Eastern equine encephalitis
Eastern equine encephalitis (EEE) is one of many viral encephalitides and results from infection with the eastern equine encephalitis virus.
Most patients have non-specific viral prodromal symptoms for approximately one week, including fevers, headache, nausea and vomiting, and malaise 1. This is followed by the emergence of neurological features, such as confusion, focal neurological signs, generalized or focal seizures, meningism, and somnolence 1. These neurological features rapidly progress and the patient's condition typically deteriorates within days to become stuporous or comatose 1.
EEE is caused by the eastern equine encephalitis virus (EEEV), a single-stranded RNA alphavirus 1,2. Mosquitos and wild birds, especially in the east and Gulf coasts of the Unites States of America, are reservoirs for the virus, which is then spread by mosquitoes 1,2. Importantly, only approximately 5% of patients with EEEV infection develop EEE 2.
Asymmetric bilateral basal ganglia and thalami involvement is classical regardless of CT or MR imaging 1-6. Less commonly, the internal and external capsules, brain stem, periventricular white matter, cortex, and leptomeninges may also be involved 1-6.
MRI is the imaging modality of choice and is nearly always abnormal in patients with EEE 1,3. The most striking feature are asymmetric high T2-weighted signal intensities classically affecting the basal ganglia and thalami 1-6. It is thought that these lesions represent a combination of edema, inflammation, and ischemia 1,3. As with CT, in large lesions, local mass-effect may also be appreciated 1-6.
Uncommonly, and as aforementioned, there may be additional involvement of the adjacent internal and external capsules, the brainstem (especially the midbrain), the periventricular white matter, the cortex (especially the medial temporal lobes), and the leptomeninges (see leptomeningeal enhancement) 1-6. Particular involvement of the internal and external capsules, visualized as linear T2-weighted regions of high signal, has emerged as a sign that may be unique to EEE compared to other viral encephalitides, and has been described as the ‘parentheses sign’ 5.
In regards to other MR sequences, all of these lesions regardless of location may demonstrate low signal on T1 with variable contrast enhancement, and usually demonstrate iso-intense diffusion signal on DWI 2,6.
Treatment and prognosis
There is no specific antiviral treatment available for EEE, and thus supportive management (e.g. anti-epileptics, corticosteroids) is encouraged 1. Mortality is thought to be between 50-75%, however surviving infection with EEEV does confers lifelong immunity 1,3.
History and etymology
EEE was first recognized in the United States of America in horses from Virginia, Delaware, New Jersey, and Maryland during an equine epizootic in 1933 7,8. The first human cases followed during a 1938 outbreak in Massachusetts, when there were 25 deaths from 34 cases 7.
Other infectious causes that can cause a similar imaging pattern include the Flavivirus encephalitides:
Furthermore, other causes of increased T2 signal in the basal ganglia should also be considered, such as:
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- 2. Badar Z, Thakur D, Hameedi A. CT and MRI findings in a pediatric case of eastern equine encephalitis. Pediatric Infectious Disease. 30;6(2):66-71.
- 3. Ketai L, Alrahji AA, Hart B, Enria D, Mettler F. Radiologic manifestations of potential bioterrorist agents of infection. AJR. American journal of roentgenology. 180 (3): 565-75. doi:10.2214/ajr.180.3.1800565 - Pubmed
- 4. Babi MA, Raleigh T, Shapiro RE, McSherry J, Applebee A. MRI and encephalography in fatal eastern equine encephalitis. Neurology. 83 (16): 1483. doi:10.1212/WNL.0000000000000876 - Pubmed
- 5. Nickerson JP, Kannabiran S, Burbank HN. MRI findings in eastern equine encephalitis: the "parenthesis" sign. Clinical imaging. 40 (2): 222-3. doi:10.1016/j.clinimag.2015.10.012 - Pubmed
- 6. Golomb MR, Durand ML, Schaefer PW, McDonald CT, Maia M, Schwamm LH. A case of immunotherapy-responsive eastern equine encephalitis with diffusion-weighted imaging. Neurology. 56 (3): 420-1. Pubmed
- 7. Armstrong PM, Andreadis TG. Eastern equine encephalitis virus--old enemy, new threat. The New England journal of medicine. 368 (18): 1670-3. doi:10.1056/NEJMp1213696 - Pubmed
- 8. Rutledge CR. Eastern Equine Encephalitis. InEncyclopedia of Entomology 2008 (pp. 1265-1268). Springer Netherlands.
Related Radiopaedia articles
Infections of the central nervous system
- classification by etiology
- Eastern equine encephalitis
- enterovirus rhombencephalitis
- flavivirus encephalitis
- herpes virus family
- 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)
- neurobrucellosis (Brucella sp.)
- 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)
- Creutzfeldt-Jakob disease (sporadic, variant, familial, and iatrogenic)
- fatal familial insomnia
- Gerstmann-Straussler-Scheinker disease
- variably protease-sensitive prionopathy
- others or those with possible infectious etiologies
- classification by location
- classification by etiology