Encephalitis lethargica (von Economo disease)
Recent hystory of pharyngitis. Somnolence. Ophthalmoplegia and oculogyric crises. Bradykinesia. Emotional lability.
ASNR 2016: This case was submitted as part of the American Society of Neuroradiology (ASNR 2016) Case Of The Day competition, in collaboration with Radiopaedia.org.
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Bilateral and symmetric high signal lesions in mesencephalon (including substantia nigra), hypothalamus, striatum and internal temporal lobe.
Sparing of the insular and basi frontal regions. Enhancement of the basal ganglia, anterior commissure, striatum, and right medial temporal region.
Lipids peak, low ratio NAA/ creatine.
4 case questions available
Association of clinic pharyngitis followed by sleep disorder, basal ganglia signs (particularly Parkinsonism), neuropsychiatric sequelae and MRI swelling in the basal ganglia and the substantia nigra is very suggestive of Encephalitis lethargica. It is a rare disease, first described by von Economo in 1916 during an epidemic, affecting 500,000 people worldwide in 1916-1927. There has been no further epidemic of EL reported since the 1920s although sporadic cases have been described.
Alternative diagnoses were excluded with the help of multiple immunohistochemistry test in LCR. Infectious/inflammatory laboratory and serological workup were normal. Furthermore a scan of the chest, abdomen, and pelvis was normal. Intrathecal oligoclonal bands (OCB) were found.
Sparing of the insular and basifrontal regions rule out confounding diagnosis.
Steroid and immunomodulating therapy were administered with a beneficial effect. There were only mild neuropsychiatric symptoms and Parkinsonism sequelae.
- K. von Economo. Encepahlitis lethargica. Wiener klinische Wochenschrift, May 10, 1917, 30: 581-585. Die Encephalitis lethargica. Leipzig and Vienna, Franz Deuticke, 1918
- Encephalitis lethargica syndrome: 20 new cases and evidence of basal ganglia autoimmunity Brain, 127 (2004), pp. 21–33
- Morris S. Dickman, MD von Economo Encephalitis Arch Neurol. 2001; 58; 1696-1698
- Lopez-Alberola R, Georgiou M, Sfakianakis G, Singer C, Papapetropoulos S. Contemporary Encephalitis Lethargica: Phenotype, laboratory findings and treatment outcomes. J Neurol (2009) 256:396–404