Acute Disseminated Encephalomyelitis (ADEM): A Comprehensive Guide to Symptoms, Diagnosis, and Treatment
Acute disseminated encephalomyelitis (ADEM) is a condition that results from an immune-mediated inflammatory response that affects the white matter of the brain and spinal cord. Typically, this condition presents with an acute onset of encephalopathy and multiple neurologic symptoms that are spread throughout the body. Fortunately, ADEM often resolves on its own.
Multiple sclerosis and other juvenile acute demyelinating syndromes (ADS) share strong clinical and pathological similarities with ADEM. The diagnosis is based on clinical characteristics, results from neuroimaging, and results from laboratory tests. ADEM in kids is easily distinguished from other diagnoses.
Long-term follow-up is crucial since there are situations where an illness first classified as ADEM is finally substituted with a diagnosis of MS. This is because ADEM lacks a clearly identifiable biological signature, making a definitive laboratory diagnosis impossible.
Symptoms:
ADEM develops suddenly and quickly grows worse. These signs include:
- Fever
- Headache
- Sleepiness
- Modifications in behavior like irritability or disorientation
- Nausea and diarrhoea
- Muscle tremor
- Balance or mobility issues
- Vision issues
- Unsteady speech
- One side of the body may experience numbness or paralysis
- Seizures
- Coma
Anti-inflammatory medications are used as part of ADEM treatment to reduce brain inflammation. The majority of patients benefit from several days of treatment with intravenous corticosteroids, including methylprednisolone, accompanied by oral corticosteroid therapy. Plasmapheresis or intravenous immunoglobulin treatments are potential secondary treatment options that are claimed to be effective in certain severe instances when corticosteroids are ineffective. Additional treatment consists of symptomatic and supportive measures.
Alarming factors:
Although the precise origin of ADEM is unknown, it may be linked to an abnormal immune response that occurs after a viral trigger. In between 70 and 80 percent of individuals with ADEM, a first illness or infection is seen. For instance, ADEM may develop after a viral infection in the upper respiratory tract. Influenza, measles, mumps, rubella, varicella-zoster, Epstein-Barr virus, cytomegalovirus, and herpes simplex virus are other illnesses that can cause ADEM.
A vaccine may cause ADEM; however, this is extremely uncommon and only happens in around 5% of instances of ADEM. These case reports may not necessarily prove that immunization causes ADEM. According to recent research, antibodies to myelin oligodendrocyte glycoprotein are also related to ADEM (MOG).
The CNS contains a protein called MOG, which is a part of the myelin sheath. A number of inflammatory demyelinating disorders, including ADEM, have been associated with MOG protein antibodies, which are more frequently seen in youngsters.
Diagnosis:
No test is able to detect ADEM. The most common methods used by doctors to diagnose it are lumbar puncture (fluid taken out from the vicinity of the spinal cord and then tested) and MRI (which produces images of your brain using radio waves and a strong magnet)
Your doctor will perform these tests in order to rule out illnesses with comparable symptoms. On testing, ADEM and MS present differently:
- When you have MS, tests of your spinal fluid often reveal certain proteins, but not ADEM.
- Spinal fluid with ADEM often contains extra white blood cells than normal.
- On an MRI, brain damage brought on by ADEM and MS appear differently. ADEM has a wider distribution of it.
- The doctors would also exclude infections of the brain and the spinal cord, like meningitis.
Prognosis:
Acute disseminated encephalomyelitis (ADEM) is a rare autoimmune disorder that affects the central nervous system. The treatment for ADEM aims to reduce inflammation in the brain and manage symptoms. In some cases, symptoms may resolve on their own, so treatment may not even be necessary. However, in cases of more severe ADEM, treatment may include:
- Corticosteroids: These anti-inflammatory drugs are often prescribed to reduce inflammation in the brain and relieve symptoms. High doses of corticosteroids are typically given intravenously and gradually tapered down over several weeks.
- Plasma Exchange (plasmapheresis): This procedure involves removing blood from the body, separating plasma from the blood cells, and then returning the blood cells to the body. This may be used in severe cases of ADEM that do not respond to other treatments.
- Intravenous Immunoglobulin (IVIG): This treatment involves infusing a solution of antibodies obtained from healthy donors into the patient's bloodstream. IVIG may help reduce inflammation and boost the immune system.
- Rehabilitation Therapy: After initial treatment, rehabilitation therapy may be necessary to help patients recover from any physical or cognitive impairments caused by ADEM.
Conclusion:
Acute Disseminated Encephalomyelitis (ADEM) is a temporary but severe bout of inflammation (swelling) that destroys the myelin sheath (the white layer of nerve fibres) in the brain, spinal cord, and, infrequently, the optic nerves. Post-infectious encephalomyelitis or immune-mediated encephalomyelitis are other names for ADEM.