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encephalitis lethargica; sleeping sickness; von Economo's disease
Etiology: unknown, ? viral
Epidemiology:
1) epidemics documented in early 1915-1920 in France, Austria, England
2) practically disappeared by 1930s
3) sporadic cases still occur
Pathology:
1) congestion & inflammation of grey matter
a) cerebral cortex
b) basal ganglia
c) brainstem
d) cervical spinal cord (sometimes)
2) perivascular infiltration with lymphocytes & plasma cells
3) inflammation greatest in the midbrain
4) neuronal degeneration in
a) substantia nigra
b) oculomotor nuclei
c) lentiform nucleus
d) hypothalamus
5) gliosis accompanying neuronal degeneration
Clinical manifestations:
1) fever
2) somnolence, lethargy
3) coma may occur
4) oculomotor palsies
5) meningeal irritation
6) headache
Complications:
1) death
2) post-encephalitic parkinsonism
Management:
1) symptomatic
2) Sinemet & other anti-Parkinson agents may be useful
Related
post-encephalitic parkinsonism
General
encephalitis
References
- Greenfield's Neuropathology, 5th ed., 1992 p.383, 997-8
- NINDS Encephalitis Lethargica Information Page
https://www.ninds.nih.gov/Disorders/All-Disorders/Encephalitis-Lethargica-Information-Page