Acute Necrotizing Encephalopathy Type 1—Encephalitis

Encephalitis is an inflammation of the brain. Usually the cause is a viral infection, but bacteria can also cause it. It can be mild or severe. Most cases are mild. You may have flu-like symptoms. With a mild case, you may just need rest, plenty of fluids, and a pain reliever.

Severe cases need immediate treatment. Symptoms of severe cases include

• Severe headache
• Sudden fever
• Drowsiness
• Vomiting
• Confusion
• Seizures

In babies, additional symptoms may include constant crying, poor feeding, body stiffness, and bulging in the soft spots of the skull.

Severe cases may require a stay in the hospital. Treatments include oral and intravenous (IV) medicines to reduce inflammation and treat infection. Patients with breathing difficulties may need artificial respiration. Some people may need physical, speech, and occupational therapy once the illness is under control.

NIH: National Institute of Neurological Disorders and Stroke

• Cerebral spinal fluid (CSF) collection (Medical Encyclopedia)
• CSF analysis (Medical Encyclopedia)
• Encephalitis (Medical Encyclopedia)
• Japanese Encephalitis Vaccine: What You Need to Know (Centers for Disease Control and Prevention) – PDF
• Subacute sclerosing panencephalitis (Medical Encyclopedia)

Acute necrotizing encephalopathy type 1, also known as susceptibility to infection-induced acute encephalopathy 3 or IIAE3, is a rare type of brain disease (encephalopathy) that occurs following a viral infection such as the flu.Acute necrotizing encephalopathy type 1 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.People with acute necrotizing encephalopathy type 1 develop areas of damage (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.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. 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.It is estimated that half of individuals with acute necrotizing encephalopathy type 1 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.