/dL, and glucose 34 mg/dL. Brain MRI revealed hazy brain stem enhancement. CSF EBV DNA PCR and serum EBV IgM tests were positive.
Among the 27 retrospectively reviewed cases of EBV-associated rhombencephalitis, 17 (63%) were male, and 10 were female (37%). The mean age was 29.1 years (range: 8 months to 79 years). The presenting symptoms evaluated were fever (n=19, 70%), altered mental status (n=22, 81%), cranial nerve involvement (n=21, 78%), ataxia (n=16, 59%), and meningeal signs (n=5, 18%). CSF analysis showed mean TNCs of 72 cells/mm3 (range 2–247, n=24), protein levels of 107.8 mg/dL (range 16–300, n=20), and glucose levels of 58.2 mg/dL (range 31-98, n=16). The MRI findings were abnormal in 25 cases (92%); cerebellum involvement was found in 18 cases (67%), and pons was found in 5 cases (19%). Hydrocephalus was reported in 6 cases (22%); 17 cases (63 %) tested positive for CSF EBV PCR; and 19 (70%) tested positive for serum EBV IgM.
Discussion:EBV-associated rhombencephalitis occurs in a wide age distribution; however, it is more commonly reported in younger populations. It frequently presents with fever and altered mental status. Ataxia is a common neurological manifestation. MRI findings involving the cerebellum or pons are valuable for diagnosis. CSF abnormalities are generally nonspecific. Both CSF EBV DNA PCR and serum EBV serology should be ordered to increase the diagnostic yield.