Transverse myelitis
Transverse myelitis (TM) is a rare neurological condition in which the
spinal cord is inflamed. Transverse implies that the inflammation extends
horizontally across the spinal cord.[1] Partial transverse myelitis and partial
myelitis are terms sometimes used to specify inflammation that only affects
part of the width of the spinal cord.[1] 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 that can lead to episodes of high blood pressure. 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 fibres, by loss of myelin.[1] As opposed to leukomyelitis
which affects only the white matter, it affects the entire cross-section of the
spinal cord.[3] Decreased electrical conductivity in the nervous system can
result.
Transverse myelitis
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 that can lead to
episodes of high blood pressure.[1] Symptoms typically develop over the
course of hours to a few weeks.[1][4] Sensory symptoms of TM may include a
sensation of pins and needles traveling up from the feet.[1] 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.[1]
Disturbances in sensory nerves and motor nerves and dysfunction of the
autonomic nervous system at the level of the lesion or below, are noted.
Therefore, the signs and symptoms depend on the area of the spine
involved.[5] Back pain can occur at the level of any inflamed segment of the
spinal cord.[1]
An MRI showing a transverse myelitis lesion, which is lighter, oval shape
at center-right. The patient recovered 3 months later.
Specialty
Neurology
Symptoms
Weakness of the limbs[1]
Causes
Uncertain[2]
Diagnostic method
Neurological exam[2]
Treatment
Corticosteroids[2]
If the upper cervical segment of the spinal cord is involved, all four limbs may be affected and there is risk of respiratory failure – the phrenic nerve
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 signs in the upper limbs, and exclusively
upper motor neuron signs in the lower limbs. Cervical lesions account for about 20% of cases.[5]
A lesion of the thoracic segment (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.[6]
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.[5]
Causes
TM is a heterogeneous condition, that is, there are several identified causes. Sometimes the term Transverse myelitis spectrum disorders is used.[7]
In 60% of patients the cause is idiopathic.[8] In rare cases, it may be associated with meningococcal meningitis[9]
When it appears as a comorbid condition with neuromyelitis optica (NMO), it is considered to be caused by NMO-IgG autoimmunity, and when it
appears in multiple sclerosis (MS) cases, it is considered to be produced by the same underlying condition that produces the MS plaques.
Other causes of TM include infections,
immune system disorders, and
demyelinating diseases.[10] Viral infections
known to be associated with TM include
HIV, herpes simplex, herpes zoster,
cytomegalovirus, and Epstein-Barr.[11]
Flavivirus infections such as Zika virus and
West Nile virus have also been associated.
Viral association of transverse myelitis
could result from the infection itself or
from the response to it.[10] Bacterial causes
associated with TM include Mycoplasma
Cytomegalovirus
pneumoniae, Bartonella henselae, and the
types of Borrelia that cause Lyme disease.
Lyme disease gives rise to neuroborreliosis which is seen in a small percentage (4 to 5 per cent)
of acute transverse myelitis cases.[12] The diarrhea-causing bacteria Campylobacter jejuni is also a
reported cause of transverse myelitis.[13]
Other associated causes include the helminth infection schistosomiasis, spinal cord injuries,
vascular disorders that impede the blood flow through vessels of the spinal cord, and
paraneoplastic syndrome.[10]
Borrelia burgdorferi spirochetes cause Lyme
disease and are one of many infections
associated with transverse myelitis.
Pathophysiology
This progressive loss of the fatty myelin sheath surrounding the nerves in the affected spinal cord occurs for unclear reasons following infections or
due to multiple sclerosis. Infections may cause TM through direct tissue damage or by immune-mediated infection-triggered tissue damage.[4] The
lesions present are usually inflammatory. Spinal cord involvement is usually central, uniform, and symmetric in comparison to multiple sclerosis
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.[4]
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.[14] The causes of LETM are also heterogeneous[15] and the presence of MOG autoantibodies has been proposed as a diagnostic biomarker.[16]
Diagnosis
Diagnostic criteria
In 2002, the Transverse Myelitis Consortium Working Group proposed the following
diagnostic criteria for idiopathic acute transverse myelitis:[17]
◾ 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, or elevated
immunoglobulin G, 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
Axial T2 MRI of cervical spine demonstrating
normal cord signal (green circle) and increased T2
signal in the central cord (red circle).
◾ Exclusion criteria
◾ Irradiation of the spine (e.g., radiotherapy) in the last 10 years
◾ Evidence of thrombosis of the anterior spinal artery
◾ Evidence of extra-axial compression on neuroimaging
◾ Evidence of arteriovenous malformation (abnormal flow voids on surface
of spine)
◾ Evidence of connective tissue disease, e.g. sarcoidosis, Behçet's disease,
Sjögren's syndrome, systemic lupus erythematosus or mixed connective
tissue disease
◾ Evidence of optic neuritis (diagnostic of neuromyelitis optica (NMO))
◾ Evidence of infection (syphilis, Lyme disease, Human immunodeficiency
virus, Human T-lymphotropic virus 1, mycoplasma, Herpes simplex virus,
Varicella-zoster virus, Epstein-Barr virus, cytomegalovirus, Human
herpesvirus 6 or enteroviruses)
◾ Evidence of multiple sclerosis (abnormalities detected on MRI and
presence of oligoclonal antibodies in cerebrospinal fluid (CSF))
Investigations
Individuals who develop TM are typically transferred to a neurologist who can urgently investigate the patient in a hospital. If breathing is affected,
particularly in upper spinal cord lesions, methods of artificial ventilation 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. A lumbar puncture can be performed after the MRI or at
the time of CT myelography. Corticosteroids 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.[2]
Differential diagnosis
The differential diagnosis of acute TM includes demyelinating disorders, such as multiple sclerosis and neuromyelitis optica, infections, such as
herpes zoster and herpes simplex virus, and other types of inflammatory disorders, such as systemic lupus erythematosus and neurosarcoidosis. It is
important to also rule out an acute cause of compression on the spinal cord.[18]
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.[19] 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.[20]
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.[10]
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.[21]
History
The earliest reports describing the signs and symptoms of transverse myelitis were published in 1882 and 1910 by the English neurologist Henry
Bastian.[5][22]
In 1928, Frank Ford noted that in mumps patients who developed acute myelitis, symptoms only emerged after the mumps infection and associated
vaccinal encephalomyelitis" subsequent to receiving the rabies vaccine which then was made from brain
tissue carrying the virus. The pathological examination of those who had succumbed to the disease revealed
inflammatory cells and demyelination as opposed to the vascular lesions predicted by Bastian.[23]
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 and rubella[24] 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.[5] 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.[25]
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
Henry Charlton Bastian
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.[26][27] 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.[28]
Society and culture
In 2016, former Slipknot drummer Joey Jordison revealed that he had been hospitalised by the disease in 2013 and that this was the reason for his
controversial firing.[29] 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. No cause of death was announced.[30]
Etymology
The word is from Latin: myelitis transversa and the disorder's name is derived from Greek myelós referring to the "spinal cord", and the suffix -itis,
which denotes inflammation.[31]
See also
◾ Acute disseminated encephalomyelitis
References
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Knecht, S. (ed.). Klinische Neurologie. pp. 485–96. doi:10.1007/9783-662-08118-1_21. ISBN 978-3-662-08119-8.
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18. ^ Jacob A, Weinshenker BG (February 2008). "An approach to the
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19. ^ Cohen JA, Rudick RA (2011). Multiple Sclerosis Therapeutics.
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20. ^ "Transverse Myelitis Fact Sheet". National Institute of Neurological
Disorders and Stroke (NINDS). "About one-third of patients do not
recover at all: These patients are often wheelchair-bound or bedridden,
with marked dependence on others for basic functions of daily living."
21. ^ Mumenthaler M, Mattle H (2011). Neurology. Thieme. ISBN 978-160406-135-2.
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"Acute transverse myelitis: incidence and etiologic considerations".
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27. ^ Ropper AH, Poskanzer DC (July 1978). "The prognosis of acute and
subacute transverse myelopathy based on early signs and symptoms".
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28. ^ Christensen PB, Wermuth L, Hinge HH, Bømers K (May 1990).
"Clinical course and long-term prognosis of acute transverse
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29. ^ "Ex-Slipknot Drummer Reveals Struggle With Rare Disease: 'I Lost
My Legs' ". Billboard.com. Retrieved July 3, 2021.
30. ^ Atkinson, Katie. "Ex-Slipknot Drummer Joey Jordison Dies at 46".
Billboard.com. Retrieved July 28, 2021.
31. ^ Chamberlin SL, Narins B, eds. (2005). The Gale Encyclopedia of
Neurological Disorders. Detroit: Thomson Gale. pp. 1859–70.
ISBN 978-0-7876-9150-9.
Further reading
◾ Frontera WR, Silver JK, Rizzo TD (2008). Essentials of Physical Medicine and Rehabilitation: Musculoskeletal Disorders, Pain, and Rehabilitation.
Elsevier Health Sciences. ISBN 978-1-4160-4007-1.
◾ Cassell DK, Rose NR (2003). The Encyclopedia of Autoimmune Diseases. Infobase Publishing. ISBN 978-1-4381-2094-2.
External links
Classification
ICD-10: G37.3 · ICD-9-CM: 323.82, 341.2 · MeSH: D009188 · DiseasesDB: 13265
D