Progressive Course of Chronic Tick-Borne Encephalitis Manifesting as Amyotrophic Lateral Sclerosis-like Syndrome 35 Years after the Acute Disease

Abstract

The chronic form of tick-borne encephalitis (TBE) is understudied and seems to be linked exclusively to Siberian and Far Eastern TBE virus (TBEV) subtypes. There are limited clinical descriptions demonstrating that chronic TBE can resemble an amyotrophic lateral sclerosis (ALS)-like disease. Here, we present a description of a clinical case of progressive chronic TBEV infection with a relapse 35 years after the initial acute infection following a tick bite. The disease manifested as an ALS-like syndrome with bulbar signs, progressive muscle weakness and atrophy, decreased reflexes, and eventual respiratory failure and death. There is no clear differentiation between chronic TBE and postencephalitic syndrome described in European sources. The reactivation of TBEV infection was supported by the presence of anti-TBEV antibodies in serum and antibodies to E protein and to the nonstructural protein NS1 in the CSF. These findings support the diagnosis of a relapse of chronic TBE in this patient.