N. Kranjc
Klinični oddelek za otroško, mladostniško in razvojno nevrologijo, Pediatrična klinika, Univerzitetni klinični center Ljubljana, Ljubljana, Slovenija

M. Perković Benedik
Klinični oddelek za otroško, mladostniško in razvojno nevrologijo, Pediatrična klinika, Univerzitetni klinični center Ljubljana, Ljubljana, Slovenija

Abstract

The causes of encephalitis are numerous, and extensive investigations for infectious agents and other aetiologies are often negative. The discovery that the causes are often immune-mediated has changed the approach to the diagnosis and treatment of these disorders. Moreover, the broad spectrum of symptoms, including psychosis, catatonia, alterations in behaviour and memory, seizures, abnormal movements and autonomic dysregulation, usually requires a multidisciplinary treatment approach. This review focuses on several forms of encephalitis that occur in children, and for which an autoimmune aetiology has been demonstrated (e.g. anti-N-methyl-D-aspartate receptor encephalitis, anti-NMDA-R) or is strongly suspected (e.g. Rasmussen encephalitis, limbic encephalitis, opsoclonus-myoclonus syndrome). The authors also review several disorders that may be immune-mediated, such as rapid-onset obesity with hypothalamic dysfunction, hypoventilation and autonomic dysregulation and some encephalopathies with fever and status epilepticus. Recognition of new forms of immune-mediated encephalitis is important because some of these disorders are highly responsive to immunotherapy.

Key words: encephalitis, autoimmune, children.