Encephalitis - Symptoms, Causes, Images, and Treatment Options

Diseases

Highlights & Basics

Key Highlights

Encephalitis is a pathologic state of brain parenchymal inflammation leading to an altered state of consciousness or focal neurologic signs.
It is a serious, complex, and potentially fatal disorder with both infectious and noninfectious causes.
Patients present with acute to subacute onset of altered mental status; typical features include fever, headache, seizures, and focal neurologic signs.
Initial investigations should always include blood cultures, neuroimaging (preferably magnetic resonance imaging), and cerebrospinal fluid analysis (unless contraindicated).
Intravenous acyclovir should be administered as soon as possible in all cases of suspected viral encephalitis.

Noncontrast head CT of a patient with HSV encephalitis: shows subtle hypodensities involving the left insular region. Some blurring of gray-white margins and sulcal effacement over the left temporal region is discernible

From the personal collection of Catalina C. Ionita, MD; used with permission

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Definition

Epidemiology

Etiology

Pathophysiology

Images

Noncontrast head CT of a patient with HSV encephalitis: shows subtle hypodensities involving the left insular region. Some blurring of gray-white margins and sulcal effacement over the left temporal region is discernible
The first 5 images are FLAIR images of patient with varicella zoster virus meningoencephalitis showing white and gray matter hyperintensities. The last image is T1 image with contrast showing parenchymal and diffuse leptomeningeal enhancement
Series of MRI images of brain of patient with acute disseminated encephalomyelitis: hyperintense lesions of fluid attenuated inversion recovery (FLAIR) involving the left cerebellar peduncle
Series of MRI images of brain of patient with acute disseminated encephalomyelitis: asymmetric "fluffy" lesions over the bilateral ventricular horns and thalami
Series of MRI images of brain of patient with acute disseminated encephalomyelitis: periventricular regions
Series of MRI images of brain of patient with acute disseminated encephalomyelitis: centrum semiovale
Series of MRI images of brain of patient with acute disseminated encephalomyelitis: T1 post-gadolinium enhanced image shows ring enhancement around a lesion in the right centrum semiovale region and faint diffuse enhancement just above this area
Series of MRI images of brain of patient with acute disseminated encephalomyelitis: diffusion-weighted image from the same patient shows high signal intensity in the same area, and this correlates with increased (bright on ADC maps) diffusion
MRI brain: the pulvinar sign (a term referencing bilateral pulvinar hyperintensity) in a patient with Creutzfeldt-Jakob disease on diffusion-weighted images
MRI brain: cortical ribboning in a patient with Creutzfeldt-Jakob disease on diffusion-weighted images
Biopsy of brain from right temporal lobe: the classic H&E stain shows evidence of patchy but extensive inflammatory infiltrate of small mononuclear cells (lymphocytes) in the brain parenchyma, predominantly around the blood vessel walls. PCR studies of the biopsy sample were positive for EBV infection
Biopsy of brain from right temporal lobe: a close-up view of a blood vessel with its surrounding marked inflammatory infiltrate is also seen. PCR studies of the biopsy sample were positive for EBV infection.
Biopsy from hippocampus of patient with rabies showing 2 neurons with eosinophilic Negri bodies (red arrows). These are found in areas, often with little inflammatory reaction. The blue arrows highlight microglial cells
Biopsy from hippocampus of patient with rabies showing neurons with eosinophilic Negri bodies (red arrow). The blue arrow highlights a collection of satelliting oligodendrocytes
Biopsy from hippocampus of patient with rabies showing a neuron with an eosinophilic cytoplasmic Negri body (red arrow). The blue arrow highlights a collection of microglial cells next to a blood vessel
Biopsy from the brainstem of HIV patient with CMV encephalitis. The ependymal lining shows enlarged cells (arrows) with intranuclear inclusions
Biopsy from cortex of neonate with CMV encephalitis showing enlarged cells (arrows) with intranuclear inclusions. The top arrow points to a neuron with two nuclei each with a nuclear inclusion
Biopsy from brain of immunocompromised patient with cryptococcal meningitis at low magnification. The meninges are expanded (arrow), but the cortex is histologically relatively uninvolved
Biopsy from brain of immunocompromised patient with cryptococcal meningitis showing the meninges with round translucent cryptococcal organism (red arrow) as well as a budding yeast (blue arrow)
Biopsy from brain of an immunocompetent patient with cryptococcal meningitis at low magnification showing the meninges with inflammation (red arrow)
Biopsy from brain of immunocompetent patient with cryptococcal meningitis showing the meninges with inflammatory cells and Cryptococcus
Biopsy from meninges of patient with cryptococcal meningitis stained with mucicarmine, demonstrating fungal organisms, particularly in giant cells
Coronal slice of the brain of patient with cryptococcal meningoencephalitis showing classical appearance of "soap bubble" structures in the basal ganglia (arrows) resulting from the cryptococcal expansion of Virchow-Robbin spaces around the lenticulostriate vessels
Biopsy from basal ganglia of patient with cryptococcal meningoencephalitis showing cryptococcal (blue arrow) expansion of Virchow-Robbin spaces around a lenticulostriate vessel (red arrow)
Gross autopsy of brain of patient with cryptococcal meningitis showing the surface with a "glazed" look. There is also a shunt present
Biopsy from brain of patient with subacute HIV leukoencephalitis showing the distinctive multinucleated cell (red arrow) in the white matter next to inflammatory cells in the Virchow-Robin space
Biopsy from brain of patient with subacute HIV leukoencephalitis showing the distinctive multinucleated cell (red arrow) in the white matter
Biopsy from brain of patient with subacute HIV encephalitis showing the distinctive multinucleated giant cell (red arrow), which contains the HIV virus. The multinucleated giant cells are from histiocyte/macrophage lineage. There is also associated astrocytosis
Coronal slice of the brain of HIV patient in his 30s. He had subacute HIV encephalitis involving both the white matter and gray matter diffusely. The ventricles were enlarged reflecting white matter and cortical loss
Coronal slice of the brain of HIV patient with toxoplasmosis, showing infection of the periventricular superior part of the left thalamus
Biopsy of the brain of an HIV patient with toxoplasmosis, showing encysted bradyzoites (red arrow) and tachyzoites (blue arrow)
Biopsy of HIV patient with toxoplasmosis, showing both pieces of cellular debris and tachyzoites. The tachyzoites are round, smooth, and hard to identify without antibody staining (see next image)
Biopsy of HIV patient with toxoplasmosis, with the tachyzoites identified using immunohistochemistry
Biopsy of the posterior thalamus of patient with Creutzfeldt-Jakob disease showing the spongiform changes

Citations

Key Articles

Venkatesan A, Tunkel AR, Bloch KC, et al. Case definitions, diagnostic algorithms, and priorities in encephalitis: consensus statement of the International Encephalitis Consortium. Clin Infect Dis. 2013 Oct;57(8):1114-28.[Abstract][Full Text]
Solomon T, Michael BD, Smith PE, et al. Management of suspected viral encephalitis in adults--Association of British Neurologists and British Infection Association National Guidelines. J Infect. 2012 Apr;64(4):347-73.[Abstract][Full Text]
Miller JM, Binnicker MJ, Campbell S, et al. Guide to utilization of the microbiology laboratory for diagnosis of infectious diseases: 2024 update by the Infectious Diseases Society of America (IDSA) and the American Society for Microbiology (ASM). Clin Infect Dis. 2024 Mar 5:ciae104.[Abstract][Full Text]
Kneen R, Michael BD, Menson E, et al. Management of suspected viral encephalitis in children - Association of British Neurologists and British Paediatric Allergy, Immunology and Infection Group national guidelines. J Infect. 2012 May;64(5):449-77.[Abstract][Full Text]

Other Online Resources

CDC: Division of vector-borne diseases (DVBD)
CDC: immunization schedules
CDC: Influenza (Flu)

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