Intravenous immunoglobulins in an adult case of post-EBV cerebellitis

Abstract

Post-Epstein-Barr virus (EBV) cerebellitis is very rare complication of infectious mononucleosis and only a few adult cases are reported in literature. We present a 23-year-old patient who was admitted to the neurology service with worsening ataxia, nystagmus and dysarthria, 1 week after infectious mononucleosis. Imaging and cerebrospinal fluid studies were normal, serum studies revealed acute transaminitis and positive EBV viral capsid IgM and IgG. The patient underwent a 5-day course of intravenous immunoglobulins with rapid resolution of all his symptoms and was safely discharged home. The pathophysiology of post-EBV cerebellitis involves autoreactive antibodies, rather than a direct viral insult. Antineuronal antibodies might be the result of a mimicry between EBV proteins and neuronal antigens or they can be secreted by the EBV-transformed lymphocytes themselves. Many reports stress the benign, self-limiting nature of this syndrome; however, immunotherapy might de facto decrease the severity and duration of illness.

Post-Infectious Acute Cerebellar Ataxia Treatment, a Case Report and Review of Literature

“…Several studies suggest an autoimmune process [ 7 , 40 , 41 ], where ataxia is usually a self-benign manifestation driven by an immunomediated mechanism triggered by the viral infection [ 14 , 42 ]. These observations suggest that therapies directed at the immune system might be useful in treating post-EBV cerebellar ataxia.…”

Case Report: Epstein–Barr Virus Encephalitis Complicated With Brain Stem Hemorrhage in an Immune-Competent Adult

“…The autopsy results of patients who died of EBV encephalitis show mononuclear cell infiltrates in the perivascular and necrotic hemorrhagic focus in the brain (9,15,29). Therefore, the pathogenesis of EBV encephalitis is more inclined towards inflammation caused by the immune response rather than the virus itself (30), and most patients with EBV encephalitis are treated with intravenous immunoglobulins (31) and steroids (15,21). Currently, ganciclovir (6,13,27) and acyclovir (21,27) are often used for treatment, but there is no standard treatment strategy for EBV encephalitis.…”

Treatment Options for Epstein-Barr Virus-Related Disorders of the Central Nervous System

“…Intravenous gamma globulin and plasmapheresis have been used in the treatment of cerebellar ataxia complicating EBV primary infection, although this condition is usually benign and self-limiting. 141 Treatment with corticosteroids was used in a few cases, with doubtful results 73 (Table 2). 58 Reactivation is suggested by temporal relation to immune deficiency state due to HIV infection or post-transplantation drug-induced immune deficiency, as well as by a constellation of EBV antibodies with high anti-EBNA1 and low anti-VCA IgM.…”