Myelitis Transversa
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Transverse myelitis (TM) is a rare
neurological Neurology (from el, νεῦρον (neûron), "string, nerve" and the suffix -logia, "study of") is the branch of medicine dealing with the diagnosis and treatment of all categories of conditions and disease involving the brain, the spinal ...
condition wherein the
spinal cord The spinal cord is a long, thin, tubular structure made up of nervous tissue, which extends from the medulla oblongata in the brainstem to the lumbar region of the vertebral column (backbone). The backbone encloses the central canal of the spi ...
is
inflamed Inflammation (from la, inflammatio) is part of the complex biological response of body tissues to harmful stimuli, such as pathogens, damaged cells, or irritants, and is a protective response involving immune cells, blood vessels, and molecu ...
. The adjective ''
transverse Transverse may refer to: *Transverse engine, an engine in which the crankshaft is oriented side-to-side relative to the wheels of the vehicle *Transverse flute, a flute that is held horizontally * Transverse force (or ''Euler force''), the tangen ...
'' implies that the spinal inflammation ( myelitis) extends horizontally throughout the
cross section Cross section may refer to: * Cross section (geometry) ** Cross-sectional views in architecture & engineering 3D *Cross section (geology) * Cross section (electronics) * Radar cross section, measure of detectability * Cross section (physics) **Abs ...
of the spinal cord; the terms ''partial transverse myelitis'' and ''partial myelitis'' are sometimes used to specify inflammation that affects only part of the width of the spinal cord. TM is characterized by weakness and numbness of the limbs, deficits in sensation and motor skills, dysfunctional urethral and anal sphincter activities, and dysfunction of the
autonomic nervous system The autonomic nervous system (ANS), formerly referred to as the vegetative nervous system, is a division of the peripheral nervous system that supplies viscera, internal organs, smooth muscle and glands. The autonomic nervous system is a control ...
that can lead to episodes of
high blood pressure Hypertension (HTN or HT), also known as high blood pressure (HBP), is a long-term medical condition in which the blood pressure in the arteries is persistently elevated. High blood pressure usually does not cause symptoms. Long-term high bl ...
. Signs and symptoms vary according to the affected level of the spinal cord. The underlying cause of TM is unknown. The spinal cord inflammation seen in TM has been associated with various infections, immune system disorders, or damage to nerve fibers, by loss of myelin. As opposed to
leukomyelitis Leukomyelitis, also known as acute spasmodic paraplegia, is a disorder of the central nervous system. It affects only the "white matter" of the spinal cord The spinal cord is a long, thin, tubular structure made up of nervous tissue, which exten ...
which affects only the
white matter White matter refers to areas of the central nervous system (CNS) that are mainly made up of myelinated axons, also called tracts. Long thought to be passive tissue, white matter affects learning and brain functions, modulating the distribution ...
, it affects the entire cross-section of the spinal cord. Decreased
electrical conductivity Electrical resistivity (also called specific electrical resistance or volume resistivity) is a fundamental property of a material that measures how strongly it resists electric current. A low resistivity indicates a material that readily allow ...
in the nervous system can result.


Signs and symptoms

Symptoms include weakness and numbness of the limbs, deficits in sensation and motor skills, dysfunctional urethral and anal sphincter activities, and dysfunction of the
autonomic nervous system The autonomic nervous system (ANS), formerly referred to as the vegetative nervous system, is a division of the peripheral nervous system that supplies viscera, internal organs, smooth muscle and glands. The autonomic nervous system is a control ...
that can lead to episodes of
high blood pressure Hypertension (HTN or HT), also known as high blood pressure (HBP), is a long-term medical condition in which the blood pressure in the arteries is persistently elevated. High blood pressure usually does not cause symptoms. Long-term high bl ...
. Symptoms typically develop over the course of hours to a few weeks. Sensory symptoms of TM may include a sensation of
pins and needles ''Pins and Needles'' (1937) is a musical revue with a book by Arthur Arent, Marc Blitzstein, Emmanuel Eisenberg, Charles Friedman, David Gregory, Joseph Schrank, Arnold B. Horwitt, John Latouche, and Harold Rome, and music and lyrics by Rome. ...
traveling up from the feet. The degree and type of sensory loss will depend upon the extent of the involvement of the various sensory tracts, but there is often a "sensory level" at the spinal ganglion of the segmental spinal nerve, below which sensation to pain or light touch is impaired. Motor weakness occurs due to involvement of the pyramidal tracts and mainly affects the muscles that flex the legs and extend the arms. Disturbances in sensory nerves and motor nerves and dysfunction of the
autonomic nervous system The autonomic nervous system (ANS), formerly referred to as the vegetative nervous system, is a division of the peripheral nervous system that supplies viscera, internal organs, smooth muscle and glands. The autonomic nervous system is a control ...
at the level of the lesion or below, are noted. Therefore, the signs and symptoms depend on the area of the spine involved. Back pain can occur at the level of any inflamed segment of the spinal cord. If the upper
cervical segment The spinal cord is a long, thin, tubular structure made up of nervous tissue, which extends from the medulla oblongata in the brainstem to the lumbar region of the vertebral column (backbone). The backbone encloses the central canal of the spinal ...
of the spinal cord is involved, all four limbs may be affected and there is risk of respiratory failure – the
phrenic nerve The phrenic nerve is a mixed motor/sensory nerve which originates from the C3-C5 spinal nerves in the neck. The nerve is important for breathing because it provides exclusive motor control of the diaphragm, the primary muscle of respiration. In ...
which is formed by the cervical spinal nerves C3, C4, and C5 innervates the main muscle of respiration, the
diaphragm Diaphragm may refer to: Anatomy * Thoracic diaphragm, a thin sheet of muscle between the thorax and the abdomen * Pelvic diaphragm or pelvic floor, a pelvic structure * Urogenital diaphragm or triangular ligament, a pelvic structure Other * Diap ...
. Lesions of the lower cervical region (C5–T1) will cause a combination of upper and lower motor neuron signs in the upper limbs, and exclusively upper motor neuron signs in the lower limbs. Cervical lesions account for about 20% of cases. A lesion of the
thoracic segment The spinal cord is a long, thin, tubular structure made up of nervous tissue, which extends from the medulla oblongata in the brainstem to the lumbar region of the vertebral column (backbone). The backbone encloses the central canal of the spi ...
( T1–12) will produce upper motor neuron signs in the lower limbs, presenting as a spastic paraparesis. This is the most common location of the lesion, and therefore most individuals will have weakness of the lower limbs.{{cite book , vauthors = Alexander MA, Matthews DJ, Murphy KP , title=Pediatric Rehabilitation, Fifth Edition: Principles and Practice, url=https://books.google.com/books?id=Gp4sCQAAQBAJ&pg=PA523, year=2015, publisher=Demos Medical Publishing, isbn=978-1-62070-061-7, pages=523, 524 A lesion of the
lumbar segment The spinal cord is a long, thin, tubular structure made up of nervous tissue, which extends from the medulla oblongata in the brainstem to the lumbar region of the vertebral column (backbone). The backbone encloses the central canal of the spinal ...
, the lower part of the spinal cord ( L1S5) often produces a combination of upper and lower motor neuron signs in the lower limbs. Lumbar lesions account for about 10% of cases.


Causes

TM is a
heterogeneous condition A heterogeneous medical condition or heterogeneous disease is a medical term referring to a medical condition with several etiologies (root causes), such as hepatitis or diabetes. Medical conditions are normally defined pathologically (i.e. based o ...
, that is, there are several identified causes. Sometimes the term ''Transverse myelitis spectrum disorders'' is used. In 60% of patients the cause is idiopathic. In rare cases, it may be associated with
meningococcal meningitis Meningococcal disease describes infections caused by the bacterium ''Neisseria meningitidis'' (also termed meningococcus). It has a high mortality rate if untreated but is vaccine-preventable. While best known as a cause of meningitis, it can a ...
{{cite journal , pmid=2380146, year=1990, last1=Khare, first1=K. C., last2=Masand, first2=U., last3=Vishnar, first3=A., title=Transverse myelitis – a rare complication of meningococcal meningitis, journal=The Journal of the Association of Physicians of India, volume=38, issue=2, page=188 When it appears as a
comorbid In medicine, comorbidity - from Latin morbus ("sickness"), co ("together"), -ity (as if - several sicknesses together) - is the presence of one or more additional conditions often co-occurring (that is, concomitant or concurrent) with a primary c ...
condition with
neuromyelitis optica Neuromyelitis optica spectrum disorders (NMOSD), including neuromyelitis optica (NMO), are autoimmune diseases characterized by acute inflammation of the optic nerve (optic neuritis, ON) and the spinal cord (myelitis). Episodes of ON and myelitis ...
(NMO), it is considered to be caused by NMO-
IgG Immunoglobulin G (Ig G) is a type of antibody. Representing approximately 75% of serum antibodies in humans, IgG is the most common type of antibody found in blood circulation. IgG molecules are created and released by plasma B cells. Each IgG a ...
autoimmunity, and when it appears in
multiple sclerosis Multiple (cerebral) sclerosis (MS), also known as encephalomyelitis disseminata or disseminated sclerosis, is the most common demyelinating disease, in which the insulating covers of nerve cells in the brain and spinal cord are damaged. This d ...
(MS) cases, it is considered to be produced by the same underlying condition that produces the MS plaques.{{citation needed, date=July 2018 Other causes of TM include infections, immune system disorders, and demyelinating diseases.{{cite web, url=http://www.ninds.nih.gov/disorders/transversemyelitis/detail_transversemyelitis.htm, title=Transverse Myelitis Fact Sheet , work = National Institute of Neurological Disorders and Stroke (NINDS) , access-date=2015-08-06
Viral Viral means "relating to viruses" (small infectious agents). Viral may also refer to: Viral behavior, or virality Memetic behavior likened that of a virus, for example: * Viral marketing, the use of existing social networks to spread a marke ...
infections known to be associated with TM include
HIV The human immunodeficiency viruses (HIV) are two species of ''Lentivirus'' (a subgroup of retrovirus) that infect humans. Over time, they cause acquired immunodeficiency syndrome (AIDS), a condition in which progressive failure of the immune ...
, herpes simplex,
herpes zoster Shingles, also known as zoster or herpes zoster, is a viral disease characterized by a painful skin rash with blisters in a localized area. Typically the rash occurs in a single, wide mark either on the left or right side of the body or face. ...
,
cytomegalovirus ''Cytomegalovirus'' (''CMV'') (from ''cyto-'' 'cell' via Greek - 'container' + 'big, megalo-' + -''virus'' via Latin 'poison') is a genus of viruses in the order ''Herpesvirales'', in the family ''Herpesviridae'', in the subfamily ''Betaherpe ...
, and Epstein-Barr.{{cite journal , vauthors = Levin SN, Lyons JL , title = Infections of the Nervous System , journal = The American Journal of Medicine , volume = 131 , issue = 1 , pages = 25–32 , date = January 2018 , pmid = 28889928 , doi = 10.1016/j.amjmed.2017.08.020 , type = Review
Flavivirus ''Flavivirus'' is a genus of positive-strand RNA viruses in the family ''Flaviviridae''. The genus includes the West Nile virus, dengue virus, tick-borne encephalitis virus, yellow fever virus, Zika virus and several other viruses which may caus ...
infections such as
Zika virus ''Zika virus'' (ZIKV; pronounced or ) is a member of the virus family ''Flaviviridae''. It is spread by daytime-active ''Aedes'' mosquitoes, such as '' A. aegypti'' and '' A. albopictus''. Its name comes from the Ziika Forest of Uganda, whe ...
and
West Nile virus West Nile virus (WNV) is a single-stranded RNA virus that causes West Nile fever. It is a member of the family ''Flaviviridae'', from the genus ''Flavivirus'', which also contains the Zika virus, dengue virus, and yellow fever virus. The virus ...
have also been associated. Viral association of transverse myelitis could result from the infection itself or from the response to it. Bacterial causes associated with TM include '' Mycoplasma pneumoniae'', '' Bartonella henselae'', and the types of '' Borrelia'' that cause
Lyme disease Lyme disease, also known as Lyme borreliosis, is a vector-borne disease caused by the ''Borrelia'' bacterium, which is spread by ticks in the genus ''Ixodes''. The most common sign of infection is an expanding red rash, known as erythema migran ...
. Lyme disease gives rise to
neuroborreliosis Neuroborreliosis is a disorder of the central nervous system. A neurological manifestation of Lyme disease, neuroborreliosis is caused by a systemic infection of spirochetes of the genus ''Borrelia.'' Symptoms of the disease include erythema migr ...
which is seen in a small percentage (4 to 5 per cent) of acute transverse myelitis cases. The diarrhea-causing bacteria '' Campylobacter jejuni'' is also a reported cause of transverse myelitis.{{cite journal , vauthors = Ross AG, Olds GR, Cripps AW, Farrar JJ, McManus DP , title = Enteropathogens and chronic illness in returning travelers , journal = The New England Journal of Medicine , volume = 368 , issue = 19 , pages = 1817–25 , date = May 2013 , pmid = 23656647 , doi = 10.1056/NEJMra1207777 , type = Review , url = https://semanticscholar.org/paper/7cd0634d720dddde7866f5bec49c311bb124ee41 , hdl = 10072/54169 , s2cid = 13789364 , hdl-access = free Other associated causes include the
helminth Parasitic worms, also known as helminths, are large macroparasites; adults can generally be seen with the naked eye. Many are intestinal worms that are soil-transmitted and infect the gastrointestinal tract. Other parasitic worms such as schi ...
infection
schistosomiasis Schistosomiasis, also known as snail fever, bilharzia, and Katayama fever, is a disease caused by parasitic flatworms called schistosomes. The urinary tract or the intestines may be infected. Symptoms include abdominal pain, diarrhea, bloody s ...
, spinal cord injuries, vascular disorders that impede the blood flow through vessels of the spinal cord, and
paraneoplastic syndrome A paraneoplastic syndrome is a syndrome (a set of signs and symptoms) that is the consequence of a tumor in the body (usually a cancerous one), specifically due to the production of chemical signaling molecules (such as hormones or cytokines) by ...
.


Pathophysiology

This progressive loss of the fatty myelin sheath surrounding the nerves in the affected spinal cord occurs for unclear reasons following
infection An infection is the invasion of tissues by pathogens, their multiplication, and the reaction of host tissues to the infectious agent and the toxins they produce. An infectious disease, also known as a transmissible disease or communicable dise ...
s or due to
multiple sclerosis Multiple (cerebral) sclerosis (MS), also known as encephalomyelitis disseminata or disseminated sclerosis, is the most common demyelinating disease, in which the insulating covers of nerve cells in the brain and spinal cord are damaged. This d ...
. Infections may cause TM through direct tissue damage or by immune-mediated infection-triggered tissue damage. The lesions present are usually inflammatory. Spinal cord involvement is usually central, uniform, and symmetric in comparison to
multiple sclerosis Multiple (cerebral) sclerosis (MS), also known as encephalomyelitis disseminata or disseminated sclerosis, is the most common demyelinating disease, in which the insulating covers of nerve cells in the brain and spinal cord are damaged. This d ...
which typically affects the cord in a patchy way and the lesions are usually peripheral. The lesions in acute TM are mostly limited to the spinal cord with no involvement of other structures in the
central nervous system The central nervous system (CNS) is the part of the nervous system consisting primarily of the brain and spinal cord. The CNS is so named because the brain integrates the received information and coordinates and influences the activity of all par ...
.


Longitudinally extensive transverse myelitis

A proposed special clinical presentation is the "longitudinally extensive transverse myelitis" (LETM), which is defined as a TM with a spinal cord lesion that extends over three or more vertebral segments. The causes of LETM are also heterogeneous{{cite journal , vauthors = Pekcevik Y, Mitchell CH, Mealy MA, Orman G, Lee IH, Newsome SD, Thompson CB, Pardo CA, Calabresi PA, Levy M, Izbudak I , display-authors = 6 , title = Differentiating neuromyelitis optica from other causes of longitudinally extensive transverse myelitis on spinal magnetic resonance imaging , journal = Multiple Sclerosis (Houndmills, Basingstoke, England) , volume = 22 , issue = 3 , pages = 302–11 , date = March 2016 , pmid = 26209588 , pmc = 4797654 , doi = 10.1177/1352458515591069 and the presence of
MOG Mog may refer to: Entertainment Characters * Mog (''Final Fantasy VI''), in the game * Mog (Judith Kerr), a cat in Kerr's children's books * Mog, a half-man/half-dog in the film ''Spaceballs'' * A cat in the Meg and Mog children's books by Helen ...
auto-antibodies has been proposed as a diagnostic biomarker.{{cite journal , vauthors = Cobo-Calvo Á, Sepúlveda M, Bernard-Valnet R, Ruiz A, Brassat D, Martínez-Yélamos S, Saiz A, Marignier R , display-authors = 6 , title = Antibodies to myelin oligodendrocyte glycoprotein in aquaporin 4 antibody seronegative longitudinally extensive transverse myelitis: Clinical and prognostic implications , journal = Multiple Sclerosis , volume = 22 , issue = 3 , pages = 312–19 , date = March 2016 , pmid = 26209592 , doi = 10.1177/1352458515591071 , s2cid = 8356201


Diagnosis


Diagnostic criteria

In 2002, the Transverse Myelitis Consortium Working Group proposed the following diagnostic criteria for idiopathic acute transverse myelitis:{{cite journal , author = Transverse Myelitis Consortium Working Group , title = Proposed diagnostic criteria and nosology of acute transverse myelitis , journal = Neurology , volume = 59 , issue = 4 , pages = 499–505 , date = August 2002 , pmid = 12236201 , doi = 10.1212/WNL.59.4.499 {{columns-list, colwidth=30em, * Inclusion criteria ** Motor, sensory or autonomic dysfunction attributable to spinal cord ** Signs and symptoms on both sides of the body (not necessarily symmetrical) ** Clearly defined sensory level ** Signs of inflammation ( pleocytosis of the
cerebrospinal fluid Cerebrospinal fluid (CSF) is a clear, colorless body fluid found within the tissue that surrounds the brain and spinal cord of all vertebrates. CSF is produced by specialised ependymal cells in the choroid plexus of the ventricles of the bra ...
, or elevated
immunoglobulin G Immunoglobulin G (Ig G) is a type of antibody. Representing approximately 75% of serum antibodies in humans, IgG is the most common type of antibody found in blood circulation. IgG molecules are created and released by plasma B cells. Each IgG a ...
, or evidence of inflammation on gadolinium-enhanced (MRI) Magnetic resonance imaging) ** Peak of this condition can occur anytime between 4 hours to 21 days after onset * Exclusion criteria ** Irradiation of the spine (e.g.,
radiotherapy Radiation therapy or radiotherapy, often abbreviated RT, RTx, or XRT, is a therapy using ionizing radiation, generally provided as part of cancer treatment to control or kill malignant cells and normally delivered by a linear accelerator. Radia ...
) in the last 10 years ** Evidence of thrombosis of the
anterior spinal artery In human anatomy, the anterior spinal artery is the artery that supplies the anterior portion of the spinal cord. It arises from branches of the vertebral arteries and courses along the anterior aspect of the spinal cord. It is reinforced by sever ...
** Evidence of extra-axial compression on neuroimaging ** Evidence of
arteriovenous malformation Arteriovenous malformation is an abnormal connection between arteries and veins, bypassing the capillary system. This vascular anomaly is widely known because of its occurrence in the central nervous system (usually cerebral AVM), but can appea ...
(abnormal flow voids on surface of spine) ** Evidence of connective tissue disease, e.g.
sarcoidosis Sarcoidosis (also known as ''Besnier-Boeck-Schaumann disease'') is a disease involving abnormal collections of inflammatory cells that form lumps known as granulomata. The disease usually begins in the lungs, skin, or lymph nodes. Less commonly af ...
, Behçet's disease, Sjögren's syndrome,
systemic lupus erythematosus Lupus, technically known as systemic lupus erythematosus (SLE), is an autoimmune disease in which the body's immune system mistakenly attacks healthy tissue in many parts of the body. Symptoms vary among people and may be mild to severe. Comm ...
or
mixed connective tissue disease Mixed connective tissue disease, commonly abbreviated as MCTD, is an autoimmune disease characterized by the presence of elevated blood levels of a specific autoantibody, now called anti-U1 ribonucleoprotein (RNP) together with a mix of symptoms of ...
** Evidence of optic neuritis (diagnostic of
neuromyelitis optica Neuromyelitis optica spectrum disorders (NMOSD), including neuromyelitis optica (NMO), are autoimmune diseases characterized by acute inflammation of the optic nerve (optic neuritis, ON) and the spinal cord (myelitis). Episodes of ON and myelitis ...
(NMO)) ** Evidence of infection (
syphilis Syphilis () is a sexually transmitted infection caused by the bacterium ''Treponema pallidum'' subspecies ''pallidum''. The signs and symptoms of syphilis vary depending in which of the four stages it presents (primary, secondary, latent, an ...
,
Lyme disease Lyme disease, also known as Lyme borreliosis, is a vector-borne disease caused by the ''Borrelia'' bacterium, which is spread by ticks in the genus ''Ixodes''. The most common sign of infection is an expanding red rash, known as erythema migran ...
, Human immunodeficiency virus,
Human T-lymphotropic virus 1 Human T-cell lymphotropic virus type 1 or human T-lymphotropic virus (HTLV-I), also called the adult T-cell lymphoma virus type 1, is a retrovirus of the human T-lymphotropic virus (HTLV) family that has been implicated in several kinds of disea ...
,
mycoplasma ''Mycoplasma'' is a genus of bacteria that, like the other members of the class ''Mollicutes'', lack a cell wall around their cell membranes. Peptidoglycan (murein) is absent. This characteristic makes them naturally resistant to antibiotics ...
,
Herpes simplex virus Herpes simplex virus 1 and 2 (HSV-1 and HSV-2), also known by their taxonomical names ''Human alphaherpesvirus 1'' and '' Human alphaherpesvirus 2'', are two members of the human ''Herpesviridae'' family, a set of viruses that produce viral inf ...
, Varicella-zoster virus, Epstein-Barr virus,
cytomegalovirus ''Cytomegalovirus'' (''CMV'') (from ''cyto-'' 'cell' via Greek - 'container' + 'big, megalo-' + -''virus'' via Latin 'poison') is a genus of viruses in the order ''Herpesvirales'', in the family ''Herpesviridae'', in the subfamily ''Betaherpe ...
, Human herpesvirus 6 or enteroviruses) ** Evidence of
multiple sclerosis Multiple (cerebral) sclerosis (MS), also known as encephalomyelitis disseminata or disseminated sclerosis, is the most common demyelinating disease, in which the insulating covers of nerve cells in the brain and spinal cord are damaged. This d ...
(abnormalities detected on MRI and presence of oligoclonal antibodies in
cerebrospinal fluid Cerebrospinal fluid (CSF) is a clear, colorless body fluid found within the tissue that surrounds the brain and spinal cord of all vertebrates. CSF is produced by specialised ependymal cells in the choroid plexus of the ventricles of the bra ...
(CSF))


Investigations

Individuals who develop TM are typically transferred to a neurologist who can urgently investigate the patient in a hospital. If
breathing Breathing (or ventilation) is the process of moving air into and from the lungs to facilitate gas exchange with the internal environment, mostly to flush out carbon dioxide and bring in oxygen. All aerobic creatures need oxygen for cellular ...
is affected, particularly in upper spinal cord lesions, methods of
artificial ventilation Artificial ventilation (also called artificial respiration) is a means of assisting or stimulating respiration, a metabolic process referring to the overall exchange of gases in the body by pulmonary ventilation, external respiration, and interna ...
must be on hand before and during the transfer procedure. The patient should also be catheterized to test for and, if necessary, drain an over-distended
bladder The urinary bladder, or simply bladder, is a hollow organ in humans and other vertebrates that stores urine from the kidneys before disposal by urination. In humans the bladder is a distensible organ that sits on the pelvic floor. Urine enters ...
. A
lumbar puncture Lumbar puncture (LP), also known as a spinal tap, is a medical procedure in which a needle is inserted into the spinal canal, most commonly to collect cerebrospinal fluid (CSF) for diagnostic testing. The main reason for a lumbar puncture is to ...
can be performed after the MRI or at the time of CT myelography.
Corticosteroid Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are involv ...
s are often given in high doses when symptoms begin with the hope that the degree of inflammation and swelling of the spinal cord will be lessened, but whether this is truly effective is still debated.


Differential diagnosis

The
differential diagnosis In healthcare, a differential diagnosis (abbreviated DDx) is a method of analysis of a patient's history and physical examination to arrive at the correct diagnosis. It involves distinguishing a particular disease or condition from others that p ...
of acute TM includes
demyelinating Myelin is a lipid-rich material that surrounds nerve cell axons (the nervous system's "wires") to insulate them and increase the rate at which electrical impulses (called action potentials) are passed along the axon. The myelinated axon can be l ...
disorders, such as
multiple sclerosis Multiple (cerebral) sclerosis (MS), also known as encephalomyelitis disseminata or disseminated sclerosis, is the most common demyelinating disease, in which the insulating covers of nerve cells in the brain and spinal cord are damaged. This d ...
and
neuromyelitis optica Neuromyelitis optica spectrum disorders (NMOSD), including neuromyelitis optica (NMO), are autoimmune diseases characterized by acute inflammation of the optic nerve (optic neuritis, ON) and the spinal cord (myelitis). Episodes of ON and myelitis ...
, infections, such as herpes zoster and herpes simplex virus, and other types of inflammatory disorders, such as
systemic lupus erythematosus Lupus, technically known as systemic lupus erythematosus (SLE), is an autoimmune disease in which the body's immune system mistakenly attacks healthy tissue in many parts of the body. Symptoms vary among people and may be mild to severe. Comm ...
and
neurosarcoidosis Neurosarcoidosis (sometimes shortened to neurosarcoid) refers to a type of sarcoidosis, a condition of unknown cause featuring granulomas in various tissues, in this type involving the central nervous system (brain and spinal cord). Neurosarcoidosi ...
. It is important to also rule out an acute cause of compression on the spinal cord.


Treatment

If treated early, some people experience complete or near complete recovery. Treatment options also vary according to the underlying cause. One treatment option includes
plasmapheresis Plasmapheresis (from the Greek πλάσμα, ''plasma'', something molded, and ἀφαίρεσις ''aphairesis'', taking away) is the removal, treatment, and return or exchange of blood plasma or components thereof from and to the blood circulati ...
. Recovery from TM is variable between individuals and also depends on the underlying cause. Some patients begin to recover between weeks 2 and 12 following onset and may continue to improve for up to two years. Other patients may never show signs of recovery.


Prognosis

The prognosis for TM depends on whether there is improvement in 3 to 6 months. Complete recovery is unlikely if no improvement occurs within this time. Incomplete recovery can still occur; however, aggressive physical therapy and rehabilitation will be very important. One-third of people with TM experience full recovery, one-third experience fair recovery but have significant neurological deficits, such as spastic gait. The final third experience no recovery at all.


Epidemiology

The incidence of TM is 4.6 per 1 million per year, affecting men and women equally. TM can occur at any age, but there are peaks around age 10, age 20, and after age 40.


History

The earliest reports describing the signs and symptoms of transverse myelitis were published in 1882 and 1910 by the English neurologist
Henry Bastian Henry may refer to: People *Henry (given name) *Henry (surname) * Henry Lau, Canadian singer and musician who performs under the mononym Henry Royalty * Portuguese royalty ** King-Cardinal Henry, King of Portugal ** Henry, Count of Portugal, ...
.{{cite book , vauthors = Dale RC, Vincent A , title=Inflammatory and Autoimmune Disorders of the Nervous System in Children, url=https://books.google.com/books?id=jwlt4t3GKj4C&pg=PA96, year=2010, publisher=John Wiley & Sons, isbn=978-1-898683-66-7, pages=96–106 In 1928, Frank Ford noted that in
mumps MUMPS ("Massachusetts General Hospital Utility Multi-Programming System"), or M, is an imperative, high-level programming language with an integrated transaction processing key–value database. It was originally developed at Massachusetts Gener ...
patients who developed acute myelitis, symptoms only emerged after the mumps infection and associated symptoms began to recede. In an article in
The Lancet ''The Lancet'' is a weekly peer-reviewed general medical journal and one of the oldest of its kind. It is also the world's highest-impact academic journal. It was founded in England in 1823. The journal publishes original research articles, ...
, Ford suggested that acute myelitis could be a post-infection syndrome in most cases (i.e. a result of the body's immune response attacking and damaging the spinal cord) rather than an infectious disease where a virus or some other infectious agent caused paralysis. His suggestion was consistent with reports in 1922 and 1923 of rare instances in which patients developed "post-vaccinal encephalomyelitis" subsequent to receiving the
rabies Rabies is a viral disease that causes encephalitis in humans and other mammals. Early symptoms can include fever and tingling at the site of exposure. These symptoms are followed by one or more of the following symptoms: nausea, vomiting, vi ...
vaccine A vaccine is a biological Dosage form, preparation that provides active acquired immunity to a particular infectious disease, infectious or cancer, malignant disease. The safety and effectiveness of vaccines has been widely studied and verifie ...
which then was made from brain tissue carrying the virus. The pathological examination of those who had died from the disease revealed inflammatory cells and demyelination as opposed to the vascular lesions predicted by Bastian. Ford's theory of an
allergic response An allergic response is a hypersensitive immune reaction to a substance that normally is harmless or would not cause an immune response in everyone. An allergic response may cause harmful symptoms such as itching or inflammation or tissue injury. ...
being at the root of the disease was later shown to be only partially correct, as some infectious agents such as mycoplasma,
measles Measles is a highly contagious infectious disease caused by measles virus. Symptoms usually develop 10–12 days after exposure to an infected person and last 7–10 days. Initial symptoms typically include fever, often greater than , cough, ...
and
rubella Rubella, also known as German measles or three-day measles, is an infection caused by the rubella virus. This disease is often mild, with half of people not realizing that they are infected. A rash may start around two weeks after exposure and ...
were isolated from the spinal fluid of some infected patients, suggesting that direct infection could contribute to the manifestation of acute myelitis in certain cases. In 1948, Dr. Suchett-Kaye described a patient with rapidly progressing impairment of lower extremity motor function that developed as a complication of pneumonia. In his description, he coined the term transverse myelitis to reflect the band-like thoracic area of altered sensation that patients reported. The term 'acute transverse myelopathy' has since emerged as an acceptable synonym for 'transverse myelitis', and the two terms are currently used interchangeably in the literature.{{cite journal , vauthors = Krishnan C, Kaplin AI, Deshpande DM, Pardo CA, Kerr DA , title = Transverse Myelitis: pathogenesis, diagnosis and treatment , journal = Frontiers in Bioscience , volume = 9 , issue = 1–3, pages = 1483–99 , date = May 2004 , pmid = 14977560 , doi = 10.2741/1351, url = https://www.bioscience.org/2004/v9/af/1351/2.htm The definition of transverse myelitis has also evolved over time. Bastian's initial description included few conclusive diagnostic criteria; by the 1980s, basic diagnostic criteria were established, including acutely developing paraparesis combined with bilateral spinal cord dysfunction over a period of <4 weeks and a well-defined upper sensory level, no evidence of spinal cord compression, and a stable, non-progressive course. Later definitions, were written in order to exclude patients with underlying systemic or neurological illnesses and to include only those who progressed to maximum deficit in fewer than 4 weeks.{{cite journal , vauthors = Christensen PB, Wermuth L, Hinge HH, Bømers K , title = Clinical course and long-term prognosis of acute transverse myelopathy , journal = Acta Neurologica Scandinavica , volume = 81 , issue = 5 , pages = 431–35 , date = May 1990 , pmid = 2375246 , doi = 10.1111/j.1600-0404.1990.tb00990.x , s2cid = 44660348


Society and culture

In 2016, former Slipknot drummer
Joey Jordison Nathan Jonas "Joey" Jordison (April 26, 1975 – July 26, 2021) was an American musician. He was the original drummer and co-founder of the heavy metal band Slipknot, in which he was designated #1, as well as the guitarist for the horror punk s ...
revealed that he had been hospitalised by the disease in 2013 and that this was the reason for his controversial firing. As the first celebrity to publicly speak about having transverse myelitis, this helped to raise public awareness of the disease. Jordison died in his sleep on July 26, 2021,{{Cite web , last=Atkinson , first=Katie , title=Ex-Slipknot Drummer Joey Jordison Dies at 46 , url=https://www.billboard.com/articles/news/obituary/9606945/joey-jordison-dead-slipknot-drummer , url-status=live , access-date=July 28, 2021 , website=Billboard.com however it is not known whether the disease had any connection to his death.


Etymology

The word is from Latin: ''myelitis transversa'' and the disorder's name is derived from Greek {{transliteration, el, ''myelós'' referring to the "
spinal cord The spinal cord is a long, thin, tubular structure made up of nervous tissue, which extends from the medulla oblongata in the brainstem to the lumbar region of the vertebral column (backbone). The backbone encloses the central canal of the spi ...
", and the suffix ''
-itis Inflammation (from la, inflammatio) is part of the complex biological response of body tissues to harmful stimuli, such as pathogens, damaged cells, or irritants, and is a protective response involving immune cells, blood vessels, and molec ...
'', which denotes
inflammation Inflammation (from la, wikt:en:inflammatio#Latin, inflammatio) is part of the complex biological response of body tissues to harmful stimuli, such as pathogens, damaged cells, or Irritation, irritants, and is a protective response involving im ...
.{{cite book , veditors=Chamberlin SL, Narins B , title = The Gale Encyclopedia of Neurological Disorders , url=https://archive.org/details/isbn_9780787691516 , url-access=registration , publisher = Thomson Gale , location = Detroit , year = 2005 , pages = 1859–70, isbn = 978-0-7876-9150-9


See also

* Acute disseminated encephalomyelitis


References

{{reflist, 32em


Further reading

{{refbegin * {{cite book, title = Essentials of Physical Medicine and Rehabilitation: Musculoskeletal Disorders, Pain, and Rehabilitation, url = https://books.google.com/books?id=FiMoQ-OtbB0C&q=transverse+myelitis+nih&pg=PA911, publisher = Elsevier Health Sciences, date = 2008, isbn = 978-1-4160-4007-1 , first1 = Walter R. , last1 = Frontera , first2 = Julie K. , last2 = Silver , first3 = Thomas D. , last3 = Rizzo , name-list-style = vanc * {{cite book , title = The Encyclopedia of Autoimmune Diseases , url = https://books.google.com/books?id=zKgMHabsTwMC , publisher = Infobase Publishing , date = 2003 , isbn = 978-1-4381-2094-2 , first1 = Dana K. , last1 = Cassell , first2 = Noel R. , last2 = Rose , name-list-style = vanc {{refend


External links

{{Medical resources , DiseasesDB = 13265 , ICD10 = {{ICD10, G, 37, 3, g, 35 , ICD9 = {{ICD9, 323.82, {{ICD9, 341.2 , ICDO = , OMIM = , MedlinePlus = , eMedicineSubj = , eMedicineTopic = , MeshID = D009188 {{Scholia, topic {{Spinal cord lesions {{Diseases of the nervous system {{Paraneoplastic syndromes Autoimmune diseases Syndromes Central nervous system disorders Multiple sclerosis Rare diseases Myelin disorders