Differential Diagnosis and Management of Transverse Myelitis
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)
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
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.