Acute disseminated encephalomyelitis adem is an immunemediated inflammatory demyelinating condition that predominately affects the white matter of the brain and spinal cord. White matter is tissue composed of nerve fibers, many of which are covered by a collection of fats and proteins known as myelin. Acute disseminated encephalomyelitis and acute hemorrhagic. If you have problems viewing pdf files, download the latest version of adobe reader. National institute of neurological disorders ninds.
Commonly triggered by viral infections, adem is caused by an inflammatory reaction in the brain and spinal cord. Despite the majority of adem cases being attributed to a postinfectious aetiology, other causes were reported. It usually affects children, most commonly under the age of 10. Acute disseminated encephalomyelitis adem is a monophasic autoimmune demyelinating disease of the central nervous system that typically follows a febrile infection or a vaccination.
Source for information on acute disseminated encephalomyelitis. The lack of a universally accepted definition of adem is a major impediment to understanding and describing its clinical. Diffuse neurological signs along with multifocal lesions in brain and spinal cord characterise the disease. Acute disseminated encephalomyelitis adem what is adem. S60 annals of indian academy of neurology, july 2011, vol 14, supplement 1 acute disseminated encephalomyelitis. In most cases, it follows a viral exanthem or viruslike illness, but the disease is not specific to viral infections and has been reported after several bacterial infections, vaccinations, and drug and serum administration. Acute disseminated encephalomyelitis ade is a neurological disorder involving inflammation of the brain and spinal cord. Acute disseminated encephalomyelitis prognosis, symptoms. Acute disseminated encephalomyelitis article nursingcenter. Acute disseminated encephalomyelitis linkedin slideshare.
Adem and acute hemorrhagic encephalomyelitis ahem represent two clinical variants of a single pathological process. Acute disseminated encephalomyelitis causes the exact occurrence of why adem occurs is unknown. Adem should be distinguished from other central inflammatory demyelinating disorders of children, including multiple sclerosis. Acute disseminated encephalomyelitis adem is a brief but intense attack of inflammation swelling in the brain and spinal cord and occasionally the optic nerves that damages the brains myelin the white coating of nerve fibers. Adem is a monophasic, nonvasculitic, inflammatory, demyelinating disease that can affect the. Acute disseminated encephalomyelitis adem and multiple sclerosis ms are both autoimmune disorders, and they share certain symptoms. Post vaccine aetiology was described for 5% of all adem cases and several. Adem is a rare kind of inflammation that affects the brain and spinal cord. Acute disseminated encephalomyelitis adem, as the name would suggest, is featured by a monophasic acute inflammation and demyelination of white matter typically following a recent 12 weeks prior viral infection or vaccination 4,6. Encephalomyelitis inflammation of the brain and spinal cord as a result of infection by neurotropic viruses acute disseminated encephalomyelitis or as a complication of other infectious diseases, for example, measles, chicken pox, or rubella. Acute disseminated encephalomyelitis definition acute disseminated encephalomyelitis ade is a neurological disorder involving inflammation of the brain and spinal cord. Acute disseminated encephalomyelitis adem, also known as postinfectious encephalomyelitis, is a demyelinating disease of the central nervous system that typically presents as a monophasic disorder associated with multifocal neurologic symptoms and encephalopathy. However, they diverge when it comes to diagnosis and treatment. It is characterized by a shortlived but widespread attack of.
Acute disseminated encephalomyelitis is a rare autoimmune disease marked by a sudden, widespread attack of inflammation in the brain and spinal cord. Adem can be defined as an acute or sub acute inflammatory or demyelinating event affecting multifocal. Acute disseminated encephalomyelitis adem is a disease that is characterized by an immunemediated inflammatory reaction and demyelination in the central nervous system, including optic nerve. Acute disseminated encephalomyelitis adem is an acute demyelinating disorder of the central nervous system, and is characterised by multifocal white matter involvement. Acute disseminated encephalomyelitis adem acute disseminated encephalomyelitis is a rare inflammatory demyelinating conditionof the brain and spinal cord. Mar 27, 2019 acute disseminated encephalomyelitis adem is characterized by a brief but widespread attack of inflammation in the brain and spinal cord that damages myelin the protective covering of nerve fibers. Acute disseminated encephalomyelitis information page. Murthy1 department of neurological sciences, christian medical college, vellore, 1the institute of neurological sciences, care hospital, hyderabad, india. Jan 20, 2017 adem is short for acute disseminated encephalomyelitis. As well as causing the brain and spinal cord to become inflamed, acute disseminated encephalomyelitis also damages the nerves and myelin of the central nervous system, which, as a result, destroys. Acute disseminated encephalomyelitis the clinical establishments. Acute disseminated encephalomyelitis adem is an uncommon inflammatory disorder within the cns, predominantly within the white matter of the brain and spinal cord.
Nov 08, 2018 acute disseminated encephalomyelitis adem is an immunemediated inflammatory demyelinating condition that predominately affects the white matter of the brain and spinal cord. Acute disseminated encephalomyelitis adem people will have complete, or near complete recoveries. It is characterized by an acute or subacute encephalopathy with neurological deficits, and magnetic resonance imaging evidence of widespread demyelination that. Acute disseminated encephalomyelitis adem is an inflammatory demyelinating disease of the central nervous system that usually affects children and. It affects children more than adults, but can affect anyone. Comorbid guillainbarre syndrome and acute disseminated.
Acute disseminated encephalomyelitis is an immunemediated inflammatory demyelinating disease of the central nervous system, which is typically transitory and selflimiting. Acute disseminated encephalomyelitis adem, also known as postinfectious encephalomyelitis, is an autoimmune demyelinating disease of the central nervous system. A spectrum of inflammation and demyelination in acute. Adem often follows viral infection, or less often, vaccinations for measles, mumps, or rubella mmr. Rust multiple sclerosis ms and acute disseminated encephalomyelitis adem are conditions whose closely related pathology suggests shared pathophysiological elements, but whose clinical courses are usually, but not always quite dissimilar. Diffuse neurological signs along with multifocal lesions in brain and spinal cord. Acute disseminated encephalomyelitis adem is a neurological, immunemediated disorder in which widespread inflammation of the brain and spinal cord damages tissue known as white matter. Management of acute disseminating encephalomyelitis full.
Acute disseminated encephalomyelitis adem is an immune mediated disease of the central nervous system cns that produces multiple inflammatory lesions in the brain and spinal cord, particularly in the white matter. Adem often follows viral or bacterial infections, or less often, vaccination for measles, mumps, or rubella. The disorder manifests as an acuteonset encephalopathy associated with polyfocal neurologic deficits and is typically selflimiting. Multiple sclerosis, acute disseminated encephalomyelitis. There are a number of individuals who may experience mild to moderate longterm impairment including cognitive difficulty, weakness, loss of vision or numbness. Acute disseminated encephalomyelitis archives srna. Acute disseminated encephalomyelitis definition of acute. Multiple sclerosis, acute disseminated encephalomyelitis, and related conditions robert s. Acute disseminated encephalomyelitis postgraduate medical. Acute disseminated encephalomyelitis adem is characterized by a brief but widespread attack of inflammation in the brain and spinal cord that damages myelin the protective covering of nerve fibers. It usually affects children, most commonly under the. Acute disseminated encephalomyelitis sciencedirect.
Most people make a full recovery and dont have another attack. Acute disseminated encephalomyelitis adem is an inflammatory demyelinating disorder that is often preceded by infection or recent vaccination. Ninds acute disseminated encephalomyelitis information page. Other terms used to refer to adem include postinfectious encephalomyelitis and immunemediated encephalomyelitis. Grey matter, especially that of the basal ganglia, is also often involved, albeit to a lesser extent, as is. We do not endorse non cleveland clinic products or services. Acute disseminated encephalomyelitis adem radiology. Acute disseminated encephalomyelitis, central nervous system. Adem is a monophasic, nonvasculitic, inflammatory, demyelinating disease that can affect the entire central nervous system cns. Adem is most commonly seen in children and young adults, in which prognosis is. 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. Acute disseminated encephalomyelitis adem is an immunemediated demyelinating cns disorder with predilection to early childhood. Some people may not respond to steroid treatment and intravenous immuoglobulin. Encephalopathy and focal neurological deficits are usually manifest several weeks after a prodromal illness with rapidly progressive neurologic decline.
This may lead to damage of the layer of insulation around the nerves myelin within affected areas. Refractory fulminant acute disseminated encephalomyelitis adem. Pathophysiology of acute disseminated encephalomyelitis. Once the diagnosis was madeseveral days after admission she received highdose steroids for 5 days. Acute disseminated encephalomyelitis adem is an acute, often monophasic postinfectious or inflammatory condition that most commonly occurs in children fig. 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. Acute disseminated encephalomyelitis adem is a monophasic, immune mediated demyelinating disorder. Acute disseminated encephalomyelitis or adem, is a rare neurological disorder that predominantly affects children, but can affect individuals of any age.
Once the diagnosis was madeseveral days after admission she received highdose steroids for 5. Acute disseminated encephalomyelitis adem is a demyelinating disorder characterized by multifocal involvement of the central nervous system cns white and gray matter and, in some cases, peripheral nervous system lesions15. Other boundaries of adem merge indistinctly with a wide variety of inflammatory encephalitic and vasculitic illnesses as well as. Acute disseminated encephalomyelitis adem is a neurological condition characterized by a brief but intense attack of inflammation in the brain and spinal cord. A plethora of viral and bacterial pathogens and a number of vaccinations have been associated with adem. Acute disseminated encephalomyelitis adem is a rare monophasic inflammatory demyelinating disorder of the central nervous system cns that occurs more often in children or young adults than in older people and has been recognized for more than 200 yr. Brain mri revealed multiple white matter lesions suggestive of adem.
Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children. Aug 16, 2016 one of my relatives was diagnosed with acute disseminated encephalomyelitis in 1997. Adem is short for acute disseminated encephalomyelitis. Epidemiology according to a study published in 2008, the estimated incidence in california is 0. Tenembaum silvia, chamoles nestor, fejerman natalio. Acute disseminated encephalomyelitis adem is an uncommon monophasic idiopathic inflammatory demyelinating disease. Following is a list of causes or underlying conditions see also misdiagnosis of underlying causes of acute disseminated encephalomyelitis that could possibly cause acute disseminated encephalomyelitis includes. 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. The disorder manifests as an acute onset encephalopathy associated with polyfocal neurologic deficits and is typically selflimiting. Acute disseminated encephalomyelitis genetic and rare.
A hallmark of the disorder is damage to the myelin sheath that surrounds the nerve fibers in the brain, which results in the inflammation. For language access assistance, contact the ncats public information officer. It damages the protective coating of nerve fibers, called myelin. Possibly, a t cell mediated autoimmune response to myelin basic protein, triggered by an infection or vaccination. This article describes the course of a young female diagnosed with acute disseminated encephalomyelitis adem with an atypical presentation. Acute disseminated encephalomyelitis article about acute. Cleveland clinic is a nonprofit academic medical center.
Td is a 22yearold female who presented to the emergency department ed with sudden onset of bilateral frontal and occipital headache, nausea, vomiting, unsteady gait, and numbness and tingling in all four extremities. The peculiar clinical scenery combining systemic and neurologic. Acute disseminated encephalomyelitis adem request pdf. Treatment algorithmacute disseminated encephalomyelitis. However, recurrent adem has been described and defined as multiphasic disseminated encephalomyelitis. The location of the inflammation swelling within the brain, spinal cord and optic nerves determines what symptoms of acute disseminated encephalomyelitis adem occur, including.
A case of acute disseminated encephalomyelitis in a middle. Inflammation damages the protective covering around the nerve fibres the myelin. Acute disseminated encephalomyelitis or multiple sclerosis. Review acute disseminated encephalomyelitis r k garg postgrad med j 2003. A 50yearold male with a history of multiple sclerosis. Due to poor medical care, he has not received consistent treatment and his health has deteriorated over the years. Acute disseminated encephalomyelitis adem is an acute, inflammatory, demyelinating disease of the brain and spinal cord. Summary of results acute disseminated encephalomyelitis adem is a rare autoimmune disease of the central nervous system, marked by widespread inflammation in the brain and spinal cord. Jul 26, 2017 acute disseminated encephalomyelitis adem is a neurological condition characterized by a brief but intense attack of inflammation in the brain and spinal cord. Acute disseminated encephalomyelitis adem is a rare neurological disorder. But studies show that as mentioned earlier, a great deal of patients who are suffering from this disease actually had a history of infection prior to diagnosing them as having adem disease. List of causes of acute disseminated encephalomyelitis.
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