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).
The exact aetiology 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.
Autoantibodies reactive against human basal ganglia antigens present in the majority of encephalitis lethargica patients.
Brain MRI demonstrates inflammatory changes localised 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
- 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 4. There have been no further epidemics of encephalitis lethargica since the 1920s, although sporadic cases have continued to be reported.
General imaging differential considerations include:
- 1. Dale RC, Church AJ, Surtees RA et-al. Encephalitis lethargica syndrome: 20 new cases and evidence of basal ganglia autoimmunity. Brain. 2004;127 (1): 21-33. doi:10.1093/brain/awh008 - Pubmed citation
- 2. Morris S. Dickman, MD von Economo Encephalitis Arch Neurol. 2001; 58; 1696-1698
- 3. Lopez-Alberola R, Georgiou M, Sfakianakis GN et-al. Contemporary Encephalitis Lethargica: phenotype, laboratory findings and treatment outcomes. J. Neurol. 2009;256 (3): 396-404. doi:10.1007/s00415-009-0074-4 - Pubmed citation
- 4. von Economo, C. Encephalitis lethargica. (1917) Wiener Klinische Wochenschrift. 30, 581–585.
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Infections of the central nervous system
- classification by aetiology
- eastern equine encephalitis
- enterovirus rhomboencephalitis
- flavivirus encephalitis
herpes virus family
- herpes simplex virus 1 (HSV-1) encephalitis
- herpes simplex virus 2 (HSV-2) encephalitis
- varicella zoster virus (VZV) encephalitis
- Epstein-Barr virus (EBV) encephalitis
- cytomegalovirus (CMV) encephalitis
- human herpesvirus 6 (HHV-6) encephalitis
- HIV CNS manifestations
- HTLV-1-associated myelopathy
- JC virus
- measles encephalitis
- Nipah virus (NiV) encephalitis
- rabies encephalitis
- CNS listeriosis (Listeria monocytogenes)
- CNS nocardiosis (Nocardia spp)
- CNS tuberculosis (Mycobacterium tuberculosis)
- Lyme disease (Borrelia burgdorferi)
- neurosyphilis (Treponema pallidum)
- Rocky Mountain spotted fever (Rickettsia rickettsii)
- cerebral amoebiasis
- cerebral malaria (Plasmodium falciparum)
- cerebral sparganosis (Spirometra mansonoides)
- neurocysticercosis (Taenia solium)
- neurohydatidosis (Echinococcus spp)
- neurotoxoplasmosis (Toxoplasma gondii)
- others or those with possible infectious aetiologies
- classification by location
- meninges and ventricular system
- brain parenchyma, cerebral vasculature, brainstem, and spinal cord
- classification by aetiology