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Brain, Vol. 127, No. 1, 2-3, 2004
© 2004 Guarantors of Brain
doi: 10.1093/brain/awh063
Encephalitis lethargica: part of a spectrum of post-streptococcal autoimmune diseases?
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Encephalitis lethargica (EL) was first described by von Economo in 1917, shortly after the start of the 1916–1927 epidemic. The patients, mostly children of either sex, characteristically presented with headache and malaise, lethargy, insomnia, and ophthalmoplegia. Some recovered but the others either died during an acute fulminating disorder or developed, insidiously or after a variable period of time, movement and/or psychiatric disorders including Parkinsonism, oculogyric crises, chorea, myoclonus, mutism, catatonia or behavioural problems. Although linked by many observers to the influenza epidemic, the epidemic of EL began earlier and lasted longer, and flu virus has not been found in archival post-mortem tissue (e.g. McCall et al., 2001
; Lo and Geddes, 2003). Sporadic cases are still reported, but the acute fulminating form seems to have disappeared, and there have
1 Neurosciences Group, Department of Clinical Neurology, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Oxford UK
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