Transverse myelitis

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Transverse myelitis
Corticosteroids[2]

Transverse myelitis (TM) is a rare

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.[citation needed
]

Signs and symptoms

Symptoms include weakness and numbness of the limbs, deficits in sensation and motor skills, dysfunctional

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]

If the upper

C4, and C5 innervates the main muscle of respiration, the diaphragm.[6]

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

paraparesis. This is the most common location of the lesion, and therefore most individuals will have weakness in the lower limbs.[7]

A lesion of the lumbar segment, 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.[5]

Causes

spirochetes cause Lyme disease
and are one of many infections associated with transverse myelitis.
Cytomegalovirus

TM is a

meningococcal meningitis[10]

When it appears as a

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.[citation needed
]

Other causes of TM include infections, immune system disorders, and

Epstein-Barr.[12] 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.[11] Bacterial causes associated with TM include Mycoplasma 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.[13] The diarrhea-causing bacteria Campylobacter jejuni is also a reported cause of transverse myelitis.[14]

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.[11] Another exceptionally rare cause is heroin associated transverse myelitis.[15][16]

Pathophysiology

This

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. 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.[17] The causes of LETM are also heterogeneous[18] and the presence of MOG auto-antibodies has been proposed as a diagnostic biomarker.[19]

Diagnosis

Axial T2 MRI of cervical spine demonstrating normal cord signal (green circle) and increased T2 signal in the central cord (red circle).

Diagnostic criteria

In 2002, the Transverse Myelitis Consortium Working Group proposed the following diagnostic criteria for idiopathic acute transverse myelitis:[20]

Investigations

Individuals who develop TM are typically transferred to a

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

systemic lupus erythematosus and neurosarcoidosis. It is important to also rule out an acute cause of compression on the spinal cord.[21]

Treatment

If treated early, some people experience a complete or near complete recovery. Treatment options also vary according to the underlying cause. One treatment option includes plasmapheresis.[22] 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.[23]

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.[11]

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.[24]

History

Henry Charlton Bastian

The earliest reports describing the signs and symptoms of transverse myelitis were published in 1882 and 1910 by the English neurologist Henry Bastian.[5][25]

In 1928, Frank Ford noted that in mumps patients who developed acute myelitis, symptoms only emerged after the mumps infection and associated symptoms began to recede. In an article in The Lancet, 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 vaccine 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.[26]

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[27] 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.[28]

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.[29]

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 for <4 weeks and a well-defined upper sensory level, no evidence of spinal cord compression, and a stable, non-progressive course.[30][31] Later definitions, were written to exclude patients with underlying systemic or neurological illnesses and to include only those who progressed to maximum deficit in fewer than 4 weeks.[32]

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.[33] 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,[34]
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 myelós referring to the "spinal cord", and the suffix -itis, which denotes inflammation.[35]

See also

References

  1. ^
    PMID 24099672
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  2. ^ a b c d "Transverse myelitis". Genetic and Rare Diseases Information Center (GARD) – an NCATS Program. Retrieved 3 January 2018.
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  9. ^ "What is Transverse Myelitis (TM)? | Johns Hopkins Transverse Myelitis Center". Retrieved 2018-07-22.
  10. PMID 2380146
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  11. ^ a b c d "Transverse Myelitis Fact Sheet". National Institute of Neurological Disorders and Stroke (NINDS). Archived from the original on 2016-11-23. Retrieved 2015-08-06.
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  23. ^ "Transverse Myelitis Fact Sheet". National Institute of Neurological Disorders and Stroke (NINDS). Archived from the original on 2016-11-23. Retrieved 2007-09-16. 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.
  24. .
  25. ^ Quain R, ed. (1882). A Dictionary of Medicine: Including General Pathology, General Therapeutics, Hygiene, and the Diseases Peculiar to Women and Children. Vol. 2. Longmans, Green, and Company. pp. 1479–83.
  26. ^ Kerr D. "The History of TM: The Origins of the Name and the Identification of the Disease". The Transverse Myelitis Association. Retrieved 2018-07-22.
  27. .
  28. ^ Douglas, Kerr, MD, PhD (September 1, 1998). "The History of TM: The Origins of the Name and the Identification of the Disease, Disorders: Spinal Stroke or AVM, Transverse Myelitis".{{cite web}}: CS1 maint: multiple names: authors list (link)
  29. PMID 14977560
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  33. ^ "Ex-Slipknot Drummer Reveals Struggle With Rare Disease: 'I Lost My Legs'". Billboard.com. Retrieved July 3, 2021.
  34. ^ Atkinson K. "Ex-Slipknot Drummer Joey Jordison Dies at 46". Billboard.com. Retrieved July 28, 2021.
  35. .

Further reading