Europäische Schlafkrankheit
Encephalitis lethargica, also known as von Economo encephalitis, is a rare disease of unknown etiology that affects the midbrain and basal ganglia.
Clinical presentation
Symptoms are initially that of pharyngitis followed by lethargy, extrapyramidal movements (parkinsonism and dyskinesias), neuropsychiatric
disturbance (obsessive, compulsive disorder, catatonia, mutism, apathy and conduct disorders), and ocular movement disorder (oculogyric crises).
Pathology
The exact etiology is unknown, but there is lymphocyte infiltration of the midbrain and basal ganglia. It is thought that there may be an autoimmune component, possibly triggered after a viral infection.
Markers
Autoantibodies reactive against human basal ganglia antigens present in the majority of encephalitis lethargica patients.
Radiographic features
MRI
Brain MRI demonstrates inflammatory changes localized to the deep grey matter
in 40% of patients.
- T1: low signal
- T2/FLAIR: high signal in the basal ganglia (including the substantia nigra), midbrain, thalamus, cerebral peduncle and/or temporal cortex
- T1 C+ (Gd): leptomeningeal and focal patchy enhancement
- DWI/ADC: isointense without restriction of ADC
Nuclear medicine
- PET-CT: increased glucose metabolism in the basal ganglia
Treatment and prognosis
Treatment approaches to encephalitis lethargica include immunomodulating therapies and steroids.
History and etymology
First described by Constantin Freiherr von Economo (1876-1931), a Romanian psychiatrist and neurologist, in 1916 during an epidemic affecting 500,000 people worldwide in 1916-1927 . There have been no further epidemics of encephalitis lethargica since the 1920s, although sporadic cases have continued to be reported.
Differential diagnosis
General imaging differential considerations include:
Siehe auch:
Assoziationen und Differentialdiagnosen zu Europäische Schlafkrankheit:
