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Encephalitis
Encephalitis
is inflammation of the brain.[5] Severity is variable.[1] Symptoms may include headache, fever, confusion, a stiff neck, and vomiting.[1] Complications may include seizures, hallucinations, trouble speaking, memory problems, and problems with hearing.[1] Causes of encephalitis include viruses such as herpes simplex virus or rabies, bacteria, fungus, or parasites.[2][1] Other causes include autoimmune diseases and certain medication.[2] In many cases the cause remains unknown.[2] Risk factors include a weak immune system.[2] Diagnosis is typically based on symptoms and supported by blood tests, medical imaging, and analysis of cerebrospinal fluid.[2] Certain types are preventable with vaccines.[5] Treatment may include, antiviral medication (such as acyclovir), anticonvulsants, and corticosteroids.[1] Treatment generally takes place in hospital.[1] Some people require artificial respiration.[1] Once the immediate problem is under control, rehabilitation may be required.[2] In 2015, encephalitis was estimated to have affected 4.3 million people and resulted in 150,000 deaths worldwide.[3][4]

Contents

1 Signs and symptoms 2 Cause

2.1 Viral 2.2 Bacterial and other 2.3 Limbic encephalitis 2.4 Autoimmune encephalitis 2.5 Encephalitis
Encephalitis
lethargica

3 Diagnosis 4 Prevention 5 Treatment 6 Prognosis 7 Epidemiology 8 Terminology 9 See also 10 References 11 Further reading 12 External links

Signs and symptoms[edit] Adult patients with encephalitis present with acute onset of fever, headache, confusion, and sometimes seizures. Younger children or infants may present irritability, poor appetite and fever.[6] Neurological examinations usually reveal a drowsy or confused patient. Stiff neck, due to the irritation of the meninges covering the brain, indicates that the patient has either meningitis or meningoencephalitis.[7] Cause[edit]

Rabies
Rabies
virus

Viral[edit] Main articles: Viral encephalitis and Herpesviral encephalitis Viral encephalitis can occur either as a direct effect of an acute infection, or as one of the sequelae of a latent infection. The majority of viral cases of encephalitis have an unknown cause, however the most common identifiable cause of viral encephalitis is from herpes simplex infection.[8] Other causes of acute viral encephalitis are rabies virus, poliovirus, and measles virus.[9] Additional possible viral causes are arbovirus (St. Louis encephalitis, West Nile encephalitis
West Nile encephalitis
virus), bunyavirus (La Crosse strain), arenavirus (lymphocytic choriomeningitis virus) and reovirus (Colorado tick virus).[10] The Powassan virus is a rare cause of encephalitis. Bacterial and other[edit] It can be caused by a bacterial infection, such as bacterial meningitis,[11] or may be a complication of a current infectious disease syphilis (secondary encephalitis).[12] Certain parasitic or protozoal infestations, such as toxoplasmosis, malaria, or primary amoebic meningoencephalitis, can also cause encephalitis in people with compromised immune systems. Lyme disease or Bartonella henselae
Bartonella henselae
may also cause encephalitis.[citation needed] Other bacterial pathogens, like Mycoplasma
Mycoplasma
and those causing rickettsial disease, cause inflammation of the meninges and consequently encephalitis. A non-infectious cause includes acute disseminated encephalitis which is demyelinated.[13] Limbic encephalitis[edit] Limbic encephalitis refers to inflammatory disease confined to the limbic system of the brain. The clinical presentation often includes disorientation, disinhibition, memory loss, seizures, and behavioral anomalies. MRI
MRI
imaging reveals T2 hyperintensity in the structures of the medial temporal lobes, and in some cases, other limbic structures. Some cases of limbic encephalitis are of autoimmune origin.[14] Autoimmune encephalitis[edit] Main article: Autoimmune encephalitis Autoimmune encephalitis
Autoimmune encephalitis
signs can include catatonia, psychosis, abnormal movements, and autonomic dysregulation. Antibody-mediated anti-N-methyl-D-aspartate-receptor encephalitis and Rasmussen encephalitis are examples of autoimmune encephalitis.[15] Anti-N-methyl-D-aspartate-receptor encephalitis is the most common autoimmune form, and is accompanied by ovarian teratoma in 58 percent of affected women 18-45 years of age.[16] Encephalitis
Encephalitis
lethargica[edit] Main article: Encephalitis
Encephalitis
lethargica Encephalitis lethargica
Encephalitis lethargica
is identified by high fever, headache, delayed physical response, and lethargy. Individuals can exhibit upper body weakness, muscular pains, and tremors, though the cause of encephalitis lethargica is not currently known. From 1917 to 1928, an epidemic of encephalitis lethargica occurred worldwide.[17] Diagnosis[edit]

Spinal tap on a newborn

People should only be diagnosed with encephalitis if they have a decreased or altered level of consciousness, lethargy, or personality change for at least twenty-four hours without any other explainable cause.[18] Diagnosing encephalitis is done via a variety of tests:[19]

Brain scan, done by MRI, can determine inflammation and differentiate from other possible causes. EEG, in monitoring brain activity, encephalitis will produce abnormal signal. Lumbar puncture
Lumbar puncture
(spinal tap), this helps determine via a test using the cerebral-spinal fluid, obtained from the lumbar region. Blood test Urine analysis Polymerase chain reaction
Polymerase chain reaction
(PCR) testing of the cerebrospinal fluid, to detect the presence of viral DNA
DNA
which is a sign of viral encephalitis.[20]

Prevention[edit] Vaccination is available against tick-borne[21] and Japanese encephalitis[22] and should be considered for at-risk individuals. Post-infectious encephalomyelitis complicating smallpox vaccination is avoidable, for all intents and purposes, as smallpox is nearly eradicated.[23] Contraindication to Pertussis
Pertussis
immunization should be observed in patients with encephalitis.[24] Treatment[edit] Treatment (which is based on supportive care) is as follows:[25]

Antiviral medications (if virus is cause) Antibiotics, (if bacteria is cause) Steroids
Steroids
are used to reduce brain swelling Sedatives for restlessness Acetaminophen
Acetaminophen
for fever Occupational and physical therapy (if brain is affected post-infection)

Pyrimethamine-based maintenance therapy is often used to treat Toxoplasmic Encephalitis
Encephalitis
(TE), which is caused by Toxoplasma gondii and can be life-threatening for people with weak immune systems.[26] The use of highly active antiretroviral therapy (HAART), in conjunction with the established pyrimethamine-based maintenance therapy, decreases the chance of relapse in patients with HIV and TE from approximately 18% to 11%.[26] This is a significant difference as relapse may impact the severity and prognosis of disease and result in an increase in healthcare expenditure.[26] Prognosis[edit] Identification of poor prognostic factors include thrombocytopenia, cerebral edema, status epilepticus, and thrombocytopenia.[27] In contrast, a normal encephalogram at the early stages of diagnosis is associated with high rates of survival.[27] Epidemiology[edit]

Encephalitis
Encephalitis
deaths per million persons in 2012

  0-0   1-1   2-2   3-4   5-9   10-14   15-24   25-45

The number of new cases a year of acute encephalitis in Western countries is 7.4 cases per 100,000 population per year. In tropical countries, the incidence is 6.34 per 100,000 per year.[28] The number of cases of encephalitis has not changed much over time, with about 250,000 cases a year from 2005 to 2015 in the US. Approximately seven per 100,000 people were hospitalized for encephalitis in the US during this time.[27] In 2015, encephalitis was estimated to have affected 4.3 million people and resulted in 150,000 deaths worldwide.[4][3] Herpes simplex encephalitis
Herpes simplex encephalitis
has an incidence of 2–4 per million population per year.[29] Terminology[edit] Encephalitis
Encephalitis
with meningitis is known as meningoencephalitis. While encephalitis with involvement of the spinal cord is known as encephalomyelitis.[2] The word is from Ancient Greek
Ancient Greek
ἐγκέφαλος, enképhalos "brain",[30] composed of ἐν, en, "in" and κεφαλή, kephalé, "head", and the medical suffix -itis "inflammation".[31] See also[edit]

Rasmussen's encephalitis Bickerstaff's encephalitis La Crosse encephalitis Wernicke's encephalopathy Meningitis Cerebritis Encephalomyelitis Zika Virus Naegleriasis
Naegleriasis
(primary amoebic meningoencephalitis/PAM) World Encephalitis
Encephalitis
Day

References[edit]

^ a b c d e f g h i j k l " Meningitis
Meningitis
and Encephalitis
Encephalitis
Information Page". NINDS. Archived from the original on 29 October 2017. Retrieved 29 October 2017.  ^ a b c d e f g h i j " Meningitis
Meningitis
and Encephalitis
Encephalitis
Fact Sheet". National Institute of Neurological Disorders and Stroke. Archived from the original on 29 October 2017. Retrieved 29 October 2017.  ^ a b c GBD 2015 Disease and Injury Incidence and Prevalence Collaborators (8 October 2016). "Global, regional, and national incidence, prevalence, and years lived with disability for 310 diseases and injuries, 1990–2015: a systematic analysis for the Global Burden of Disease Study 2015". Lancet. 388 (10053): 1545–1602. doi:10.1016/S0140-6736(16)31678-6. PMC 5055577 . PMID 27733282.  ^ a b c GBD 2015 Mortality and Causes of Death Collaborators (8 October 2016). "Global, regional, and national life expectancy, all-cause mortality, and cause-specific mortality for 249 causes of death, 1980–2015: a systematic analysis for the Global Burden of Disease Study 2015". Lancet. 388 (10053): 1459–1544. doi:10.1016/S0140-6736(16)31012-1. PMC 5388903 . PMID 27733281.  ^ a b "Encephalitis". NHS Choices. 2016. Archived from the original on 22 September 2017. Retrieved 29 October 2017.  ^ "Symptoms of encephalitis". NHS. Archived from the original on 5 January 2015. Retrieved 5 January 2015.  ^ Shmaefsky, Brian; Babcock, Hilary (2010-01-01). Meningitis. Infobase Publishing. ISBN 9781438132167. Archived from the original on 2015-10-30.  ^ Roos, Karen L.; Tyler, Kenneth L. (2015). "Meningitis, Encephalitis, Brain Abscess, and Empyema". Harrison's Principles of Internal Medicine (19 ed.). New York, NY: McGraw-Hill Education. ISBN 9780071802154.  ^ Fisher, D. L.; Defres, S.; Solomon, T. (2015). "Measles-induced encephalitis". QJM. 108 (3): 177–182. doi:10.1093/qjmed/hcu113. PMID 24865261.  ^ Kennedy, P. G. E. (2004-03-01). "Viral Encephalitis: Causes, Differential Diagnosis, and Management". Journal of Neurology, Neurosurgery & Psychiatry. 75 (Suppl 1): i10–5. doi:10.1136/jnnp.2003.034280. PMC 1765650 . PMID 14978145. Archived from the original on 2015-10-02.  ^ Ashar, Bimal H.; Miller, Redonda G.; Sisson, Stephen D. (2012-01-01). Johns Hopkins Internal Medicine Board Review: Certification and Recertification. Elsevier Health Sciences. ISBN 1455706922. Archived from the original on 2015-11-29.  ^ Hama, Kiwa; Ishiguchi, Hiroshi; Tuji, Tomikimi; Miwa, Hideto; Kondo, Tomoyoshi (2008-01-01). "Neurosyphilis with Mesiotemporal Magnetic Resonance Imaging Abnormalities". Internal Medicine. 47 (20): 1813–7. doi:10.2169/internalmedicine.47.0983. PMID 18854635. Archived from the original on 2016-01-31.  ^ "Encephalitis: Practice Essentials, Background, Pathophysiology". Archived from the original on 2015-08-03.  ^ Larner, A. J. (2013-05-02). Neuropsychological Neurology: The Neurocognitive Impairments of Neurological Disorders. Cambridge University Press. ISBN 9781107607606. Archived from the original on 2015-10-30.  ^ Armangue, Thaís; Petit-Pedrol, Mar; Dalmau, Josep (2012-11-01). "Autoimmune Encephalitis
Encephalitis
in Children". Journal of child neurology. 27 (11): 1460–9. doi:10.1177/0883073812448838. PMC 3705178 . PMID 22935553.  ^ Ropper, Allan H.; Dalmau, Josep; Graus, Francesc (March 2018). "Antibody-Mediated Encephalitis". New England Journal of Medicine. 378 (9): 840–851. doi:10.1056/NEJMra1708712.  ^ encephalitis_lethargica at NINDS ^ Venkatesan, A; Tunkel, AR; Bloch, KC; Lauring, AS; Sejvar, J; Bitnun, A; Stahl, JP; Mailles, A; Drebot, M; Rupprecht, CE; Yoder, J; Cope, JR; Wilson, MR; Whitley, RJ; Sullivan, J; Granerod, J; Jones, C; Eastwood, K; Ward, KN; Durrheim, DN; Solbrig, MV; Guo-Dong, L; Glaser, CA; International Encephalitis, Consortium. (October 2013). "Case definitions, diagnostic algorithms, and priorities in encephalitis: consensus statement of the international encephalitis consortium". Clinical Infectious Diseases. 57 (8): 1114–28. doi:10.1093/cid/cit458. PMC 3783060 . PMID 23861361.  ^ "Encephalitis: Diagnosis". NHS Choices. Archived from the original on 2015-07-16. Retrieved 2015-08-05.  ^ Kneen R, Michael BD, Menson E, Mehta B, Easton A, Hemingway C, Klapper PE, Vincent A, Lim M, Carrol E, Solomon T (2012). "Management of suspected viral encephalitis in children - Association of British Neurologists and British Paediatric Allergy, Immunology and Infection Group national guidelines". J. Infect. 64 (5): 449–77. doi:10.1016/j.jinf.2011.11.013. PMID 22120594.  ^ "Tick-borne Encephalitis: Vaccine". International travel and health. World Health Organization. Archived from the original on 3 December 2013. Retrieved 2 April 2013.  ^ "Japanese encephalitis". Immunization, Vaccines and Biologicals. World Health Organization. Archived from the original on 3 December 2013. Retrieved 2 April 2013.  ^ "CDC Media Statement on Newly Discovered Smallpox Specimens". www.cdc.gov. Archived from the original on 2016-05-20. Retrieved 2016-05-19.  ^ "Contraindications and Precautions to Commonly Used Vaccines in Adults". Vaccines. Center for Disease Control. Archived from the original on 2015-08-23. Retrieved 2015-08-05.  ^ MedlinePlus Encyclopedia Encephalitis ^ a b c Connolly MP, Goodwin E, Schey C, Zummo J (2017). "Toxoplasmic encephalitis relapse rates with pyrimethamine-based therapy: systematic review and meta-analysis". Pathogens and global health. 111 (1): 31–44. doi:10.1080/20477724.2016.1273597. PMC 5375610 . PMID 28090819.  ^ a b c Venkatesan, A (June 2015). "Epidemiology and outcomes of acute encephalitis". Current Opinion in Neurology. 28 (3): 277–82. doi:10.1097/WCO.0000000000000199. PMID 25887770.  ^ Jmor F, Emsley HC, et al. (October 2008). "The incidence of acute encephalitis syndrome in Western industrialised and tropical countries". Virology Journal. 5 (134): 134. doi:10.1186/1743-422X-5-134. PMC 2583971 . PMID 18973679.  ^ Rozenberg, F; Deback C; Agut H (June 2011). "Herpes simplex encephalitis: from virus to therapy". Infectious Disorders Drug Targets. 11 (3): 235–250. doi:10.2174/187152611795768088. PMID 21488834.  ^ "Woodhouse's English-Greek Dictionary" (in German). The University of Chicago Library. Retrieved 2013-01-10.  ^ The word seems to have had a meaning of “lithic imitation of the human brain” at first, according to the Trésor de la langue française informatisé (cf. the article on “encéphalite” Archived 2017-11-05 at the Wayback Machine.). The first use in the medical sense is attested from the early 19th century in French (J. Capuron, Nouveau dictionnaire de médecine, chirurgie…, 1806), and from 1843 in English respectively (cf. the article “encephalitis” in the Online Etymology Dictionary). Retrieved 11 March 2017.

Further reading[edit]

Steiner, I.; Budka, H.; Chaudhuri, A.; Koskiniemi, M.; Sainio, K.; Salonen, O.; Kennedy, P. G. E. (1 May 2005). "Viral encephalitis: a review of diagnostic methods and guidelines for management". European Journal of Neurology. 12 (5): 331–343. doi:10.1111/j.1468-1331.2005.01126.x. PMID 15804262.  Basavaraju, Sridhar V.; Kuehnert, Matthew J.; Zaki, Sherif R.; Sejvar, James J. (September 2014). " Encephalitis
Encephalitis
Caused by Pathogens Transmitted through Organ Transplants, United States, 2002–2013". Emerging Infectious Diseases. 20 (9): 1443–51. doi:10.3201/eid2009.131332. PMC 4178385 . PMID 25148201.  National Center for Biotechnology Information. "Encephalitis". PubMed Health. National Library of Medicine. Retrieved 2015-08-05. 

External links[edit]

WHO: Viral Encephalitis

Classification

V · T · D

ICD-10: A83-A86, B94.1, G05 ICD-9-CM: 323 MeSH: D004660 DiseasesDB: 22543

External resources

MedlinePlus: 001415 eMedicine: emerg/163

v t e

Inflammation

Acute

Plasma-derived mediators

Bradykinin complement

C3 C5a MAC

coagulation

Factor XII Plasmin Thrombin

Cell-derived mediators

preformed:

Lysosome granules biogenic amines

Histamine Serotonin

synthesized on demand:

cytokines

IFN-γ IL-8 TNF-α IL-1

eicosanoids

Leukotriene B4 Prostaglandins

Nitric oxide Kinins

Chronic

Macrophage Epithelioid cell Giant cell Granuloma

Processes

Traditional

Rubor Calor Tumor Dolor Functio laesa

Modern

Acute-phase reaction/Fever Vasodilation Increased vascular permeability Exudate Leukocyte extravasation Chemotaxis

Specific locations

Nervous

CNS

Encephalitis Myelitis

Meningitis

Arachnoiditis

PNS

Neuritis

eye

Dacryoadenitis Scleritis Episcleritis Keratitis chorioretinitis Retinitis Chorioretinitis Blepharitis Conjunctivitis Uveitis

ear

Otitis externa Otitis media Labyrinthitis Mastoiditis

Cardiovascular

Carditis

Endocarditis Myocarditis Pericarditis

Vasculitis

Arteritis Phlebitis Capillaritis

Respiratory

upper

Sinusitis Rhinitis Pharyngitis Laryngitis

lower

Tracheitis Bronchitis Bronchiolitis Pneumonitis Pleuritis

Mediastinitis

Digestive

mouth

Stomatitis Gingivitis Gingivostomatitis Glossitis Tonsillitis Sialadenitis/Parotitis Cheilitis Pulpitis Gnathitis

tract

Esophagitis Gastritis Gastroenteritis Enteritis Colitis Enterocolitis Duodenitis Ileitis Caecitis Appendicitis Proctitis

accessory

Hepatitis Ascending cholangitis Cholecystitis Pancreatitis Peritonitis

Integumentary

Dermatitis

Folliculitis

Cellulitis Hidradenitis

Musculoskeletal

Arthritis Dermatomyositis soft tissue

Myositis Synovitis/Tenosynovitis Bursitis Enthesitis Fasciitis Capsulitis Epicondylitis Tendinitis Panniculitis

Osteochondritis: Osteitis/Osteomyelitis

Spondylitis Periostitis

Chondritis

Urinary

Nephritis

Glomerulonephritis Pyelonephritis

Ureteritis Cystitis Urethritis

Reproductive

female

Oophoritis Salpingitis Endometritis Parametritis Cervicitis Vaginitis Vulvitis Mastitis

male

Orchitis Epididymitis Prostatitis Seminal vesiculitis Balanitis Posthitis Balanoposthitis

pregnancy/newborn

Chorioamnionitis Funisitis Omphalitis

Endocrine

Insulitis Hypophysitis Thyroiditis Parathyroiditis Adrenalitis

Lymphatic

Lymphangitis Lymphadenitis

v t e

Diseases of the nervous system, primarily CNS (G04–G47, 323–349)

Inflammation

Brain

Encephalitis

Viral encephalitis Herpesviral encephalitis Limbic encephalitis Encephalitis
Encephalitis
lethargica

Cavernous sinus thrombosis Brain abscess

Amoebic

Spinal cord

Myelitis: Poliomyelitis Demyelinating disease

Transverse myelitis

Tropical spastic paraparesis Epidural abscess

Both/either

Encephalomyelitis

Acute disseminated Myalgic

Meningoencephalitis

Brain/ encephalopathy

Degenerative

Extrapyramidal and movement disorders

Basal ganglia disease

Parkinsonism

PD Postencephalitic NMS

PKAN Tauopathy

PSP

Striatonigral degeneration Hemiballismus HD OA

Dyskinesia

Dystonia

Status dystonicus Spasmodic torticollis Meige's Blepharospasm

Athetosis Chorea

Choreoathetosis

Myoclonus

Myoclonic epilepsy

Akathisia

Tremor

Essential tremor Intention tremor

Restless legs Stiff person

Dementia

Tauopathy

Alzheimer's

Early-onset

Primary progressive aphasia

Frontotemporal dementia/Frontotemporal lobar degeneration

Pick's Dementia
Dementia
with Lewy bodies

Posterior cortical atrophy

Vascular dementia

Mitochondrial disease

Leigh disease

Demyelinating

autoimmune

Multiple sclerosis Neuromyelitis optica Schilder's disease

hereditary

Adrenoleukodystrophy Alexander Canavan Krabbe ML PMD VWM MFC CAMFAK syndrome

Central pontine myelinolysis Marchiafava–Bignami disease Alpers' disease

Episodic/ paroxysmal

Seizure/epilepsy

Focal Generalised Status epilepticus Myoclonic epilepsy

Headache

Migraine

Familial hemiplegic

Cluster Tension

Cerebrovascular

TIA

Amaurosis fugax Transient global amnesia Acute aphasia

Stroke

MCA ACA PCA Foville's Millard–Gubler Lateral medullary Medial medullary Weber's Lacunar stroke

Sleep disorders

Insomnia Hypersomnia Sleep apnea

Obstructive Congenital central hypoventilation syndrome

Narcolepsy Cataplexy Kleine–Levin Circadian rhythm sleep disorder

Advanced sleep phase disorder Delayed sleep phase disorder Non-24-hour sleep–wake disorder Jet lag

CSF

Intracranial hypertension

Hydrocephalus/NPH Choroid plexus papilloma Idiopathic intracranial hypertension

Cerebral edema Intracranial hypotension

Other

Brain herniation Reye's Hepatic encephalopathy Toxic encephalopathy Hashimoto's encephalopathy

Spinal cord/ myelopathy

Syringomyelia Syringobulbia Morvan's syndrome Vascular myelopathy

Foix–Alajouanine syndrome

Spinal cord
Spinal cord
compression

Both/either

Degenerative

SA

Friedreich's ataxia Ataxia-telangiectasia

MND

UMN only:

Primary lateral sclerosis Pseudobulbar palsy Hereditary spastic paraplegia

LMN only:

Distal hereditary motor neuronopathies Spinal muscular atrophies

SMA SMAX1 SMAX2 DSMA1 Congenital DSMA SMA-PCH SMA-LED SMA-PME

Progressive muscular atrophy Progressive bulbar palsy

Fazio–Londe Infantile progressive bulbar palsy

both:

Amyotrophic lateral sclerosis

Authority control

.