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K E L S E Y T E R R E S O N , S P T
Q U E N T I N M E A S E C O M M U N I T Y H O S P I T A L
Transverse Myelitis: Differential
Diagnosis, Medical and Therapeutic
Management
Objectives
 Understand the clinical differences between
Transverse myelitis other syndromes that present
similarly
 Understand common medical management for
transverse myelitis
 Review briefly the role of physical therapy in treating
people with transverse myelitis
Description of Transverse Myelitis
 Inflammation (and demyelination) of
one level of the spinal cord (7,8)
 Present with rapidly progressing muscle
weakness, diminished sensation (pain,
temperature and pin prick) below level of injury
 May also have loss of proprioception and
vibration
 Bowel and bladder dysfunction
 Most commonly thoracic level of injury
(7,8)
 Symptoms greater in LEs
 UE involvement rarely occurs
 70-95% cases monophasic
 Generally no brain involvement
Transverse Myelitis
 Percentage of SCI (6)
 MVA 36.5%, Falls 28.5%, Acts of violence 14.3%, Sports and
recreation 9.2%, Disease/unknown 11.4%
 Epidemiology (7, 8)
 Approximately 1400 new cases of TM every year
 Affects people of all ages, but two peaks in frequency between
10-19 and 30-39
 Approximately 25% cases are children
 No gender, familial, ethnic association
Causes of Transverse Myelitis
 Autoimmune, inflammatory, and infectious etiologies
(7,8)
 Onset typically following an acute viral infection caused by: varicella
zoster, herpes simplex, cytomegalovirus, Epstein-Barr, influenza,
echovirus, HIV, hepatitis A, or rubella
 Can also follow (less commonly) bacterial skin infections, middle-ear
infections and bacterial pneumonia
 Poorly understood, while each of these infections can individually
attack the spinal cord it is also believed that in the post-acute phase
of the body mistakenly attacks the spinal cord
 Idiopathic – where the infection is not identified (7,8)
Differential Diagnosis
 Multiple Sclerosis
 Neuromyelitis Optica
 Other Diagnoses
 Diagnostic Testing
Differential Diagnosis – Multiple Sclerosis
 Multiple Sclerosis
 Autoimmune disorder that specifically targets the CNS
 Ruled out with the use of IgG test and MRI
 Lesions across space and time
 T cell mediated
 Epidemiology (9):
 Approximately 200 people diagnosed with MS each week
 Onset between 20-40 years of age
 Women are affected approximately twice as often as men,
except in individuals with the primary-progressive form of the
disease
 Increased prevalence farther from the equator
Differential Diagnosis – Neuromyelitis Optica
 Neuromyelitis Optica (4, 5)
 Rare autoimmune disorder that preferentially causes inflammation in
the optic nerve and spinal cord
 2006 Criteria: Optic neuritis + transverse myelitis, two of the following
(1) longitudinally extensive lesions (≥3 vertebral segments in length); (2)
magnetic resonance imaging (MRI) of the brain with normal findings or
with findings not consistent with MS; and (3) NMO-IgG seropositivity
 Aquaporin 4 antibody mediated
 Epidemiology (4)
 Average age of onset 41.1 years old
 NMO (67.4%) Seropositive (68.3%), Seronegative (31.7%)
 NMOSD (32.6%)
 Female 86.4%, Male 13.4%
 Race: Caucasian (47.6%), African descent (36.9%), Latin American (8%),
Asian (5.3%), Native American (2.1%)
Other Diagnoses/Blood Screens
 Sjorgren’s – systemic autoimmune disorder in which the
WBC attack moisture producing glands, people generally
present with dry mouth dry eyes, fatigue and joint pain
 Toxoplasmosis- parasitic disease, generally
asymptomatic or flue-like symptoms but in the immuno-
compromised can cause encephalitis or necrotizing
retinochoroiditis (decreased visual acuity in one eye)
 Anti-nuclear antibody (ANA) – found in people with
tendency toward autoimmune disorders
 Anti-dsDNA – test used to diagnose lupus (generally with
positive ANA)
 VDRL/RPR – screening tests for syphilis
 HSV – Herpes simplex can attack the eyes
Diagnostic Testing
 IgG test
 Blood testing, anti-aquaporin-4 antibody (NMO-IgG) highly
specific (>99%), sensitivity ranges from 49-72%
 Positive test more meaningful than a negative test
 MRI
 MS suspect to see other lesions in the CNS
 Looking with particular attention to the brain, as TM and NMO do
not attack the brain
 Looking for lesions displaced in time and space
 Involvement of the optic nerve
 CSF
 Oligoclonal bands ****
Differential Diagnosis Summary
Diagnosis Auto-immune or
Infection Based
General Progression
Neuromyelitis Optica Auto-immune 70% recurrent
Transverse Myelitis Infection based, immune
mediated
Typically monophasic
(70-90% of the time)
Multiple Sclerosis Auto-immune Recurrent and/or
progressive
Transverse Myelitis - Acute Phase
• Neurologic function declines during first 4-21 days
• 80% reach their maximum functional deficit
within 10 days of symptom onset (7,8)
• At this time 50% have lost all volitional movement in LEs
• 80-94% experience numbness, paresthesias,
banding/girdling
• Almost all have some bowel and/or bladder dysfunction
• Medical Management (7, 8, 10)
 Methylprednisolone
 Plasma exchange
 Cyclophosphamide – NMO, TM
 Rituximab - NMO
Rehabilitation – Transverse Myelitis
 Recovery usually begins 2 to 12 weeks after onset of
symptoms
 May continue for up to 2 years
 However, if there is no improvement within the first
3 to 6 months, significant recovery is unlikely
 Commonly cited statistic: 1/3 make significant
recovery, 1/3 have very little recovery and 1/3 make
some recovery, but are left with significant
impairments, however is very outdated (1981) (1)
 Rehabilitation will vary based on the person, but
some things to consider (in addition to functional
considerations):
 Spasticity
 Pain
 Bowel/bladder and sexual dysfunction
 Depression
Physical Therapy
 Calis et al. 2011 – Rehabilitation results of patients
with transverse myelitis. (2)
 Considered 13 pts, described management (including brief
information regarding frequency of use of spasticity,
intermittent catheterization (slightly more than half)
 MAS, Barthel and Functional Ambulation Category
(MDC/MCID not established)
 FIM – MCID = 17 pts, average change 14.9pts
 Significant difference made across all measures
 Conclusion that therapy is helpful for improving outcomes for
people with TM
Questions?
References
1. Berman M, Feldman S, Alter M, Zilber N, Kahana E. Acute transverse myelitis: incidence and etiologic
considerations. Neurology. 1981 Aug;31(8):966-71
2. Calis M, Kirnap M, Calis H, Mistik S, Demir H. Rehabilitation results of patients with transverse myelitis.
Bratisl Lek Listy. 2011; 112(3):154-156.
3. Flanagan EP, Weinshenker BG, Krecke KN, Lennon VA, Luccinetti CF, McKeon A, Wingerchuk DM,
Shuster EA, Jiao Y, Horta ES, Pittock SJ. Short myelitis in aquaporin-4-IgG-positive neuromyelitis optica
spectrum disorders. JAMA Neurology. Jan 2015. 72(1):81-7.
4. Mealy MA, Wingerchuk DM, Greenberg BM, Levy M. Epidemiology of Neuromyelitis Optica in the United
States: A Multicenter Analysis Arch Neurol. 2012;69(9):1176-1180. doi:10.1001/archneurol.2012.314.
5. Neuromyelitis Information Page. National Institute of Neurological Disorders and Stroke. January 2015.
http://www.ninds.nih.gov/disorders/neuromyelitis_optica/neuromyelitis_optica.htm
6. Spinal Cord Injury Facts and Figures. National Spinal Cord Injury Statistical Center. February 2013.
https://www.nscisc.uab.edu/PublicDocuments/fact_figures_docs/Facts%202013.pdf
7. Transverse Myelitis. Christopher and Dana Reeve Foundation.
http://www.christopherreeve.org/site/c.mtKZKgMWKwG/b.4453415/k.FE54/Transverse_Myelitis.htm#
8. Transverse Myelitis Fact Sheet. National Institute of Neurological Disorders and Stoke. February 2015.
http://www.ninds.nih.gov/disorders/transversemyelitis/detail_transversemyelitis.htm
9. Tullman MJ. Overview of the Epidemiology, Diagnosis, and Disease Progression Associated With Multiple
Sclerosis. Am J Manag Care. 2013;19:S15-S20
10. Wingerchuk DM. Diagnosis and Treatment of Neuromyelitis Optica. Neurologist. January 2007. 13(1): 2-
11.

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Transversemyelitis2

  • 1. K E L S E Y T E R R E S O N , S P T Q U E N T I N M E A S E C O M M U N I T Y H O S P I T A L Transverse Myelitis: Differential Diagnosis, Medical and Therapeutic Management
  • 2. Objectives  Understand the clinical differences between Transverse myelitis other syndromes that present similarly  Understand common medical management for transverse myelitis  Review briefly the role of physical therapy in treating people with transverse myelitis
  • 3. Description of Transverse Myelitis  Inflammation (and demyelination) of one level of the spinal cord (7,8)  Present with rapidly progressing muscle weakness, diminished sensation (pain, temperature and pin prick) below level of injury  May also have loss of proprioception and vibration  Bowel and bladder dysfunction  Most commonly thoracic level of injury (7,8)  Symptoms greater in LEs  UE involvement rarely occurs  70-95% cases monophasic  Generally no brain involvement
  • 4. Transverse Myelitis  Percentage of SCI (6)  MVA 36.5%, Falls 28.5%, Acts of violence 14.3%, Sports and recreation 9.2%, Disease/unknown 11.4%  Epidemiology (7, 8)  Approximately 1400 new cases of TM every year  Affects people of all ages, but two peaks in frequency between 10-19 and 30-39  Approximately 25% cases are children  No gender, familial, ethnic association
  • 5. Causes of Transverse Myelitis  Autoimmune, inflammatory, and infectious etiologies (7,8)  Onset typically following an acute viral infection caused by: varicella zoster, herpes simplex, cytomegalovirus, Epstein-Barr, influenza, echovirus, HIV, hepatitis A, or rubella  Can also follow (less commonly) bacterial skin infections, middle-ear infections and bacterial pneumonia  Poorly understood, while each of these infections can individually attack the spinal cord it is also believed that in the post-acute phase of the body mistakenly attacks the spinal cord  Idiopathic – where the infection is not identified (7,8)
  • 6. Differential Diagnosis  Multiple Sclerosis  Neuromyelitis Optica  Other Diagnoses  Diagnostic Testing
  • 7. Differential Diagnosis – Multiple Sclerosis  Multiple Sclerosis  Autoimmune disorder that specifically targets the CNS  Ruled out with the use of IgG test and MRI  Lesions across space and time  T cell mediated  Epidemiology (9):  Approximately 200 people diagnosed with MS each week  Onset between 20-40 years of age  Women are affected approximately twice as often as men, except in individuals with the primary-progressive form of the disease  Increased prevalence farther from the equator
  • 8. Differential Diagnosis – Neuromyelitis Optica  Neuromyelitis Optica (4, 5)  Rare autoimmune disorder that preferentially causes inflammation in the optic nerve and spinal cord  2006 Criteria: Optic neuritis + transverse myelitis, two of the following (1) longitudinally extensive lesions (≥3 vertebral segments in length); (2) magnetic resonance imaging (MRI) of the brain with normal findings or with findings not consistent with MS; and (3) NMO-IgG seropositivity  Aquaporin 4 antibody mediated  Epidemiology (4)  Average age of onset 41.1 years old  NMO (67.4%) Seropositive (68.3%), Seronegative (31.7%)  NMOSD (32.6%)  Female 86.4%, Male 13.4%  Race: Caucasian (47.6%), African descent (36.9%), Latin American (8%), Asian (5.3%), Native American (2.1%)
  • 9. Other Diagnoses/Blood Screens  Sjorgren’s – systemic autoimmune disorder in which the WBC attack moisture producing glands, people generally present with dry mouth dry eyes, fatigue and joint pain  Toxoplasmosis- parasitic disease, generally asymptomatic or flue-like symptoms but in the immuno- compromised can cause encephalitis or necrotizing retinochoroiditis (decreased visual acuity in one eye)  Anti-nuclear antibody (ANA) – found in people with tendency toward autoimmune disorders  Anti-dsDNA – test used to diagnose lupus (generally with positive ANA)  VDRL/RPR – screening tests for syphilis  HSV – Herpes simplex can attack the eyes
  • 10. Diagnostic Testing  IgG test  Blood testing, anti-aquaporin-4 antibody (NMO-IgG) highly specific (>99%), sensitivity ranges from 49-72%  Positive test more meaningful than a negative test  MRI  MS suspect to see other lesions in the CNS  Looking with particular attention to the brain, as TM and NMO do not attack the brain  Looking for lesions displaced in time and space  Involvement of the optic nerve  CSF  Oligoclonal bands ****
  • 11. Differential Diagnosis Summary Diagnosis Auto-immune or Infection Based General Progression Neuromyelitis Optica Auto-immune 70% recurrent Transverse Myelitis Infection based, immune mediated Typically monophasic (70-90% of the time) Multiple Sclerosis Auto-immune Recurrent and/or progressive
  • 12. Transverse Myelitis - Acute Phase • Neurologic function declines during first 4-21 days • 80% reach their maximum functional deficit within 10 days of symptom onset (7,8) • At this time 50% have lost all volitional movement in LEs • 80-94% experience numbness, paresthesias, banding/girdling • Almost all have some bowel and/or bladder dysfunction • Medical Management (7, 8, 10)  Methylprednisolone  Plasma exchange  Cyclophosphamide – NMO, TM  Rituximab - NMO
  • 13. Rehabilitation – Transverse Myelitis  Recovery usually begins 2 to 12 weeks after onset of symptoms  May continue for up to 2 years  However, if there is no improvement within the first 3 to 6 months, significant recovery is unlikely  Commonly cited statistic: 1/3 make significant recovery, 1/3 have very little recovery and 1/3 make some recovery, but are left with significant impairments, however is very outdated (1981) (1)
  • 14.  Rehabilitation will vary based on the person, but some things to consider (in addition to functional considerations):  Spasticity  Pain  Bowel/bladder and sexual dysfunction  Depression
  • 15. Physical Therapy  Calis et al. 2011 – Rehabilitation results of patients with transverse myelitis. (2)  Considered 13 pts, described management (including brief information regarding frequency of use of spasticity, intermittent catheterization (slightly more than half)  MAS, Barthel and Functional Ambulation Category (MDC/MCID not established)  FIM – MCID = 17 pts, average change 14.9pts  Significant difference made across all measures  Conclusion that therapy is helpful for improving outcomes for people with TM
  • 17. References 1. Berman M, Feldman S, Alter M, Zilber N, Kahana E. Acute transverse myelitis: incidence and etiologic considerations. Neurology. 1981 Aug;31(8):966-71 2. Calis M, Kirnap M, Calis H, Mistik S, Demir H. Rehabilitation results of patients with transverse myelitis. Bratisl Lek Listy. 2011; 112(3):154-156. 3. Flanagan EP, Weinshenker BG, Krecke KN, Lennon VA, Luccinetti CF, McKeon A, Wingerchuk DM, Shuster EA, Jiao Y, Horta ES, Pittock SJ. Short myelitis in aquaporin-4-IgG-positive neuromyelitis optica spectrum disorders. JAMA Neurology. Jan 2015. 72(1):81-7. 4. Mealy MA, Wingerchuk DM, Greenberg BM, Levy M. Epidemiology of Neuromyelitis Optica in the United States: A Multicenter Analysis Arch Neurol. 2012;69(9):1176-1180. doi:10.1001/archneurol.2012.314. 5. Neuromyelitis Information Page. National Institute of Neurological Disorders and Stroke. January 2015. http://www.ninds.nih.gov/disorders/neuromyelitis_optica/neuromyelitis_optica.htm 6. Spinal Cord Injury Facts and Figures. National Spinal Cord Injury Statistical Center. February 2013. https://www.nscisc.uab.edu/PublicDocuments/fact_figures_docs/Facts%202013.pdf 7. Transverse Myelitis. Christopher and Dana Reeve Foundation. http://www.christopherreeve.org/site/c.mtKZKgMWKwG/b.4453415/k.FE54/Transverse_Myelitis.htm# 8. Transverse Myelitis Fact Sheet. National Institute of Neurological Disorders and Stoke. February 2015. http://www.ninds.nih.gov/disorders/transversemyelitis/detail_transversemyelitis.htm 9. Tullman MJ. Overview of the Epidemiology, Diagnosis, and Disease Progression Associated With Multiple Sclerosis. Am J Manag Care. 2013;19:S15-S20 10. Wingerchuk DM. Diagnosis and Treatment of Neuromyelitis Optica. Neurologist. January 2007. 13(1): 2- 11.

Editor's Notes

  1. The STM episode was defined as the first manifestation of NMOSD in 10 patients (40%) preceded by optic neuritis in 13 patients (52%) and preceded by a nausea and vomiting episode in 2 patients (8%). In comparison with the excluded patients with NMOSD who had an initial longitudinally extensive transverse myelitis, delay to diagnosis/treatment was greater when initial lesions were short (P = .02). In AQP4-IgG-positive STM cases, subsequent myelitis episodes were longitudinally extensive in 92%. Attributes more common in patients with AQP4-IgG-positive STM than in 27 population-based patients with AQP4-IgG-negative STM included the following: nonwhite race/ethnicity; tonic spasms; coexisting autoimmunity; magnetic resonance imaging (central cord lesions, T1 hypointensity, and a brain inconsistent with multiple sclerosis); and cerebrospinal fluid (oligoclonal bands lacking). JAMA Neurol. 2015 Jan;72(1):81-7. doi: 10.1001/jamaneurol.2014.2137. Short myelitis lesions in aquaporin-4-IgG-positive neuromyelitis optica spectrum disorders. Flanagan EP1, Weinshenker BG1, Krecke KN2, Lennon VA3, Lucchinetti CF1, McKeon A4, Wingerchuk DM5, Shuster EA6, Jiao Y7, Horta ES7, Pittock SJ4.