Transverse myelitis
Encyclopedia
Transverse myelitis is a neurological disorder caused by an inflammatory
process of the spinal cord
, and can cause axonal demyelination. The name is derived from Greek referring to the "spinal cord
", and the suffix -itis, which denotes inflammation
. Transverse implies that the inflammation is across the thickness of the spinal cord.
ally following infection
s or vaccination
, or due to multiple sclerosis
.
One major theory posits that immune-mediated inflammation is present as the result of exposure to a viral
antigen
.
The lesions are inflammatory
, and involve the spinal cord
typically on both sides.
With acute transverse myelitis, the onset is sudden and progresses rapidly in hours and days.
The lesions can be present anywhere in the spinal cord, though it is usually restricted to only a small portion.
In some cases, the disease is presumed to be caused by viral infections such as cytomegalovirus
(CMV) and has also been associated with spinal cord injuries, immune reactions, schistosomiasis
and insufficient blood flow through spinal cord vessels. Acute myelitis accounts for 4 to 5 percent of all cases of neuroborreliosis
.
A major differentiation or distinction to be made is a similar condition due to compression of the spinal cord in the spinal canal, due to disease of the surrounding vertebral column. An urgent MRI is thus indicated.
Another possible cause is dissection of the Aorta, extending into one or more of the spinal arteries.
TM can be a rare complication following cat scratch disease
.
Some patients may never show signs of recovery. However, if treated early, some patients experience complete or near complete recovery.
Treatment is usually symptomatic only, corticosteroids being used with limited success.
s and signs depend upon the level of the spinal cord involved and the extent of the involvement of the various long tracts. In some cases, there is almost total paralysis
and sensory loss below the level of the lesion. In other cases, such loss is only partial.
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 sensory segmental level of the spinal cord below which sensation to pin or light touch is impaired). This has proven to be a reasonably reliable sign of the level of the lesion. Bladder paralysis often occurs and urinary retention is an early manifestation. Considerable pain often occurs in the back, extending laterally to involve the sensory distribution of the diseased spinal segments—so-called "radicular pain." Thus, a lesion at the T8 level will produce pain radiating from the spine laterally along the lower costal margins. These signs and symptoms may progress to severe weakness within hours. (Because of the acuteness of this lesion, signs of spinal shock
may be evident, in which the lower limbs will be flaccid and areflexic, rather than spastic and hyperreflexic as they should be in upper motor neuron paralysis.
Some patients have also described the feeling of their abdominal area being in a binder.
However, within several days, this spinal shock will disappear and signs of spasticity
will become evident.
are: acute spinal cord trauma, acute compressive lesions of the spinal cord such as epidural metastatic tumour, and infarction
of the spinal cord, usually due to insufficiency of the anterior spinal artery
. Lyme disease serology is indicated in patients with transverse myelitis keeping in mind that dissociation in Lyme antibody titers between the blood and the CSF is possible.
From the symptoms and signs, it may be very difficult to distinguish acute transverse myelitis from these conditions and it is almost invariably necessary to perform an emergency magnetic resonance imaging
(MRI) scan or computerised tomographic (CT
) myelogram. Before doing this, routine x-ray
s are taken of the entire spine, mainly to detect signs of metastatic disease of the vertebrae, that would imply direct extension into the epidural
space and compression of the spinal cord. Often, such bony lesions are absent and it is only the MRI or CT that discloses the presence or absence of a compressive lesion.
A family physician seeing such a patient for the first time should immediately arrange transfer to the care of a neurologist
or neurosurgeon who can urgently investigate the patient in hospital. Before arranging this transfer, the physician should be certain that respiration
is not affected, particularly in high spinal cord lesions. If there is any evidence of this, methods of respiratory assistance must be on hand before and during the transfer procedure. The patient should also be catheter
ized to test for and, if necessary, drain an over-distended bladder. A lumbar puncture
can be performed after the MRI or at the time of CT myelography. Steroid
s are often given in high dose at the onset, in hope that the degree of inflammation
and swelling of the cord will be lessened, but whether this is truly effective is still debated.
Unfortunately, the prognosis for significant recovery from acute transverse myelitis is poor in approximately 80% of the cases; that is, significant long-term disabilities will remain. Approximately 5% of these patients will, in later months or years, show lesions in other parts of the central nervous system, indicating, in retrospect, that this was a first attack of multiple sclerosis
.
Inflammation
Inflammation is part of the complex biological response of vascular tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. Inflammation is a protective attempt by the organism to remove the injurious stimuli and to initiate the healing process...
process of the spinal cord
Spinal cord
The spinal cord is a long, thin, tubular bundle of nervous tissue and support cells that extends from the brain . The brain and spinal cord together make up the central nervous system...
, and can cause axonal demyelination. The name is derived from Greek referring to the "spinal cord
Spinal cord
The spinal cord is a long, thin, tubular bundle of nervous tissue and support cells that extends from the brain . The brain and spinal cord together make up the central nervous system...
", and the suffix -itis, which denotes inflammation
Inflammation
Inflammation is part of the complex biological response of vascular tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. Inflammation is a protective attempt by the organism to remove the injurious stimuli and to initiate the healing process...
. Transverse implies that the inflammation is across the thickness of the spinal cord.
Presentation
This demyelination arises idiopathicIdiopathic
Idiopathic is an adjective used primarily in medicine meaning arising spontaneously or from an obscure or unknown cause. From Greek ἴδιος, idios + πάθος, pathos , it means approximately "a disease of its own kind". It is technically a term from nosology, the classification of disease...
ally following infection
Infection
An infection is the colonization of a host organism by parasite species. Infecting parasites seek to use the host's resources to reproduce, often resulting in disease...
s or vaccination
Vaccination
Vaccination is the administration of antigenic material to stimulate the immune system of an individual to develop adaptive immunity to a disease. Vaccines can prevent or ameliorate the effects of infection by many pathogens...
, or due to multiple sclerosis
Multiple sclerosis
Multiple sclerosis is an inflammatory disease in which the fatty myelin sheaths around the axons of the brain and spinal cord are damaged, leading to demyelination and scarring as well as a broad spectrum of signs and symptoms...
.
One major theory posits that immune-mediated inflammation is present as the result of exposure to a viral
Virus
A virus is a small infectious agent that can replicate only inside the living cells of organisms. Viruses infect all types of organisms, from animals and plants to bacteria and archaea...
antigen
Antigen
An antigen is a foreign molecule that, when introduced into the body, triggers the production of an antibody by the immune system. The immune system will then kill or neutralize the antigen that is recognized as a foreign and potentially harmful invader. These invaders can be molecules such as...
.
The lesions are inflammatory
Inflammation
Inflammation is part of the complex biological response of vascular tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. Inflammation is a protective attempt by the organism to remove the injurious stimuli and to initiate the healing process...
, and involve the spinal cord
Spinal cord
The spinal cord is a long, thin, tubular bundle of nervous tissue and support cells that extends from the brain . The brain and spinal cord together make up the central nervous system...
typically on both sides.
With acute transverse myelitis, the onset is sudden and progresses rapidly in hours and days.
The lesions can be present anywhere in the spinal cord, though it is usually restricted to only a small portion.
Causes
Transverse myelitis can appear for several reasons. Sometimes they can be referred as "Transverse myelitis spectrum disorders"In some cases, the disease is presumed to be caused by viral infections such as cytomegalovirus
Cytomegalovirus
Cytomegalovirus is a viral genus of the viral group known as Herpesviridae or herpesviruses. It is typically abbreviated as CMV: The species that infects humans is commonly known as human CMV or human herpesvirus-5 , and is the most studied of all cytomegaloviruses...
(CMV) and has also been associated with spinal cord injuries, immune reactions, schistosomiasis
Schistosomiasis
Schistosomiasis is a parasitic disease caused by several species of trematodes , a parasitic worm of the genus Schistosoma. Snails often act as an intermediary agent for the infectious diseases until a new human host is found...
and insufficient blood flow through spinal cord vessels. Acute myelitis accounts for 4 to 5 percent of all cases of neuroborreliosis
Neuroborreliosis
Neuroborreliosis is a disorder of the central nervous system caused by infection with a spirochete of the genus Borrelia. The microbiological progression of the disease is similar to that of neurosyphilis, another spirochetal infection...
.
A major differentiation or distinction to be made is a similar condition due to compression of the spinal cord in the spinal canal, due to disease of the surrounding vertebral column. An urgent MRI is thus indicated.
Another possible cause is dissection of the Aorta, extending into one or more of the spinal arteries.
TM can be a rare complication following cat scratch disease
Cat scratch disease
Cat scratch disease is a usually benign infectious disease caused by the intracellular bacterium Bartonella. It is most commonly found in children following a scratch or bite from a cat by about one to two weeks...
.
Prognosis
Recovery from transverse myelitis usually begins between weeks 2 and 12 following onset and may continue for up to 2 years in some patients.Some patients may never show signs of recovery. However, if treated early, some patients experience complete or near complete recovery.
Treatment is usually symptomatic only, corticosteroids being used with limited success.
Symptoms and signs
Symptoms include weakness and numbness of the limbs as well as motor, sensory, and sphincter deficits. Severe back pain may occur in some patients at the onset of the disease. The symptomSymptom
A symptom is a departure from normal function or feeling which is noticed by a patient, indicating the presence of disease or abnormality...
s and signs depend upon the level of the spinal cord involved and the extent of the involvement of the various long tracts. In some cases, there is almost total paralysis
Paralysis
Paralysis is loss of muscle function for one or more muscles. Paralysis can be accompanied by a loss of feeling in the affected area if there is sensory damage as well as motor. A study conducted by the Christopher & Dana Reeve Foundation, suggests that about 1 in 50 people have been diagnosed...
and sensory loss below the level of the lesion. In other cases, such loss is only partial.
- If the upper cervical cord is involved, all four limbs may be involved and there is risk of respiratory paralysis (segments C3,4,5 to diaphragm).
- Lesions of the lower cervical (C5-T1) region 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.
- A lesion of the thoracic spinal cord (T1-12) will produce upper motor neuron signs in the lower limbs, presenting as a spastic diplegiaSpastic diplegiaSpastic diplegia, historically known as Little's Disease, is a form of cerebral palsy that is a neuromuscular condition of hypertonia and spasticity in the muscles of the lower extremities of the human body, usually those of the legs, hips and pelvis...
. - A lesion of the lower part of the spinal cordSpinal cordThe spinal cord is a long, thin, tubular bundle of nervous tissue and support cells that extends from the brain . The brain and spinal cord together make up the central nervous system...
(L1-S5) often produces a combination of upper and lower motor neuron signs in the lower limbs.
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 sensory segmental level of the spinal cord below which sensation to pin or light touch is impaired). This has proven to be a reasonably reliable sign of the level of the lesion. Bladder paralysis often occurs and urinary retention is an early manifestation. Considerable pain often occurs in the back, extending laterally to involve the sensory distribution of the diseased spinal segments—so-called "radicular pain." Thus, a lesion at the T8 level will produce pain radiating from the spine laterally along the lower costal margins. These signs and symptoms may progress to severe weakness within hours. (Because of the acuteness of this lesion, signs of spinal shock
Spinal shock
Spinal shock was first defined by Whytt in 1750 as a loss of sensation accompanied by motor paralysis with initial loss but gradual recovery of reflexes, following a spinal cord injury – most often a complete transection. Reflexes in the spinal cord caudal to the SCI are depressed or absent ,...
may be evident, in which the lower limbs will be flaccid and areflexic, rather than spastic and hyperreflexic as they should be in upper motor neuron paralysis.
Some patients have also described the feeling of their abdominal area being in a binder.
However, within several days, this spinal shock will disappear and signs of spasticity
Spasticity
Spasticity is a feature of altered skeletal muscle performance in muscle tone involving hypertonia, which is also referred to as an unusual "tightness" of muscles...
will become evident.
Differential diagnosis
The three main conditions to be considered in the differential diagnosisDifferential diagnosis
A differential diagnosis is a systematic diagnostic method used to identify the presence of an entity where multiple alternatives are possible , and may also refer to any of the included candidate alternatives A differential diagnosis (sometimes abbreviated DDx, ddx, DD, D/Dx, or ΔΔ) is a...
are: acute spinal cord trauma, acute compressive lesions of the spinal cord such as epidural metastatic tumour, and infarction
Infarction
In medicine, infarction refers to tissue death that is caused by a local lack of oxygen due to obstruction of the tissue's blood supply. The resulting lesion is referred to as an infarct.-Causes:...
of the spinal cord, usually due to insufficiency of the anterior spinal artery
Anterior spinal artery
In human anatomy, the anterior spinal artery is the blood vessel that supplies the anterior portion of the spinal cord. It arises from branches of the vertebral arteries and is supplied by the anterior segmental medullary arteries, including the artery of Adamkiewicz, and courses along the anterior...
. Lyme disease serology is indicated in patients with transverse myelitis keeping in mind that dissociation in Lyme antibody titers between the blood and the CSF is possible.
From the symptoms and signs, it may be very difficult to distinguish acute transverse myelitis from these conditions and it is almost invariably necessary to perform an emergency magnetic resonance imaging
Magnetic resonance imaging
Magnetic resonance imaging , nuclear magnetic resonance imaging , or magnetic resonance tomography is a medical imaging technique used in radiology to visualize detailed internal structures...
(MRI) scan or computerised tomographic (CT
Computed tomography
X-ray computed tomography or Computer tomography , is a medical imaging method employing tomography created by computer processing...
) myelogram. Before doing this, routine x-ray
X-ray
X-radiation is a form of electromagnetic radiation. X-rays have a wavelength in the range of 0.01 to 10 nanometers, corresponding to frequencies in the range 30 petahertz to 30 exahertz and energies in the range 120 eV to 120 keV. They are shorter in wavelength than UV rays and longer than gamma...
s are taken of the entire spine, mainly to detect signs of metastatic disease of the vertebrae, that would imply direct extension into the epidural
Epidural
The term epidural is often short for epidural analgesia, a form of regional analgesia involving injection of drugs through a catheter placed into the epidural space...
space and compression of the spinal cord. Often, such bony lesions are absent and it is only the MRI or CT that discloses the presence or absence of a compressive lesion.
A family physician seeing such a patient for the first time should immediately arrange transfer to the care of a neurologist
Neurologist
A neurologist is a physician who specializes in neurology, and is trained to investigate, or diagnose and treat neurological disorders.Neurology is the medical specialty related to the human nervous system. The nervous system encompasses the brain, spinal cord, and peripheral nerves. A specialist...
or neurosurgeon who can urgently investigate the patient in hospital. Before arranging this transfer, the physician should be certain that respiration
Respiration (physiology)
'In physiology, respiration is defined as the transport of oxygen from the outside air to the cells within tissues, and the transport of carbon dioxide in the opposite direction...
is not affected, particularly in high spinal cord lesions. If there is any evidence of this, methods of respiratory assistance must be on hand before and during the transfer procedure. The patient should also be catheter
Catheter
In medicine, a catheter is a tube that can be inserted into a body cavity, duct, or vessel. Catheters thereby allow drainage, administration of fluids or gases, or access by surgical instruments. The process of inserting a catheter is catheterization...
ized to test for and, if necessary, drain an over-distended bladder. A lumbar puncture
Lumbar puncture
A lumbar puncture is a diagnostic and at times therapeutic procedure that is performed in order to collect a sample of cerebrospinal fluid for biochemical, microbiological, and cytological analysis, or very rarely as a treatment to relieve increased intracranial pressure.-Indications:The...
can be performed after the MRI or at the time of CT myelography. Steroid
Steroid
A steroid is a type of organic compound that contains a characteristic arrangement of four cycloalkane rings that are joined to each other. Examples of steroids include the dietary fat cholesterol, the sex hormones estradiol and testosterone, and the anti-inflammatory drug dexamethasone.The core...
s are often given in high dose at the onset, in hope that the degree of inflammation
Inflammation
Inflammation is part of the complex biological response of vascular tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. Inflammation is a protective attempt by the organism to remove the injurious stimuli and to initiate the healing process...
and swelling of the cord will be lessened, but whether this is truly effective is still debated.
Unfortunately, the prognosis for significant recovery from acute transverse myelitis is poor in approximately 80% of the cases; that is, significant long-term disabilities will remain. Approximately 5% of these patients will, in later months or years, show lesions in other parts of the central nervous system, indicating, in retrospect, that this was a first attack of multiple sclerosis
Multiple sclerosis
Multiple sclerosis is an inflammatory disease in which the fatty myelin sheaths around the axons of the brain and spinal cord are damaged, leading to demyelination and scarring as well as a broad spectrum of signs and symptoms...
.
Notable sufferers
- Anjali Forber-PrattAnjali Forber-PrattAnjali Forber-Pratt is an American wheelchair racer who competes in sprint events at the Paralympic level.Raised in Natick, Massachusetts, Forber-Pratt was diagnosed with transverse myelitis at four and a half months of age. The disorder left her paralyzed from the waist down...
- Stephen Morris and Gillian GilbertGillian GilbertGillian Lesley Gilbert is an English musician, keyboardist, guitarist and singer, best known as a member of New Order and a founding member of The Other Two.-Biography:...
's daughter, Grace - Akua Lezli HopeAkua Lezli HopeAkua Lezli Hope is an African American woman artist, poet and writer. Akua is a third generation New Yorker. She was born in Manhattan and grew up in the South Bronx and Queens. Akua has degrees from Williams College and Columbia University in psychology, journalism and business. Akua is a talented...
- Tiger JKTiger JKSeo Jung-kwon better known by his stage name Tiger JK is a Korean American rapper, producer, songwriter, enterprenuer, and founding member of Drunken Tiger. He is the founder and former CEO of Jungle Entertainment an imprint distributed by CJ E&M...
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- Allen RuckerAllen RuckerAllen Rucker is an American writer and author. Born in Wichita Falls, Texas, and raised in Bartlesville, Oklahoma, he earned a B.A. from Washington University in St. Louis , an M.A. in American Culture from the University of Michigan , and another M.A...
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- André Venter
- Cody Unser, daughter of Al Unser Jr. and granddaughter of Al UnserAl UnserAlfred "Al" Unser is a former American automobile racing driver, the younger brother of fellow racing drivers Jerry and Bobby Unser, and father of Al Unser, Jr....