Transverse myelitis

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Transverse myelitis
Classification and external resources
Transverse myelitis MRI.jpg
An MRI showing lesion of Transverse myelitis (the lesion is the lighter, oval shape at center-right), this MRI was taken 3 months after patient recovered
ICD-10 G37.3
ICD-9 323.82, 341.2
DiseasesDB 13265
MeSH D009188

Transverse myelitis (Latin: myelitis transversa) is a neurological condition consisting of an inflammatory process of the spinal cord. The inflammation can cause axonal demyelination. The name is derived from Greek myelós referring to the "spinal cord", and the suffix -itis, which denotes inflammation.[1] Transverse implies that the inflammation is across the thickness of the spinal cord.

Signs and symptoms[edit]

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 symptoms 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.

  • 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 diplegia.
  • A lesion of the lower part of the spinal cord (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 pain 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[citation needed].

However, within several days, this spinal shock will disappear and signs of spasticity will become evident[citation needed].

Causes[edit]

Transverse myelitis can appear for several reasons. Sometimes the disorders classified as such can be referred to as "Transverse myelitis spectrum disorders"[2]

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.[3]

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.

Another possible cause is dissection of the aorta, extending into one or more of the spinal arteries.

Transverse myelitis can be a rare complication following cat scratch disease.[4] As well as it can be associated with : Bacterial Infections-Mycoplasma pneumoniae, Lyme borreliosis, syphilis (tabes dorsalis), tuberculosis. Viral Infections-herpes simplex, herpes zoster, cytomegalovirus, Epstein-Barr virus, enteroviruses (poliomyelitis, Coxsackie virus, echovirus), human T-cell, leukemia virus, human immunodeficiency virus, influenza, rabies. Post-Vaccination-Rabies, cowpox. Multiple Sclerosis. Paraneoplastic syndromes. Vascular-Thrombosis of spinal arteries, vasculitis secondary to heroin abuse, spinal Arteriovenous malformations.

Pathophysiology[edit]

This demyelination arises idiopathically following infections or due to multiple sclerosis (and has been reported to occur following vaccination[5]). One major theory posits that immune-mediated inflammation is present as the result of exposure to a viral antigen. The diarrhea-causing bacteria Campylobacter jejuni is also a reported cause of transverse myelitis.[6]

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 they are usually restricted to only a small portion.

A special case is the "Longitudinally extensive transverse myelitis" (LETM) which is defined as a spinal cord lesion that extends over 3 or more vertebral segments.[7]

Differential diagnosis[edit]

The symptoms considered in the differential diagnosis 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.[8]

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-rays 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 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. Steroids 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.

The prognosis for significant recovery from acute transverse myelitis is poor in approximately 66% of the cases; that is, significant long-term disabilities will remain. Approximately 2% 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. [9]

Prognosis[edit]

Recovery from transverse myelitis 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 2 years. Other patients may never show signs of recovery.[10] However, if treated early, some patients experience complete or near complete recovery. Treatment options also vary according to the underlying cause and one treatment option in some cases is plasmapheresis.

Notable cases[edit]

See also[edit]

References[edit]

  1. ^ Chamberlin SL, Narins B, ed. (2005). The Gale Encyclopedia of Neurological Disorders. Detroit: Thomson Gale. pp. 1859–70. ISBN 0-7876-9150-X. 
  2. ^ Pandit L (Mar–Apr 2009). "Transverse myelitis spectrum disorders". Neurol India 57 (2): 126–33. doi:10.4103/0028-3886.51278. PMID 19439840. 
  3. ^ Blanc F, Froelich S, Vuillemet F, et al. (November 2007). "[Acute myelitis and Lyme disease]". Rev. Neurol. (Paris) (in French) 163 (11): 1039–47. PMID 18033042. 
  4. ^ Dr Thomas Stuttaford; Cat nipped;Body And Mind; The Times; 26 August 1993
  5. ^ Akkad W, Salem B, Freeman JW, Huntington MK (August 2010). "Longitudinally extensive transverse myelitis following vaccination with nasal attenuated novel influenza A(H1N1) vaccine". Arch. Neurol. 67 (8): 1018–20. doi:10.1001/archneurol.2010.167. PMID 20697056. 
  6. ^ Enteropathogens and Chronic Illness in Returning TravelersAllen G.P. Ross, M.D., Ph.D., et al. N Engl J Med 2013; 368:1817-1825May 9, 2013DOI: 10.1056/NEJMra1207777 http://www.nejm.org/doi/full/10.1056/NEJMra1207777
  7. ^ Juan Pablo C, Julia R, de Ory F, Clara de A., Longitudinally extensive varicella-zoster virus myelitis in a multiple sclerosis patient, Spine (Phila Pa 1976). 2013 Sep 15;38(20):E1282-4. doi: 10.1097/BRS.0b013e31829ecb98, PMID 24042721
  8. ^ Walid MS, Ajjan M, Ulm AJ (2008). "Subacute transverse myelitis with Lyme profile dissociation". Ger Med Sci 6: Doc04. PMC 2703261. PMID 19675732. 
  9. ^ Jeffery DR, Mandler RN, Davis LE (May 1993). "Transverse myelitis. Retrospective analysis of 33 cases, with differentiation of cases associated with multiple sclerosis and parainfectious events". Arch. Neurol. 50 (5): 532–5. doi:10.1001/archneur.1993.00540050074019. PMID 8489410. 
  10. ^ 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. Transverse Myelitis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS)
  11. ^ http://www.equestrianteamgbr.co.uk/rider.aspx?rider=Natasha-Baker
  12. ^ "Greg Ball Profile". Australian Paralympic Committee. 2008. Archived from the original on 17 September 2008. Retrieved 25 January 2012. 
  13. ^ "Amanda Carter". Australian Paralympic Committee. Retrieved 19 May 2012. 
  14. ^ Humphreys, Rod (14 November 1976). "A Hamburger and Onions with Gold". The Sydney Morning Herald. p. 38. Retrieved 22 February 2012. 
  15. ^ Breitrose, Charlie (July 3, 2008). "She is among the elite". Natick Bulletin and Tab. Retrieved 2008-10-16. 
  16. ^ Kristen Johnston Diagnosed with Lupus Myelitis
  17. ^ "Tiger JK’s English interview". 16 August 2009. Retrieved 19 June 2011. 
  18. ^ [Interview]http://americanmusicchannel.com/features/comversation_corner/4-24-2009/conversation-corner-hal-ketchum by American Music Channel: 04-24-09
  19. ^ Daily Mail, 14 August 1998, I know I'm mean: I refused to let my wife have a new dustbin
  20. ^ Sham, Brad (2008-07-28). "Former Center Rafferty Battling Disease". DallasCowboys.com. Retrieved 2008-11-01. 
  21. ^ California Literary Review
  22. ^ "Venter tackles his biggest challenge". International Rugby Board. 29 June 2007. Retrieved 19 June 2011. "A veteran of 66 Tests for South Africa, Venter's life changed dramatically last year when he contracted the rare disease Transverse Myelitis, an inflammation of the spinal cord that affects between one and five people in every million." 

External links[edit]