Acute disseminated encephalomyelitis

Definition

Acute disseminated encephalomyelitis (ADE) is a neurological disorder involving inflammation of the brain and spinal cord. A hallmark of the disorder is damage to the myelin sheath that surrounds the nerve fibers in the brain, which results in the inflammation.

Description

Acute disseminating encephalomyelitis was first described in the mid-eighteenth century. The English physician who first described the disorder noted its association with people who had recently recovered from smallpox. Symptoms often develop without warning. As well, mental disorientation can occur. The disorder is also known as postinfectious encephalomyelitis and immune-mediated encephalomyelitis. The nerve demyelination that occurs in ADE also occurs in multiple sclerosis . However, the two maladies differ in that multiple sclerosis is long lasting and can recur over time, while ADE has a monophasic course, meaning that once it is over, further attacks rarely occur.

Demographics

ADE can occur in both children and adults, although it occurs more commonly in children. ADE is not rare, accounting for approximately 30% of all cases of encephalitis (brain inflammation).

Causes and symptoms

Acute disseminating encephalomyelitis can occur as a consequence of a bacterial or viral infection (including HIV), following recovery from infection with the malarial protozoan, or as a side effect of vaccination or another inoculation. ADE is usually a consequence of a viral illness, and occurs most often after measles, followed by rubella, chicken pox, Epstein-Barr, mumps and pertussis (whooping cough). Typically, symptoms appear two to three weeks after the precipitating infection or immunization. Alternatively, ADE may develop with no known associations.

Despite the different causes, the symptoms that develop are similar. A number of non-specific symptoms, which vary from one person to another, include headache , stiff neck, fever, vomiting, and weight loss. These symptoms are quickly followed by lethargic behavior, seizures , hallucinations, sight difficulties, and even coma. Paralysis can occur in an arm or leg (monoparesis) or along an entire side of the body (hemiplegia).

These symptoms can last a few weeks to a month. In some people, symptoms can progress from the appearance of symptoms to coma and death in only a few days. Brain damage is largely confined to the white matter. Microscopic examination will typically reveal invasion of white blood cells into small veins. The nerve myelin damage occurs in the regions where the white blood cells accumulate. Examination of the brains of patients who have died of the disorder has not yielded consistent results. Some brains appear normal, while others display the nerve damage and white blood cell congestion.

Diagnosis

Diagnosis is made based on the above symptoms and the patient's medical history (i.e., recent infection or vaccination). In the early stages of the disorder, diagnosis can be confused with diseases including acute meningitis, acute viral encephalitis, and multiple sclerosis. Often, the latter can be ruled out using magnetic resonance imaging (MRI ) and examination of the cerebrospinal fluid (CSF). Typically, in acute disseminating encephalomyelitis, CSF contains abnormally elevated levels of white blood cells and protein; and magnetic resonance imaging can reveal brain alterations.

Treatment team

The treatment team typically consists of a primary care physician and, when hospitalization is necessary, nurses and specialized medical care personnel.

Treatment

Corticosteroid medication is often prescribed in order to lessen the nerve inflammation. Use of high doses of steroids can often produce a rapid diminishing of the symptoms. Other kinds of treatment depend on the nature of the symptoms that develop. Supportive care includes keeping a patient comfortable and hydrated.

Recovery and rehabilitation

Persons recovering from acute disseminated encephalomyelitis need time to recover their normal consciousness and movements. Problems with memory, especially short-term memory, may be present. The recovering person sometimes has trouble controlling their emotions and is easily frustrated. Frequent periods of rest, alternating with shorter periods of mental and physical exercise are prescribed during initial recovery. The maximum possible recovery of brain and motor function may take a period of weeks or months.

Clinical trials

There are no clinical trials for the study of ADE recruiting patients or being planned in the United States, as of January 2004. However, organizations such as the National Institute for Neurological Disorders and Stroke undertake and fund studies on disorders that involve damage to the myelin sheath of nerve cells. By understanding the nature of the disorders, it is hoped that detection can be improved and strategies will evolve to prevent or reverse the nerve damage.

Prognosis

Prognosis varies from person to person. Some patients may recover fully, with no residual effects. Others may have some residual damage. Seldomly, ADE is fatal. Early detection and treatment improves a patient's outlook.

Special concerns

Although the incidence of ADE occurring after vaccination is rare, in recent years, public debate has led some parents to choose that their children not receive the recommended childhood vaccinations. The American Academy of Pediatrics asserts that, despite concerns about vaccine safety, vaccination is far safer than accepting the risks for the diseases that the vaccines prevent.

Resources

BOOKS

Icon Health Publications. The Official Patient's Sourcebook on Acute Disseminated Encephalomyelitis: A Revised and Updated Directory for the Internet Age. San Diego: Icon Group International, 2002.

PERIODICALS

Anlar, B., C. Basaran, G. Kose, A. Guven, S. Haspolat, A. Yakut, A. Serdaroglu, N. Senbil, H. Tan, E. Karaagaoglu, and K. Oguz. "Acute disseminated encephalomyelitis in children: outcome and prognosis." Neuropediatrics (August 2003): 194–199.

Brass, S. D., Z. Caramanos, C. Santos, M. E. Dilenge, Y. Lapierre, and B. Rosenblatt. "Multiple sclerosis vs acute disseminated encephalomyelitis in childhood." Pediatric Neurology (September 2003): 227–231.

Koibuchi, T., T. Nakamura, T. Miura, T. Endo, H. Nakamura, T. Takahashi, H. S. Kim, Y. Wataya, K. Washizaki, K. Yoshikawa, and A. Iwamoto. "Acute disseminated encephalomyelitis following Plasmodium vivax malaria." Journal of Infection and Chemotherapy (September 2003): 254–256.

Narciso, P., S. Galgani, B. Del Grosso, M. De Marco, A. De Santis, P. Balestra, V. Ciapparoni, and V. Tozzi. "Acute disseminated encephalomyelitis as manifestation of primary HIV infection." Neurology (November 2001): 1493–1496.

OTHER

"Acute Disseminated Encephalomyelitis Information Page." National Institute of Neurological Disorders and Stroke. <http://www.ninds.nih.gov/health_and_medical/disorders/acute_encephalomyelitis_doc.htm> (January 26, 2004).

ORGANIZATIONS

National Institute for Neurological Diseases and Stroke (NINDS). 6001 Executive Boulevard, Bethesda, MD 20892. (301) 496-5751 or (800) 352-9424. <http://www.ninds.nih.gov>.

National Organization for Rare Disorders. 55 Kenosia Avenue, Danbury, CT 06813-1968. (203) 744-0100 or (800) 999-6673; Fax: (203) 798-2291. <http://www.rarediseases.org>.

Brian Douglas Hoyle, PhD