Polyradikulomeningoenzephalitis durch Epstein-Barr-Virusinfektion - Darstellung eines Falles mit tödlichem Ausgang

Abstract

A 21 years old high school student, who, since early childhood, suffered from complete spinal paraplegia of unknown cause at D 10, suddenly, after a three week period with influenca -like prodromal symptoms, presented with acute polyradiculomeningoencephalitis . Positive reactions following Paul-Bunnell- Davidsohn -test and tests for specific antivirus IgG as well as specific IgM revealed acute Epstein-Barr-Virus infection, although no glandular signs could be found. The clinical findings consisted of peripheral sensory-motor tetraparesis, ataxia, nystagmus and disturbances of the caudal cranial nerves. Initially, the diagnosis of Fisher's syndrome was made. The orbicularis oculi reflex, however, indicated a circumscribed brainstem lesion in the medial reticular formation. This finding lead to the additional clinical diagnosis of encephalitis. Shortly after admission, a heart-arrest occurred, and, after successful reanimation, the patient presented with a "locked-in syndrome". On the 12th day after admission, he succumbed during a sudden drop of blood-pressure. Necropsy revealed septic shock as the cause of death, due to perforation of a duodenal ulcer. In the lower thoracic spinal cord a neurocytoma was found, which once lead to the paraplegia. Additionally, moderate inflammatory infiltrations were found in the basal leptomeninges, in the walls of several subcortical vessels and in the hypoglossal nerve. The infiltrations, at some sites, contained a large number of atypical lymphoid cells in the inflammatory meningeal exsudate . Microglial proliferation with satellitosis was found in the inferior olives, thus confirming the clinical diagnosis of polyradiculomeningoencephalitis . This case report should emphasize special features of EBV-infection with neurological complications: (1) in inflammatory diseases of the peripheral and central nervous system, infectious mononucleosis should always be drawn into the diagnostic considerations, even if heterophil antibodies and glandular symptoms are lacking. (2) In Guillain-Barré-syndrome, a transient external heart stimulator should be administered, in order to prevent heart arrest from acute autonomic neuropathy.