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|ICD-10||A83-A86, B941, G05|
Encephalitis is a brain disorder due to an acute inflammation of the brain. It can be caused by a bacterial infection such as bacterial meningitis spreading directly to the brain (primary encephalitis), or may be a complication of a current infectious disease like rabies or syphilis (secondary encephalitis). 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 may also cause encephalitis. Bartonella henselae can also lead to this. Brain damage occurs as the inflamed brain pushes against the skull, and can lead to death.
Patients with encephalitis suffer from fever, headache and photophobia with weakness and seizures also common. Less commonly, stiffness of the neck can occur with rare cases of patients also suffering from stiffness of the limbs, slowness in movement and clumsiness depending on which specific part of the brain is involved. The symptoms of encephalitis are caused by the brain's defense mechanisms activating to get rid of the infection. Other symptoms can include drowsiness and coughing.
- Main article: Viral 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 most common causes of acute viral encephalitis are rabies virus, Herpes simplex, poliovirus, measles virus, and JC virus. Other causes include infection by flaviviruses such as Japanese encephalitis virus, St. Louis encephalitis virus or West Nile virus, or by Togaviridae such as Eastern equine encephalitis virus (EEE virus), Western equine encephalitis virus (WEE virus) or Venezuelan equine encephalitis virus (VEE virus). Henipaviruses; Hendra (HeV) and Nipah (NiV), are also known to cause viral encephalitis.
Bacterial and other
It can be caused by a bacterial infection, such as bacterial meningitis, spreading directly to the brain (primary encephalitis), or may be a complication of a current infectious disease syphilis (secondary encephalitis). 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 and/or Bartonella henselae may also cause encephalitis. Cryptococcus neoformans is notorious for causing fungal encephalitis in the immunocompromised. Streptococci, staphylococci and certain Gram-negative bacilli cause cerebritis prior to the formation of a brain abscess.
Limbic system encephalitis
In a large number of cases, called limbic encephalitis, the pathogens responsible for encephalitis attack primarily the limbic system (a collection of structures at the base of the brain responsible for emotions and many other basic functions).
It has recently been recognised that there are types of encephalitis resulting from an attack of the brain by the body's immune system. These autoimmune conditions include but are not limited to VGKC antibody associated encephalitis, Anti-GAD antibody associated encephalitis, NMDA receptor antibody associated encephalitis, and Hashimoto's encephalitis.
The majority of patients with autoimmune encephalitis do not harbor a tumor and the etiology of the disease in these patients is less clear, often leading to a delayed diagnosis.
- Main article: Encephalitis lethargica
Encephalitis lethargica is an atypical form of encephalitis which caused an epidemic from 1918 to 1930. Those who survived sank into a semi-conscious state that lasted for decades. Neurologist Oliver Sacks used the Parkinson's drug L-DOPA to revive those still alive in the late 1960s.
There have been only a small number of isolated cases in the years since, though in recent years a few patients have shown very similar symptoms. The cause is now thought to be either a bacterial agent or an autoimmune response following infection.
The incidence 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. As of 2010 it caused about 120,000 deaths, down from 144,000 in 1990.
Herpes simplex encephalitis has an incidence of 2–4 per million population per year.
Adult patients with encephalitis present with acute onset of fever, headache, confusion, and sometimes seizures. Younger children or infants may present irritability, anorexia and fever.
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 meningeoncephalitis. Examination of the cerebrospinal fluid obtained by a lumbar puncture procedure usually reveals increased amounts of protein and white blood cells with normal glucose, though in a significant percentage of patients, the cerebrospinal fluid may be normal. CT scan often is not helpful, as cerebral abscess is uncommon. Cerebral abscess is more common in patients with meningitis than encephalitis. Bleeding is also uncommon except in patients with herpes simplex type 1 encephalitis. Magnetic resonance imaging offers better resolution. In patients with herpes simplex encephalitis, electroencephalograph may show sharp waves in one or both of the temporal lobes. Lumbar puncture procedure is performed only after the possibility of prominent brain swelling is excluded by a CT scan examination. Diagnosis is often made with detection of antibodies in the cerebrospinal fluid against a specific viral agent (such as herpes simplex virus) or by polymerase chain reaction that amplifies the RNA or DNA of the virus responsible (such as varicella zoster virus).
Treatment is usually symptomatic. Reliably tested specific antiviral agents are few in number (e.g. acyclovir for herpes simplex virus) and are used with limited success in treatment of viral infection, with the exception of herpes simplex encephalitis. In patients who are very sick, supportive treatment, such as mechanical ventilation, is equally important. Corticosteroids (e.g., methylprednisolone) are used to reduce brain swelling and inflammation. Sedatives may be needed for irritability or restlessness. For Mycoplasma infection, parenteral tetracycline is given. Encephalitis due to Toxoplasma is treated by giving a combination of pyrimethamine and sulphadimidine.
Post-infectious encephalomyelitis complicating small pox vaccination is totally avoidable now as small pox is now eradicated. Contraindication to Pertussis immunisation should be observed in patients with encephalitis. An immunodeficient patient who has had contact with chicken pox virus should be given prophylaxis with hyperimmune zoster immunoglobulin.
- Awakenings Movie (1990)
- California encephalitis virus
- Equine Encephalitis
- Granulomatous amoebic encephalitis
- Herpes simplex
- Herpes zoster
- Infectious disorders
- La Crosse encephalitis
- Limbic encephalitis
- Japanese Encephalitis
- Murray Valley encephalitis virus
- Powassan encephalitis
- Rasmussen's encephalitis
- St. Louis Encephalitis
- Tick-borne meningoencephalitis
- Jmor, F, Emsley HC, Fischer M et al. (October 2008). The incidence of acute encephalitis syndrome in Western industrialised and tropical countries. Virology Journal 5 (134): 134.
- Lozano, R (2012 Dec 15). Global and regional mortality from 235 causes of death for 20 age groups in 1990 and 2010: a systematic analysis for the Global Burden of Disease Study 2010. Lancet 380 (9859): 2095–128.
- Rozenberg, F, Deback C, Agut H (June 2011). Herpes simplex encephalitis: from virus to therapy. Infectious Disorders Drug Targets 11 (3): 235–250.
- Tick-borne Encephalitis. URL accessed on 2 April 2013.
- Japanese encephalitis. URL accessed on 2 April 2013.
- http://www.encephalitis.info resource providing evidence based support to people affected and professionals
- WHO: Encephalitis
- eMedicine-1 and eMedicine-2 Information on the causes, symptoms, and treatment options for Encephalitis.
- Encephalitis Global, Inc. Website offering information and support to encephalitis survivors, caregivers and loved ones. Encephalitis Global Inc. is a USA 501(c)(3) public charity; annual FACES Encephalitis Conference information available at the website.
- A case study of a Limbic Encephalitis patient
- A website about Garrett Taylor a 2 year old who lost his life after a battle with encephalitis
- Meningitis and Encephalitis Information Page at NINDS
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