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
Myelitis is inflammation of the spinal cord which can disrupt the normal responses from the brain to the rest of the body, and from the rest of the body to the brain. Inflammation in the spinal cord, can cause the myelin and axon to be damaged re ...
) 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
A nerve is an enclosed, cable-like bundle of nerve fibers (called axons) in the peripheral nervous system.
A nerve transmits electrical impulses. It is the basic unit of the peripheral nervous system. A nerve provides a common pathway for the e ...
, 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
The urethra (from Greek οὐρήθρα – ''ourḗthrā'') is a tube that connects the urinary bladder to the urinary meatus for the removal of urine from the body of both females and males. In human females and other primates, the urethra con ...
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
A dorsal root ganglion (or spinal ganglion; also known as a posterior root ganglion) is a cluster of neurons (a ganglion) in a dorsal root of a spinal nerve. The cell bodies of sensory neurons known as first-order neurons are located in the dorsal ...
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
The pyramidal tracts include both the corticobulbar tract and the corticospinal tract. These are aggregations of efferent nerve fibers from the upper motor neurons that travel from the cerebral cortex and terminate either in the brainstem (''cort ...
and mainly affects the muscles that
flex the legs and
extend the arms.
Disturbances in
sensory nerve
A sensory nerve, or afferent nerve, is a general anatomic term for a nerve which contains predominantly somatic afferent nerve fibers. Afferent nerve fibers in a sensory nerve carry sensory information toward the central nervous system (CNS) fr ...
s and
motor nerve
A motor nerve is a nerve that transmits motor signals from the central nervous system (CNS) to the muscles of the body. This is different from the motor neuron, which includes a cell body and branching of dendrites, while the nerve is made up of ...
s 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
Respiratory failure results from inadequate gas exchange by the respiratory system, meaning that the arterial oxygen, carbon dioxide, or both cannot be kept at normal levels. A drop in the oxygen carried in the blood is known as hypoxemia; a rise ...
– 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.
Lesions of the lower cervical region (C5–T1) will cause a combination of
upper and
lower motor neuron
Lower motor neurons (LMNs) are motor neurons located in either the anterior grey column, anterior nerve roots (spinal lower motor neurons) or the cranial nerve nuclei of the brainstem and cranial nerves with motor function (cranial nerve lower mo ...
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
Upper motor neurons (UMNs) is a term introduced by William Gowers in 1886. They are found in the cerebral cortex and brainstem and carry information down to activate interneurons and lower motor neurons, which in turn directly signal muscles t ...
signs in the lower limbs, presenting as a
spastic paraparesis
Paraplegia, or paraparesis, is an impairment in motor or sensory function of the lower extremities. The word comes from Ionic Greek ()
"half-stricken". It is usually caused by spinal cord injury or a congenital condition that affects the neura ...
. 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 lower part of the spinal cord (
L1–
S5) often produces a combination of upper and lower motor neuron signs in the lower limbs. Lumbar lesions account for about 10% of cases.
Causes
![Cytomegalovirus 01](https://upload.wikimedia.org/wikipedia/commons/a/ab/Cytomegalovirus_01.jpg)
TM is a
heterogeneous condition, that is, there are several identified causes. Sometimes the term ''Transverse myelitis spectrum disorders'' is used. In 60% of patients the cause is
idiopathic
An idiopathic disease is any disease with an unknown cause or mechanism of apparent wikt:spontaneous, spontaneous origin. From Ancient Greek, Greek ἴδιος ''idios'' "one's own" and πάθος ''pathos'' "suffering", ''idiopathy'' means approxi ...
. 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 ...
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 ...
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 disease
A demyelinating disease is any disease of the nervous system in which the myelin sheath of neurons is damaged. This damage impairs the conduction of signals in the affected nerves. In turn, the reduction in conduction ability causes deficiency i ...
s.
[{{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 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 simplex is a viral infection caused by the herpes simplex virus. Infections are categorized based on the part of the body infected.
Oral herpes involves the face or mouth. It may result in small blisters in groups often called cold ...
,
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
''Mycoplasma pneumoniae'' is a very small bacterium in the class Mollicutes. It is a human pathogen that causes the disease mycoplasma pneumonia, a form of atypical bacterial pneumonia related to cold agglutinin disease. ''M. pneumoniae'' is char ...
'', ''
Bartonella henselae
''Bartonella henselae'', formerly ''Rochalimæa henselae'', is a bacterium that is the causative agent of cat-scratch disease ( bartonellosis).
''Bartonella henselae'' is a member of the genus ''Bartonella'', one of the most common types of bacte ...
'', and the types of ''
Borrelia
''Borrelia'' is a genus of bacteria of the spirochete phylum. Several species cause Lyme disease, also called Lyme borreliosis, a zoonotic, vector-borne disease transmitted by ticks. Other species of ''Borrelia'' cause relapsing fever, and are tr ...
'' 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
''Campylobacter jejuni'' () is a species of pathogenic bacteria, one of the most common causes of food poisoning in Europe and in the US. The vast majority of cases occur as isolated events, not as part of recognized outbreaks. Active surveillan ...
'' 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 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
In medicine, pleocytosis (or pleiocytosis) is an increased cell count (from Greek ''pleion'', "more"), particularly an increase in white blood cell count, in a bodily fluid, such as cerebrospinal fluid. It is often defined specifically as an increa ...
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
Gadolinium is a chemical element with the symbol Gd and atomic number 64. Gadolinium is a silvery-white metal when oxidation is removed. It is only slightly malleable and is a ductile rare-earth element. Gadolinium reacts with atmospheric oxygen ...
-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
A connective tissue disease (collagenosis) is any disease that has the connective tissues of the body as a target of pathology. Connective tissue is any type of biological tissue with an extensive extracellular matrix that supports, binds togeth ...
, 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
Behçet's disease (BD) is a type of inflammatory disorder which affects multiple parts of the body. The most common symptoms include painful sores on the mucous membranes of the mouth and other parts of the body, inflammation of parts of the e ...
,
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
Optic neuritis describes any condition that causes inflammation of the optic nerve; it may be associated with demyelinating diseases, or infectious or inflammatory processes.
It is also known as optic papillitis (when the head of the optic nerv ...
(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
The human immunodeficiency viruses (HIV) are two species of ''Lentivirus'' (a subgroup of retrovirus) that infect humans. Over time, they cause AIDS, acquired immunodeficiency syndrome (AIDS), a condition in which progressive failure of the ...
,
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 diseas ...
,
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
Varicella-zoster virus (VZV), also known as human herpesvirus 3 (HHV-3, HHV3) or ''Human alphaherpesvirus 3'' (taxonomically), is one of nine known herpes viruses that can infect humans. It causes chickenpox (varicella) commonly affecting chil ...
,
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
Human herpesvirus 6 (HHV-6) is the common collective name for ''human betaherpesvirus 6A'' (HHV-6A) and ''human betaherpesvirus 6B'' (HHV-6B). These closely related viruses are two of the nine known herpesviruses that have humans as their primar ...
or
enterovirus
''Enterovirus'' is a genus of positive-sense single-stranded RNA virus
An RNA virus is a virusother than a retrovirusthat has ribonucleic acid (RNA) as its genetic material. The nucleic acid is usually single-stranded RNA ( ssRNA) but it ma ...
es)
** 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
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 c ...
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
catheter
In medicine, a catheter (/ˈkæθətər/) is a thin tube made from medical grade materials serving a broad range of functions. Catheters are medical devices that can be inserted in the body to treat diseases or perform a surgical procedure. Cath ...
ized 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
Myelography is a type of radiographic examination that uses a contrast medium to detect pathology of the spinal cord, including the location of a spinal cord injury, cysts, and tumors. Historically the procedure involved the injection of a radioco ...
.
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 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
![PSM V08 D008 Henry Charlton Bastian](https://upload.wikimedia.org/wikipedia/commons/0/0f/PSM_V08_D008_Henry_Charlton_Bastian.jpg)
The earliest reports describing the signs and symptoms of transverse myelitis were published in 1882 and 1910 by the English neurologist
Henry Bastian.
[{{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 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
Acute disseminated encephalomyelitis (ADEM), or acute Demyelinating disease, demyelinating encephalomyelitis, is a rare autoimmune disease marked by a sudden, widespread attack of inflammation in the brain and spinal cord. As well as causing the ...
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