Transverse myelitis
Transverse myelitis | |
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Corticosteroids[2] |
Transverse myelitis (TM) is a rare
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
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
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
When it appears as a
Other causes of TM include infections, immune system disorders, and
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
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
Diagnostic criteria
In 2002, the Transverse Myelitis Consortium Working Group proposed the following diagnostic criteria for idiopathic acute transverse myelitis:[20]
- 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
- 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 systemic lupus erythematosus or mixed connective tissue disease
- Evidence of neuromyelitis optica(NMO))
- Evidence of infection ()
- Evidence of multiple sclerosis (abnormalities detected on MRI and presence of oligoclonal antibodies in cerebrospinal fluid (CSF))
- Irradiation of the spine (e.g.,
Investigations
Individuals who develop TM are typically transferred to a
Differential diagnosis
The
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
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
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
- ^ PMID 24099672.
- ^ a b c d "Transverse myelitis". Genetic and Rare Diseases Information Center (GARD) – an NCATS Program. Retrieved 3 January 2018.
- ISBN 978-3-662-08119-8.
- ^ PMID 22379456.
- ^ ISBN 978-1-898683-66-7.
- ISBN 978-81-8473-191-0.
- ISBN 978-1-62070-061-7.
- PMID 19439840.
- ^ "What is Transverse Myelitis (TM)? | Johns Hopkins Transverse Myelitis Center". Retrieved 2018-07-22.
- PMID 2380146.
- ^ 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.
- PMID 28889928.
- PMID 18033042.
- S2CID 13789364.
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- PMID 34327090.
- S2CID 205519782.
- PMID 26209588.
- S2CID 8356201.
- PMID 12236201.
- PMID 18256991.
- ISBN 978-1-139-50237-5.
- ^ "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.
- ISBN 978-1-60406-135-2.
- ^ 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.
- ^ 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.
- .
- ^ 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) - PMID 14977560.
- S2CID 42676273.
- S2CID 38183956.
- S2CID 44660348.
- ^ "Ex-Slipknot Drummer Reveals Struggle With Rare Disease: 'I Lost My Legs'". Billboard.com. Retrieved July 3, 2021.
- ^ Atkinson K. "Ex-Slipknot Drummer Joey Jordison Dies at 46". Billboard.com. Retrieved July 28, 2021.
- 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.