Transverse myelitis

Transverse myelitis
Transverse myelitis
Classification and external resources

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 (in Latin nomenclature: myelitis transversa) is a neurological disorder caused by an inflammatory process of the spinal cord, and can cause axonal demyelination. The name is derived from Greek myelón 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.

Contents

Presentation

This demyelination arises idiopathically following infections or vaccination,[2] 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.

Causes

Transverse myelitis can appear for several reasons. Sometimes they can be referred as "Transverse myelitis spectrum disorders"[3]

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.[4]

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.[5]

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.[6] 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 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 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.

Differential diagnosis

The three main conditions to be 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.[7]

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.

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. [8]

Notable sufferers

See also

References

  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. ^ 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. http://archneur.ama-assn.org/cgi/pmidlookup?view=long&pmid=20697056. 
  3. ^ Pandit L. Transverse myelitis spectrum disorders, Neurol India. 2009 Mar-Apr;57(2):126-33. PMID 19439840
  4. ^ Blanc F, Froelich S, Vuillemet F, et al. (November 2007). "[Acute myelitis and Lyme disease"] (in French). Rev. Neurol. (Paris) 163 (11): 1039–47. PMID 18033042. http://www.masson.fr/masson/MDOI-RN-11-2007-163-11-0035-3787-101019-200703533. 
  5. ^ Dr Thomas Stuttaford; Cat nipped;Body And Mind; The Times; 26 August 1993
  6. ^ 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)
  7. ^ Walid MS, Ajjan M, Ulm AJ (2008). "Subacute transverse myelitis with Lyme profile dissociation". Ger Med Sci 6: Doc04. PMC 2703261. PMID 19675732. http://www.egms.de/en/gms/2008-6/000049.shtml. 
  8. ^ 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. PMID 8489410. http://archneur.ama-assn.org/cgi/pmidlookup?view=long&pmid=8489410. 
  9. ^ Breitrose, Charlie (July 3, 2008). "She is among the elite". Natick Bulletin and Tab. http://www.wickedlocal.com/natick/archive/x415952841/She-is-among-the-elite. Retrieved 2008-10-16. 
  10. ^ BBC — Radio 4 — Today at 50:50th Birthday — Stephen Morris
  11. ^ "Tiger JK’s English interview". 16 August 2009. http://www.allkpop.com/2009/08/tiger_jks_english_interview. Retrieved 19 June 2011. 
  12. ^ [Interview]http://americanmusicchannel.com/features/comversation_corner/4-24-2009/conversation-corner-hal-ketchum by American Music Channel: 04-24-09
  13. ^ Daily Mail, 14 August 1998, I know I'm mean: I refused to let my wife have a new dustbin
  14. ^ Sham, Brad (2008-07-28). "Former Center Rafferty Battling Disease". DallasCowboys.com. http://www.dallascowboys.com/news/news.cfm?id=6C51445D-C4C6-8B2B-408CC6D5AD8F144B. Retrieved 2008-11-01. 
  15. ^ California Literary Review
  16. ^ "Venter tackles his biggest challenge". International Rugby Board. 29 June 2007. http://www.irb.com/newsmedia/features/newsid=278103.html. 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." 
  17. ^ Cody Unser First Step Foundation. Retrieved 2010-07-24.

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