Chronic meningoencephalitis caused by Echo virus 6 in a patient with common variable immunodeficiency

Abstract

Chronic enteroviral meningoencephalitis, most commonly caused by echoviruses, can particularly be seen in agammaglobulinemic patients. In spite of the fact that no specific treatment for enteroviral infections exists, pleconaril is an antiviral drug reported to be efficient against enteroviral infections in infants and adults. We present a case of a 42-year-old male, previously diagnosed with common variable immunodeficiency, who presented with severe chronic meningoencephalitis caused by Echo virus 6 and was successfully treated with pleconaril. Enteroviruses usually cause mild symptoms, but some strains can cause life-threatening conditions especially in immunocompromised patients. Although pleconaril production is unprofitable due to the rarity of severe disease, our effective treatment should encourage further availability of pleconaril.

Chronic enteroviral meningoencephalitis in a patient on rituximab for the treatment of psoriatic arthritis: A case report and brief literature review

“…Previously, the administration of high-dose IVIG and pleconaril, an antipicornaviral agent, were the mainstay of treatment for enteroviral meningoencephalitis [1,4,10]. However, there is very little evidence in the present body of medical literature to support this.…”

“…Aseptic meningitis is a classic manifestation and is usually self-limited, however, can lead to severe neurological complications in an immunocompromised individual. It has been well-described that patients with hypogammaglobulinemia are predisposed to developing chronic enteroviral meningoencephalitis [1]. This is the first reported case of enteroviral meningoencephalitis in a patient being treated for psoriatic arthritis with rituximab.…”

Peripheral B Cell Deficiency and Predisposition to Viral Infections: The Paradigm of Immune Deficiencies

“…Although less common in comparison to XLA, chronic meningoencephalitis by enteroviruses and post-vaccination poliomyelitis have also been described in these patients (19,26,27,29). Asymptomatic infection is also common (30,31).…”

Viral infection in primary antibody deficiency syndromes

“…The chronicity and inability to successfully clear virus from the CSF are seen much more frequently in XLA than other antibody‐deficient syndromes such as CVID and X‐linked hyper IgM syndrome although the reason for this remains unclear . Despite the prolonged faecal shedding times associated with poliovirus, the same is not seen with echoviruses, suggesting that recurrent autoinfection is unlikely to be responsible for the chronicity.…”

“…Intrathecal administration of immunoglobulin has also been attempted in nonresponders, with some benefit reported, but failure is common, and the procedure is not without risk . Antivirals, most notably pleconaril, have also been reported to confer some benefit but are not widely available. In all studies of treatment, the small number of cases makes powering for meaningful effect sizes difficult.…”