Diagnosis
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| Necrotizing encephalopathy | |
|---|---|
| Other names | Acute necrotizing encephalitis, Acute necrotizing encephalopathy of childhood (ANEC), infection-induced acute encephalopathy (IIAE), Autosomal dominant acute necrotising encephalopathy (ADANE), Acute Hemorrhagic Necrotizing Encephalopathy |
| Specialty | Infectious disease Neurology. |
| Symptoms | fever, cough, congestion, vomiting and diarrhea, for several days. After these flu-like symptoms, the affected individuals develop neurological problems, such as seizures, hallucinations, difficulty coordinating movements (ataxia) or abnormal muscle tone. [1] |
| Causes | Influenza is the most common virus found in people with acute necrotizing encephalopathy type 1; other viruses that are known to trigger this condition include human herpesvirus 6, coxsackie virus, and enteroviruses. In rare cases, the bacterium Mycoplasma pneumoniae is involved. [1] |
| Prevention | Vaccination, [2] face coverings, quarantine, physical/social distancing, ventilation, hand washing [3] |
| Medication | Steroid and intravenous immunoglobulin (IVIG) |
| Prognosis | Approximately one-third of individuals with acute necrotizing encephalopathy type 1 do not survive their illness and subsequent neurological decline. Of those who do survive, about half have permanent brain damage due to tissue necrosis, resulting in impairments in walking, speech, and other basic functions, there may also be permanent brain damage. [1] |
| Frequency | At least 59 cases of this condition have been reported in the scientific literature [1] |
Acute necrotizing encephalopathy (ANE) or sometimes necrotizing encephalitis or infection-induced acute encephalopathy (IIAE) is a rare type of brain disease (encephalopathy) that occurs following a viral infection. [4] Most commonly, it develops secondary to infection with influenza A, influenza B, and the human herpes virus 6. ANE can be familial or sporadic, but both forms are very similar to each other. Multiple subtypes, associated with specific genes have been found. [5]
Acute necrotizing encephalopathy typically appears in infancy or early childhood, although some people do not develop the condition until adolescence or adulthood. People with this condition usually show typical symptoms of an infection, such as fever, cough, congestion, vomiting, and diarrhea, for a few days. Following these flu-like symptoms, affected individuals develop neurological problems, such as seizures, hallucinations, difficulty coordinating movements (ataxia), or abnormal muscle tone. Eventually, most affected individuals go into a coma, which usually lasts for a number of weeks. The condition is described as "acute" because the episodes of illness are time-limited. [1]
People with acute necrotizing encephalopathy develop areas of damages (lesions) in certain regions of the brain. As the condition progresses, these brain regions develop swelling (edema), bleeding (hemorrhage), and then tissue death (necrosis). The progressive brain damage and tissue loss results in encephalopathy. [1]
ANE can be familial or sporadic, but both forms are very similar to each other. Most familial cases are caused by genetic changes in the RANBP2 gene, and are known as infection-induced acute encephalopathy 3 (IIAE3) or acute necrotizing encephalopathy type 1 (ANE1). [5]
In addition there is acute infection-induced (herpes-specific) encephalopathy-1 (IIAE1) associated with a homozygous UNC93B1 gene; and herpes-specific IIAE2 associated with the TLR3 gene; [6] as well as IIAE4, associated with the CPT2 gene; [7] herpes-specific IIAE5 associated with the TRAF3 gene; [8] herpes-specific IIAE6 associated with the TICAM1 gene; [9] herpes-specific IIAE7 associated with the IRF3 gene; [10] herpes-specific IIAE8 associated with the TBK1 gene; [11] IIAE9 associated with the NUP214 gene; [12] herpes-specific IIAE10 associated with the SNORA31 gene; [13] and herpes-specific IIAE11 associated with the DBR1 gene. [14]
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Optimal treatment regimes for the condition are not known. Suggested treatments may be corticosteroids and cytokine regulators such as TNF inhibitors. [4] [1]
Approximately one-third of individuals with acute necrotizing encephalopathy do not survive their illness and subsequent neurological decline. Of those who do survive, about half have permanent brain damage due to tissue necrosis, resulting in impairments in walking, speech, and other basic functions. Over time, many of these skills may be regained, but the loss of brain tissue is permanent. Other individuals who survive their illness appear to recover completely. [1]
It is estimated that half of individuals with acute necrotizing encephalopathy are susceptible to recurrent episodes and will have another infection that results in neurological decline; some people may have numerous episodes throughout their lives. Neurological function worsens following each episode as more brain tissue is damaged. [1]
The first case in the medical literature was described in 1955 by Belgian neurologist Ludo van Bogaert. [15]
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.
Encephalitis is inflammation of the brain. The severity can be variable with symptoms including reduction or alteration in consciousness, headache, fever, confusion, a stiff neck, and vomiting. Complications may include seizures, hallucinations, trouble speaking, memory problems, and problems with hearing.
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.
Microcephaly is a medical condition involving a smaller-than-normal head. Microcephaly may be present at birth or it may develop in the first few years of life. Brain development is often affected; people with this disorder often have an intellectual disability, poor motor function, poor speech, abnormal facial features, seizures and dwarfism.
Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, are a group of progressive, incurable, and fatal conditions that are associated with prions and affect the brain and nervous system of many animals, including humans, cattle, and sheep. According to the most widespread hypothesis, they are transmitted by prions, though some other data suggest an involvement of a Spiroplasma infection. Mental and physical abilities deteriorate and many tiny holes appear in the cortex causing it to appear like a sponge when brain tissue obtained at autopsy is examined under a microscope. The disorders cause impairment of brain function which may result in memory loss, personality changes, and abnormal or impaired movement which worsen over time.
Encephalopathy means any disorder or disease of the brain, especially chronic degenerative conditions. In modern usage, encephalopathy does not refer to a single disease, but rather to a syndrome of overall brain dysfunction; this syndrome has many possible organic and inorganic causes.
Aseptic meningitis is the inflammation of the meninges, a membrane covering the brain and spinal cord.
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.
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.
Human herpesvirus 6 (HHV-6) is the common collective name for human betaherpesvirus 6A (HHV-6A) and human betaherpesvirus 6B (HHV-6B). These closely related viruses are two of the nine known herpesviruses that have humans as their primary host.
Limbic encephalitis is a form of encephalitis, a disease characterized by inflammation of the brain. Limbic encephalitis is caused by autoimmunity: an abnormal state where the body produces antibodies against itself. Some cases are associated with cancer and some are not. Although the disease is known as "limbic" encephalitis, it is seldom limited to the limbic system and post-mortem studies usually show involvement of other parts of the brain. The disease was first described by Brierley and others in 1960 as a series of three cases. The link to cancer was first noted in 1968 and confirmed by later investigators.
Ohtahara syndrome (OS), also known as Early Infantile Developmental & Epileptic Encephalopathy (EIDEE) is a progressive epileptic encephalopathy. The syndrome is outwardly characterized by tonic spasms and partial seizures within the first few months of life, and receives its more elaborate name from the pattern of burst activity on an electroencephalogram (EEG). It is an extremely debilitating progressive neurological disorder, involving intractable seizures and severe intellectual disabilities. No single cause has been identified, although in many cases structural brain damage is present.
A neurotropic virus is a virus that is capable of infecting nerve tissue.
Chronic mucocutaneous candidiasis is an immune disorder of T cells. It is characterized by chronic infections with Candida that are limited to mucosal surfaces, skin, and nails. It can also be associated with other types of infections, such as human papilloma virus. An association with chromosome 2 has been identified.
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.
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.
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.
Aicardi–Goutières syndrome (AGS), which is completely distinct from the similarly named Aicardi syndrome, is a rare, usually early onset childhood, inflammatory disorder most typically affecting the brain and the skin. The majority of affected individuals experience significant intellectual and physical problems, although this is not always the case. The clinical features of AGS can mimic those of in utero acquired infection, and some characteristics of the condition also overlap with the autoimmune disease systemic lupus erythematosus (SLE). Following an original description of eight cases in 1984, the condition was first referred to as 'Aicardi–Goutières syndrome' (AGS) in 1992, and the first international meeting on AGS was held in Pavia, Italy, in 2001.
SLC35A1-CDG is a rare inherited disorder that mainly affects the vascular systems of the body. It forms part of a large group of disorders called congenital disorders of glycosylation. It is caused by mutations in the SLC35A1 gene, located in the sixth chromosome.
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