ACUTE TRANSVERSE MYELITIS CLINICAL PRESENTATION, DIAGNOSIS, DDX AND MANAGEMENT

1. TRANSVERSE MYELITIS
Dr. Mohamed Sheikh Hassan
Neurology

2. Transverse Myelitis
Definition:
• A pathogenically heterogeneous focal inflammatory
disorder of the spinal cord characterized by acute or sub
acute development of motor weakness, sensory
impairment, bowel or bladder dysfunction and autonomic
dysfunction.
• The term myelitis refers to inflammation of the spinal
cord; transverse simply describes the position of the
inflammation, that is, across the width of the spinal cord.

3. Epidemiology:
 Incidence between 1-8 new cases per million per year.
 Affects people of all ages, with a range of six months to
88 years.
 Bimodal peaks between the ages of 10 to 19 years and
30 to 39 years.
 There is no gender or familial association with TM.
 It is estimated that about 1,400 new cases of
transverse myelitis are diagnosed each year in the
United States.

4. Transverse Myelitis
Etiology:
• Infectious
• Non Infectious inflammatory type
• Idiopathic
Infectious:
I. Viral:
A. Enter viruses (groups A and B Coxsackievirus, poliomyelitis, others)
B. Herpes zoster
C. Myelitis of AIDS
D. Epstein-Barr virus (EBV), cytomegalovirus (CMV), herpes simplex
E. Rabies
II. Bacterial/fungal/parasitic:
A. Mycoplasma pneumonia
B. Burrelia burdorferi ( Lyme disease)
C. Pyogenic myelitis 1.Acute epidural abscess and granuloma 2. Abscess of spinal cord
D. Tuberculous myelitis
E. Syphilitic myelitis
F. Parasitic and fungal infections producing epidural granuloma,

5. Transverse myelitis
Noninfectious inflammatory type:
 Postvaccinal myelitis
 Acute and chronic relapsing or progressive multiple
sclerosis (MS)
 Subacute necrotizing myelitis and Devic’s disease(NMO)
 Myelopathy with lupus or other forms of connective tissue
diseases
 Para neoplastic myelopathy
Idiopathic :
 Clinical events that are consistent with TM but that are not
associated with CSF abnormalities or abnormalities
detected on MRI and that have no identifiable underlying
cause are characterized as possible idiopathic TM.

6. Transverse Myelitis
IMMUNOPATHOGENESIS:
-pathologic heterogeneity with involvement of both
gray and white matter
-TM is not a pure demyelinating disorder but rather a
mixed inflammatory disorder that affects neurons,
axons, and oligodendrocytes and myelin.
-autopsy reports have described lymphocytic
infiltration with demyelination and axonal loss
-30 to 60 percent of the idiopathic TM cases, there is
an antecedent respiratory, gastrointestinal, or
systemic illness.

7. Transverse Myelitis
IMMUNOPATHOGENESIS:
-In parainfectious TM, the injury may be associated with direct
microbial infection of the central nervous system, or with the
systemic response to infection by a variety of agents such as
varicella zoster virus, herpes virus, and Listeria monocytogenes.
-Lupus-associated TM could be associated with central nervous
system vasculitis or thrombotic infarction of the spinal cord.
-TM can occur as part of the spectrum of multiple sclerosis

8. Transverse myelitis
Symptoms of TM typically develop rapidly over several
hours
Approximately 37 percent of patients worsen maximally
within 24 hours
Some cases worsen more slowly, over several weeks.
Clinical Presentation: 4 Symptom groups
Motor
Sensory
Autonomic
Pain

9. Transverse Myelitis
Motor:
Depends on level, lesion extent:
Examples:
 C3-C5 Quadriplegia, Respiratory Paralysis( diaphragm)
 T1-T2:Spastic paraplegia
 L1-S5:UMN/LMN in lower extremities, Bowel/bladder dysfunction
(urinary retention)
 Spinal shock: Sometimes in hyper acute stage( legs flaccid/areflexia);
then spasticity develops
Sensory:
 Paraesthesias, numbness, altered temp sensations., etc.
 L'hermitte sign (Paresthesias in the limbs, elicited by neck flexion)

10. Autonomic:
 Urinary urgency, incontinence, nocturia, urine retention
 Bowel: Urgency, incontinence, retention
 Sexual Dysfunction
Pain:
 “Burning” radicular pain
 “Tight squeezing”
 “banding” sensation
Note:
 Severe cases of ascending myelitis involving the cervical spinal cord may
cause neurogenic respiratory failure.
 Longitudinally extensive TM can produce hiccups due to involvement of the
medulla, and persistent nausea and vomiting due to involvement of the
area postrema

11. Diagnostic Criteria for Transverse Myelitis*
• Bilateral (not necessarily symmetric) sensorimotor and autonomic
spinal cord dysfunction.
• Clearly defined sensory level.
• Progression to nadir of clinical deficits between 4 hours and 21 days
after symptom onset
• Demonstration of spinal cord inflammation: cerebrospinal fluid
pleocytosis or elevated IgG index,† or MRI revealing a gadolinium-
enhancing cord lesion.
• Exclusion of compressive, post radiation, neoplastic, and vascular
causes.
* Clinical events that are consistent with transverse myelitis but that are
not associated with cerebrospinal fluid abnormalities or abnormalities
detected on MRI and that have no identifiable underlying cause are
categorized as possible idiopathic transverse myelitis.

12.  Types:
Complete:
 Usually longer (>3 vertebral segments) and Central--
>Symmetric decrease in motor/sensory functions associated
with decreased sphincter functions.
Incomplete:
 Shorter( <3 vertebral segments), peripheral lesions involving
limited tracts, usually incomplete loss of function.

13.  Classification based on spinal cord lesion characteristics:
Acute partial TM:
• Para infectious
• Asymmetric lesions typically of 1 or 2 vertebral segments in length
• Usually mild to moderate in severity
• High risk for MS when MRI brain reveals WM lesions compatible with
demyelination.
Longitudinally extensive TM:
• Asymmetric or symmetric spinal cord lesions that span ≥3 contiguous
vertebral segments.
• Usually moderate to very severe
• Most commonly associated with high risk for NMO spectrum disorders in
the setting of seropositivity for NMO- IgG

16. Diagnosis
 Diagnosis of TM is based on clinical and Radiological findings
 CSF studies:
 Usually depends on etiology
 Most commonly, Cerebrospinal fluid (CSF) is abnormal in half of patients,
with elevated protein level (usually 100 to 120 mg/100 mL) and moderate
lymphocytosis (usually <100/mm3). Glucose levels are normal. Oligoclonal
bands are usually not present in isolated TM, and when present suggest a
higher risk of subsequent MS.
 Lab work up depends on suspected etiology.
 Imaging:
 Lesions may occur anywhere within the cord, however the thoracic cord is
the most frequently involved site.
CT Spine
• variable enlargement of the spinal cord
• variable contrast enhancement patterns (including no enhancement)

17. MRI Spine
• Up to 40% of cases have no findings on MRI. In the remainder, the
appearance is variable and non-specific.
• large variation in lesion size, however they most commonly extend for 3-4
spinal segments.
• lesions typically occupy greater than two thirds of the cross-sectional area of
the cord.
• variable enlargement of the spinal cord.
• an intrinsic spinal cord lesion that usually enhances with gadolinium
administration. It is critically important to exclude compressive lesions of the
spinal cord, such as spinal epidural abscess, that require specific treatment.
Typical signal characteristics on MRI brain include:
 T1 : isointense or hypointense
 T2 : poorly delineated hyperintense signal
 T1 C+ (Gd) : variable enhancement patterns (none, diffuse, patchy,
peripheral)

22. Interpretation of clinical spinal cord syndromes
• Complete cord syndrome:
 Loss of all motor and sensory modalities below the level
of the lesion
 Associated with acute, severe, "necrotizing" myelitis.
• Brown-Sequard syndrome (partial or complete):
 Dysfunction of the corticospinal tracts and dorsal
columns( symptoms ipsilateral to the lesion) and
spinothalamic tract dysfunction( symptoms contralateral
to the lesion)
• Conus-Medullaris syndrome:
 Can be associated with Viral or post-viral myelitis
syndromes.

27. TM-PROGNOSIS
 Most patients with idiopathic TM have at least a partial recovery, which
usually begins within one to three months.
 Some degree of persistent disability is common, occurring in about 40
percent.
 Significant recovery is unlikely if there is no improvement by three months.
 A very rapid onset with complete paraplegia and spinal shock have been
associated with poorer outcomes.
 TM is generally a monophasic illness. However, a small percentage of patients
may suffer a recurrence.
Cont………….

28. TM-PROGNOSIS(cont…
 While patients are often treated with parenteral
corticosteroid therapy, there is limited evidence that this
approach alters outcomes.
 MS is more likely to develop in those with partial and
asymmetric cord involvement versus a complete cord
syndrome. A finding of demyelinating lesions on brain MRI
also identifies those at higher risk for MS.

29. Thank You