Acute disseminated encephalomyelitis
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Acute disseminated encephalomyelitis
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Nervous system pathology review
Congenital neurological disorders: Pathology review
Headaches: Pathology review
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Cerebral vascular disease: Pathology review
Traumatic brain injury: Pathology review
Spinal cord disorders: Pathology review
Dementia: Pathology review
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Rishi Desai, MD, MPHIn acute disseminated encephalomyelitis or ADEM, acute means that the disease evolves rapidly, disseminated refers to the fact that there are multiple sites involved, encephalo- refers to the brain, myelo- refers to the spinal cord, and -itis refers to inflammation.
So acute disseminated encephalomyelitis is an autoimmune disease of the central nervous system, where there’s sudden inflammation and demyelination at multiple sites of the brain and spinal cord.
The central nervous system consists of the brain and the spinal cord.
Grossly, the central nervous system can be divided into two main areas: the grey matter, which is made up of neuron cell bodies, and the white matter, which is made up of projections from the neuron cell bodies known as axons and dendrites.
The dendrites receive electrical impulses from other neurons; the neuron cell body has all of the neuron’s main organelles like the nucleus; and finally the axons transmit electrical impulses to the dendrites of the next neuron in the series.
Some axons are surrounded by a fatty protective sheath called myelin that helps increase the speed at which electrical impulses are sent.
This myelin is produced by oligodendrocytes, which are a group of cells that support neurons.
Now, the brain is protected by harmful things in the blood by the blood brain barrier, which only lets certain molecules and cells through. For immune cells like T and B cells that means having the right ligand or surface molecule to get through the blood brain barrier, this is kind of like having a VIP pass to get into an exclusive club.
Once a T cell makes its way in, it can get activated by something it encounters.
Once the T-cell gets activated, it changes the blood brain barrier cells to express more receptors, and this allows immune cells to more easily bind and get in, kind of like bribing the bouncer to let a lot of people in.
Summary
Acute disseminated encephalomyelitis (ADEM), also called post-infectious encephalomyelitis, is a rare but serious neurologic disorder in which nerves in the brain and spinal cord become demyelinated due to an inflammation that takes place after a course of an infection.
The cause of ADEM is not well understood, but it is believed to be autoimmune-related. Symptoms come on suddenly and include headache, fever, difficulty speaking or understanding speech, weakness or paralysis of an arm or leg, double vision, seizures, and coma.
Sources
- "Robbins Basic Pathology" Elsevier (2017)
- "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
- "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
- "Acute disseminated encephalomyelitis: A long-term follow-up study of 84 pediatric patients" Neurology (2002)
- "Acute disseminated encephalomyelitis following inactivated influenza vaccination in the Brazilian Amazon: a case report" Revista da Sociedade Brasileira de Medicina Tropical (2015)
- "Acute disseminated encephalomyelitis with severe neurological outcomes following virosomal seasonal influenza vaccine" Human Vaccines & Immunotherapeutics (2014)
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