Encephalitis

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Encephalitis
Hsv encephalitis.jpg
MRI scan image shows high signal in the temporal lobes and right inferior frontal gyrus in someone with herpes simplex encephalitis.
Specialty Neurology, infectious disease
Symptoms Headache, fever, confusion, stiff neck, vomiting [1]
Complications Seizures, trouble speaking, memory problems, problems hearing [1]
DurationWeeks to months for recovery [1]
Types Herpes simplex, West Nile, rabies, Eastern equine, others [2]
CausesInfection, autoimmune, certain medication, unknown [2]
Diagnostic method Based on symptoms, supported by blood tests, medical imaging, analysis of cerebrospinal fluid [2]
Treatment Antiviral medication, anticonvulsants, corticosteroids, artificial respiration [1]
Prognosis Variable [1]
Frequency4.3 million (2015) [3]
Deaths150,000 (2015) [4]

Encephalitis is inflammation of the brain. [5] The severity can be variable with symptoms including reduction or alteration in consciousness, aphasia, headache, fever, confusion, a stiff neck, and vomiting. [1] [6] Complications may include seizures, hallucinations, trouble speaking, memory problems, and problems with hearing. [1]

Contents

Causes of encephalitis include viruses such as herpes simplex virus and rabies virus as well as bacteria, fungi, or parasites. [1] [2] Other causes include autoimmune diseases and certain medications. [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 medications (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]

Signs and symptoms

Adults with encephalitis present with acute onset of fever, headache, confusion, and sometimes seizures. Younger children or infants may present with irritability, poor appetite and fever. [7] Neurological examinations usually reveal a drowsy or confused person. Stiff neck, due to the irritation of the meninges covering the brain, indicates that the patient has either meningitis or meningoencephalitis. [8]

Limbic encephalitis

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 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. [9]

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. [10]

Cause

Rabies virus Rabies Virus EM PHIL 1876.JPG
Rabies virus

In 30%-40% of encephalitis cases, the etiology remains unknown. [11]

Viral

Viral infections are the usual cause of infectious encephalitis. [11] 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. [12] Other causes of acute viral encephalitis are rabies virus, poliovirus, and measles virus. [13]

Additional possible viral causes are arboviral flavivirus (St. Louis encephalitis, West Nile virus), bunyavirus (La Crosse strain), arenavirus (lymphocytic choriomeningitis virus), reovirus (Colorado tick virus), and henipavirus infections. [14] [15] The Powassan virus is a rare cause of encephalitis. [16]

Bacterial

It can be caused by a bacterial infection, such as bacterial meningitis, [17] or may be a complication of a current infectious disease such as syphilis (secondary encephalitis). [18]

Other bacterial pathogens, like Mycoplasma and those causing rickettsial disease, cause inflammation of the meninges and consequently encephalitis. Lyme disease or Bartonella henselae may also cause encephalitis.[ citation needed ]

Other Infectious Causes

Certain parasitic or protozoal infestations, such as toxoplasmosis and malaria can also cause encephalitis in people with compromised immune systems.

The rare but typically deadly forms of encephalitis, primary amoebic meningoencephalitis and Granulomatous amoebic encephalitis, are caused by free-living amoeba. [19]

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. [20]

Anti-NMDA 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. [21]

Another autoimmune cause includes acute disseminated encephalitis, a demyelinating disease which primarily affects children. [22]

Diagnosis

Spinal tap on a newborn Spinal tap newborn.JPG
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. [23] Diagnosing encephalitis is done via a variety of tests: [24] [25]

Prevention

Vaccination is available against tick-borne [26] and Japanese encephalitis [27] 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. [28] Contraindication to Pertussis immunization should be observed in patients with encephalitis. [29]

Treatment

An ideal drug to treat brain infection should be small, moderately lipophilic at pH of 7.4, low level of plasma protein binding, volume of distribution of litre per kg, does not have strong affinity towards binding with P-glycoprotein, or other efflux pumps on the surface of blood–brain barrier. Some drugs such as isoniazid, pyrazinamide, linezolid, metronidazole, fluconazole, and some fluoroquinolones have good penetration to blood brain barrier. [30] Treatment (which is based on supportive care) is as follows: [31]

Pyrimethamine-based maintenance therapy is often used to treat toxoplasmic encephalitis (TE), which is caused by Toxoplasma gondii and can be life-threatening for people with weak immune systems. [32] 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%. [32] This is a significant difference as relapse may impact the severity and prognosis of disease and result in an increase in healthcare expenditure. [32]

The effectiveness of intravenous immunoglobulin for the management of childhood encephalitis is unclear. Systematic reviews have been unable to draw firm conclusions because of a lack of randomised double-blind studies with sufficient numbers of patients and sufficient follow-up. [33] There is the possibility of a benefit of intravenous immunoglobulin for some forms of childhood encephalitis on some indicators such as length of hospital stay, time to stop spasms, time to regain consciousness, and time to resolution of neuropathic symptoms and fever. [33] Intravenous immunoglobulin for Japanese encephalitis appeared to have no benefit when compared with placebo (pretend) treatment. [33]

Prognosis

Identification of poor prognostic factors include cerebral edema, status epilepticus, and thrombocytopenia. [34] In contrast, a normal encephalogram at the early stages of diagnosis is associated with high rates of survival. [34]

Epidemiology

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Encephalitis deaths per million persons in 2012
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  15-24
  25-45

The number of new cases a year of acute encephalitis in Western countries is 7.4 cases per 100,000 people per year. In tropical countries, the incidence is 6.34 per 100,000 people per year. [35] 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. [34] In 2015, encephalitis was estimated to have affected 4.3 million people and resulted in 150,000 deaths worldwide. [4] [3] Herpes simplex encephalitis has an incidence of 2–4 per million of the population per year. [36]

Terminology

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 ἐγκέφαλος, enképhalos 'brain', [37] composed of ἐν, en, 'in' and κεφαλή, kephalé, 'head', and the medical suffix -itis 'inflammation'. [38]

See also

Related Research Articles

<span class="mw-page-title-main">Acute disseminated encephalomyelitis</span> Autoimmune disease

Acute disseminated encephalomyelitis (ADEM), or acute demyelinating encephalomyelitis, is a rare autoimmune disease marked by a sudden, widespread attack of inflammation in the brain and spinal cord. As well as causing the brain and spinal cord to become inflamed, ADEM also attacks the nerves of the central nervous system and damages their myelin insulation, which, as a result, destroys the white matter. The cause is often a trigger such as from viral infection or vaccinations.

<span class="mw-page-title-main">Viral meningitis</span> Medical condition

Viral meningitis, also known as aseptic meningitis, is a type of meningitis due to a viral infection. It results in inflammation of the meninges. Symptoms commonly include headache, fever, sensitivity to light and neck stiffness.

<span class="mw-page-title-main">Encephalitis lethargica</span> Form of encephalitis (sleeping sickness)

Encephalitis lethargica is an atypical form of encephalitis. Also known as "sleeping sickness" or "sleepy sickness", it was first described in 1917 by neurologist Constantin von Economo and pathologist Jean-René Cruchet. The disease attacks the brain, leaving some victims in a statue-like condition, speechless and motionless. Between 1915 and 1926, an epidemic of encephalitis lethargica spread around the world. The exact number of people infected is unknown, but it is estimated that more than one million people contracted the disease during the epidemic, which directly caused more than 500,000 deaths. Most of those who survived never recovered their pre-morbid vigour.

<span class="mw-page-title-main">Naegleriasis</span> Rare and usually fatal brain infection by a protist

Naegleriasis, also known as primary amoebic meningoencephalitis (PAM), is an almost invariably fatal infection of the brain by the free-living unicellular eukaryote Naegleria fowleri. Symptoms are meningitis-like and include headache, fever, nausea, vomiting, a stiff neck, confusion, hallucinations and seizures. Symptoms progress rapidly over around five days, and death usually results within one to two weeks of symptoms.

<span class="mw-page-title-main">Aseptic meningitis</span> Inflammation of the meninges not due to common bacterial infection

Aseptic meningitis is the inflammation of the meninges, a membrane covering the brain and spinal cord, in patients whose cerebral spinal fluid test result is negative with routine bacterial cultures. Aseptic meningitis is caused by viruses, mycobacteria, spirochetes, fungi, medications, and cancer malignancies. The testing for both meningitis and aseptic meningitis is mostly the same. A cerebrospinal fluid sample is taken by lumbar puncture and is tested for leukocyte levels to determine if there is an infection and goes on to further testing to see what the actual cause is. The symptoms are the same for both meningitis and aseptic meningitis but the severity of the symptoms and the treatment can depend on the certain cause.

<span class="mw-page-title-main">Neuritis</span> Inflammation of a nerve or generally any part of the nervous system

Neuritis, from the Greek νεῦρον), is inflammation of a nerve or the general inflammation of the peripheral nervous system. Inflammation, and frequently concomitant demyelination, cause impaired transmission of neural signals and leads to aberrant nerve function. Neuritis is often conflated with neuropathy, a broad term describing any disease process which affects the peripheral nervous system. However, neuropathies may be due to either inflammatory or non-inflammatory causes, and the term encompasses any form of damage, degeneration, or dysfunction, while neuritis refers specifically to the inflammatory process.

<span class="mw-page-title-main">Viral encephalitis</span> Medical condition

Viral encephalitis is inflammation of the brain parenchyma, called encephalitis, by a virus. The different forms of viral encephalitis are called viral encephalitides. It is the most common type of encephalitis and often occurs with viral meningitis. Encephalitic viruses first cause infection and replicate outside of the central nervous system (CNS), most reaching the CNS through the circulatory system and a minority from nerve endings toward the CNS. Once in the brain, the virus and the host's inflammatory response disrupt neural function, leading to illness and complications, many of which frequently are neurological in nature, such as impaired motor skills and altered behavior.

<span class="mw-page-title-main">Meningoencephalitis</span> Medical condition

Meningoencephalitis, also known as herpes meningoencephalitis, is a medical condition that simultaneously resembles both meningitis, which is an infection or inflammation of the meninges, and encephalitis, which is an infection or inflammation of the brain tissue.

<span class="mw-page-title-main">Granulomatous amoebic encephalitis</span> Rare and usually fatal brain infection by certain amoebae

Granulomatous amoebic encephalitis (GAE) is a rare, often fatal, subacute-to-chronic central nervous system disease caused by certain species of free-living amoebae of the genera Acanthamoeba, Balamuthia and Sappinia. The term is most commonly used with Acanthamoeba. In more modern references, the term "balamuthia amoebic encephalitis" (BAE) is commonly used when Balamuthia mandrillaris is the cause. Similarly, Sappinia amoebic encephalitis (SAE) is the name for amoebic encephalitis caused by species of Sappinia.

<span class="mw-page-title-main">Mollaret's meningitis</span> Medical condition

Mollaret's meningitis is a recurrent or chronic inflammation of the protective membranes covering the brain and spinal cord, known collectively as the meninges. Since Mollaret's meningitis is a recurrent, benign (non-cancerous), aseptic meningitis, it is also referred to as benign recurrent lymphocytic meningitis. It was named for Pierre Mollaret, the French neurologist who first described it in 1944.

The central nervous system (CNS) controls most of the functions of the body and mind. It comprises the brain, spinal cord and the nerve fibers that branch off to all parts of the body. The CNS viral diseases are caused by viruses that attack the CNS. Existing and emerging viral CNS infections are major sources of human morbidity and mortality.

<span class="mw-page-title-main">Meningitis</span> Inflammation of the membranes around the brain and spinal cord

Meningitis is acute or chronic inflammation of the protective membranes covering the brain and spinal cord, collectively called the meninges. The most common symptoms are fever, intense headache, vomiting and neck stiffness and occasionally photophobia. Other symptoms include confusion or altered consciousness, nausea, and an inability to tolerate light or loud noises. Young children often exhibit only nonspecific symptoms, such as irritability, drowsiness, or poor feeding. A non-blanching rash may also be present.

<span class="mw-page-title-main">Herpes meningitis</span> Medical condition

Herpes meningitis is inflammation of the meninges, the protective tissues surrounding the spinal cord and brain, due to infection from viruses of the Herpesviridae family - the most common amongst adults is HSV-2. Symptoms are self-limiting over 2 weeks with severe headache, nausea, vomiting, neck-stiffness, and photophobia. Herpes meningitis can cause Mollaret's meningitis, a form of recurrent meningitis. Lumbar puncture with cerebrospinal fluid results demonstrating aseptic meningitis pattern is necessary for diagnosis and polymerase chain reaction is used to detect viral presence. Although symptoms are self-limiting, treatment with antiviral medication may be recommended to prevent progression to Herpes Meningoencephalitis.

<span class="mw-page-title-main">Herpes simplex encephalitis</span> Encephalitis associated with herpes simplex virus

Herpes simplex encephalitis (HSE), or simply herpes encephalitis, is encephalitis due to herpes simplex virus. It is estimated to affect at least 1 in 500,000 individuals per year, and some studies suggest an incidence rate of 5.9 cases per 100,000 live births.

Neurovirology is an interdisciplinary field which represents a melding of clinical neuroscience, virology, immunology, and molecular biology. The main focus of the field is to study viruses capable of infecting the nervous system. In addition to this, the field studies the use of viruses to trace neuroanatomical pathways, for gene therapy, and to eliminate detrimental populations of neural cells.

<span class="mw-page-title-main">Lymphocytic pleocytosis</span> Increase in lymphocytes within cerebrospinal fluid

Lymphocytic pleocytosis is an abnormal increase in the amount of lymphocytes in the cerebrospinal fluid (CSF). It is usually considered to be a sign of infection or inflammation within the nervous system, and is encountered in a number of neurological diseases, such as pseudomigraine, Susac's syndrome, and encephalitis. While lymphocytes make up roughly a quarter of all white blood cells (WBC) in the body, they are generally rare in the CSF. Under normal conditions, there are usually less than 5 white blood cells per μL of CSF. In a pleocytic setting, the number of lymphocytes can jump to more than 1,000 cells per μL. Increases in lymphocyte count are often accompanied by an increase in cerebrospinal protein concentrations in addition to pleocytosis of other types of white blood cells.

<span class="mw-page-title-main">Post-viral cerebellar ataxia</span> Medical condition

Post-viral cerebellar ataxia also known as acute cerebellitis and acute cerebellar ataxia (ACA) is a disease characterized by the sudden onset of ataxia following a viral infection. The disease affects the function or structure of the cerebellum region in the brain.

<i>Naegleria fowleri</i> Species of free-living excavate form of protist

Naegleria fowleri, also known as the brain-eating amoeba, is a species of the genus Naegleria. It belongs to the phylum Percolozoa and is classified as an amoeboflagellate excavate, an organism capable of behaving as both an amoeba and a flagellate. This free-living microorganism primarily feeds on bacteria but can become pathogenic in humans, causing an extremely rare, sudden, severe, and almost always fatal brain infection known as naegleriasis or primary amoebic meningoencephalitis (PAM).

References

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  37. ↑ "Woodhouse's English-Greek Dictionary" (in German). The University of Chicago Library. Archived from the original on 2017-03-05. Retrieved 2013-01-10.
  38. ↑ 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.

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