This document discusses the approach to transverse myelitis (TM). Some key points: 1. TM is an acute inflammatory disorder of the spinal cord that presents with weakness, sensory alterations, and bowel/bladder dysfunction. Symptoms progress to maximum deficit within 4 hours to 21 days. 2. It can be idiopathic or secondary to conditions like multiple sclerosis or systemic lupus erythematosus (SLE). Diagnosis involves ruling out other causes and detecting spinal cord inflammation. 3. Initial treatment involves high-dose steroids and possibly plasma exchange. Patients with underlying autoimmune diseases may require additional immunotherapy. Prognosis depends on factors like severity and speed of progression, with most recovery

1. APPROACH TO
TRANSVERSE MYELITIS
Naresh.k
Neurology registrar(1st yr)

2. • 23 yr old ms. Varsha ,nurse from chattisgarh
k/c/o SLE presented with weakness of b/l LL
and sensory loss below the umbilicus and
bladder symptoms , onset to peak is 8 hrs on
examination stable vitals with areflexic flaccid
paraparesis with sensory level at T10.
alopecia,platal ulcers, malar rash+
?

6. Compressive myelopathy
• Disc herniations, epidural masses or blood,
vertebral body compression fractures,
spondylosis, tuberculosis of spine.
• They may present without overt evidence or
history of trauma.

7. Non inflammatory myelopathy
●Vascular myelopathies
Anterior spinal artery infarction,Spinal-dural
arteriovenous fistula,Fibrocartilaginous embolism
●Metabolic and nutritional myelopathies
Vitamin B12 ,Vitamin E , Copper deficiency,Nitrous
oxide toxicity,Neurolathyrism and neurocassavism
●Neoplasms
Intramedullary primary spinal cord tumor,Primary central
nervous system lymphoma,Intravascular lymphoma
●Radiation myelitis

8. Inflammatory myelopathy
• infection (eg, enteroviruses, West Nile virus,
herpes, central nervous system Lyme disease,
mycoplasma)
• systemic rheumatologic disease (eg, systemic
lupus erythematosus, Sjögren syndrome)
• paraneoplastic syndrome
• a multifocal neurologic disease (eg, multiple
sclerosis, neuromyelitis optica, acute
disseminated encephalomyelopathy)

11. Clinical presentations of myelopathy

14. Transverse myelitis
• Acute transverse myelitis (TM) is a rare
acquired neuro-immune spinal cord disorder
that can present with the rapid onset of
weakness, sensory alterations, and bowel or
bladder dysfunction.

15. epidemiology
• incidence between one to eight new cases per
million people per year
• bimodal peak between the ages of 10 to 19
years and 30 to 39 years has been reported
• no gender or familial predisposition to TM,
although women predominate among the
cases that are associated with multiple
sclerosis .

16. classification
• Idiopathic TM , Secondary (disease-associated)
TM is most often related to a systemic
inflammatory autoimmune condition.
• Subtypes based on clinical severity, relative
involvement of spinal cord gray matter and
longitudinal extent of the spinal cord lesion.
acute flaccid myelitis (AFM), acute partial TM,
acute complete TM , longitudinally extensive TM
(LETM).

17. • acute complete TM-risk of multiple sclerosis is 5 -
10 % .
• partial or incomplete myelitis – risk of multiple
sclerosis is high.
• Patients with acute partial myelitis + cranial MRI
abnormalities of multiple sclerosis have a
transition rate to multiple sclerosis over three to
five years of 60 - 90 %.
• In contrast, patients with acute partial myelitis +
normal brain MRI develop multiple sclerosis at a
rate of only 10 - 30 % over a similar time period .

18. • Idiopathic TM usually occurs as a postinfectious complication and
appears to result from an autoimmune process.
• Secondary TM
• Acquired central nervous system autoimmune disorders that can
cause TM include :
• TM as a part of the spectrum of multiple sclerosis. In some cases,
TM is the initial demyelinating event (a clinically isolated syndrome
[CIS]) that precedes clinically definite multiple sclerosis.
• TM manifesting as a neuromyelitis optica spectrum disorder.
• TM may be seen in patients with acute disseminated
encephalomyelitis, a monophasic demyelinating disease with
multifocal neurologic symptoms and encephalopathy.

19. NMOD diagnostic crteria

23. Other CNS conditions
• Infections including enteroviruses, West Nile
virus, herpes viruses, HIV, human T-cell leukemia
virus type 1 (HTLV-1), Zika virus , neuroborreliosis
(Lyme), Mycoplasma, and Treponema pallidum .
• In general, infectious causes of spinal cord
dysfunction are rare, but outbreaks of acute
flaccid myelitis (AFM) serve as a reminder of the
myelitis outbreaks seen during poliovirus
outbreaks.
• Neurosarcoidosis
• Paraneoplastic syndromes

25. Systemic inflammatory disorders
• Ankylosing spondylitis
• Antiphospholipid antibody syndrome
• Behçet disease
• Mixed connective tissue disease
• Rheumatoid arthritis
• Sarcoidosis
• Scleroderma
• Sjögren syndrome
• Systemic lupus erythematosus

26. DIAGNOSTIC CRITERIA
• 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, postradiation, neoplastic, and
vascular causes
• T2 hyperintense signal change on spinal MRI

31. Other Investigations
• Serum anti-aquaporin-4 (AQP4)-IgG
autoantibodies, anti-myelin oligodendrocyte
glycoprotein (MOG) autoantibodies.
• Paraneoplastic panel
• human immunodeficiency antibodies, syphilis
serologies, serum antinuclear antibodies (ANA),
Ro/SSA, and La/SSB antibodies
• Salivary gland biopsy (if high suspicion for Sjögren
syndrome but negative SSA/SSB antibody)
• tests for viral etiologies (eg, West Nile virus,
enteroviruses, etc,) as appropriate.

32. • Nasopharyngeal swab for enteroviral polymerase
chain reaction (in setting of gray matter
predominant acute flaccid myelitis)
• Serum copper and ceruloplasmin (as copper
deficiency can mimic TM)
• Serum vitamin B12 ,vitamin E levels
• Spinal angiogram (in the setting of hyperacute
myelopathies that are thought to be consistent
with vascular myelopathies)
• Prothrombotic evaluation

33. Management
• high-dose intravenous glucocorticoids --
methylprednisolone (30 mg/kg up to 1000 mg
daily) or dexamethasone (up to 200 mg daily for
adults) for three to five days.
• plasma exchanges of 1.1 to 1.5 plasma volumes,
every other day for 10 days.
• Plasma exchange may be effective for acute
central nervous system demyelinating diseases
that fail to respond to high-dose glucocorticoid
treatment.

34. • patients with systemic autoimmune disease (eg,
SLE), the addition of cyclophosphamide(800 to
1200 mg/m2 administered as a single pulse dose)
to the treatment regimen improved outcomes.
• In the absence of systemic autoimmune disease,
plasma exchange plus methylprednisone was
superior to methylprednisone alone.
• patients with recurrent disease, chronic
immunomodulatory therapy should be given-
with mycophenolate (2 to 3 gm/daily)
or rituximab (1000 mg every 6 months)

36. Prognosis
• Most patients with idiopathic TM have at least a
partial recovery and it continues with exercise
and rehabilitation therapy over years.
• Some degree of persistent disability is common,
occurring in about 40 percent .
• Fallow 1/3 rd rule (complete recovery,residual
defects, No improvement from nadir)
• Recurrence in idiopathic TM -25-33%,disease-
associated TM- 70%.

37. Poor prognostic factors
1. rapid progression to maximal neurologic
deficit (<24 hours)
2. severe motor weakness
3. spinal shock
4. back pain as the initial complaint, and
5. sensory disturbances at the cervical level

38. Better prognostic factors
1. Older age
2. increased deep tendon reflexes
3. presence of the Babinski sign

39. recurrent disease predictors
• Multifocal or longitudinally extensive lesions in the spinal
cord on MRI
• Brain lesions on MRI
• +Presence of one or more autoantibodies (ANA,dsDNA,c-
ANCA)
• +Underlying mixed connective tissue disease
• + oligoclonal bands in the cerebrospinal fluid
• + neuromyelitis optica immunoglobulin G (NMO-IgG;
anti-aquaporin-4) antibody
• + anti-myelin oligodendrocyte glycoprotein (MOG)-IgG
antibody

40. ?
• Imaging showed features of LETM(T9-T12),
CSF mild pleocytosis.
• VEPS normal,aquaporin, MOG ab negative
• elevated ANA,SS-A,dsDNA titres fulfillled SLICC
critera with DAI-46, APLA workup negative
• Paraneoplastic screening with CT neck,thorax,
abdomen negative.

41. • Considered an immune mediated
demyelination associated with SLE (secondary
transverse myelitis)
• She was initiated on immunomodulation with
steriods,rituximab,cyclophosphamide

42. Update
• The cytokine IL-6 may be a useful biomarker,
as the level of IL-6 in the CSF of acute TM
patients strongly correlates with and is highly
predictive of disability.
• Clinical trials testing the efficacy of promising
axonoprotective agents in combination with
intravenous steroids in the treatment of TM
are currently underway.

43. Summary
• Rapidly progressive myelopathy of any kind is a
medical emergency and requires immediate
evaluation.
• ATM is the focal inflammtory disease of the
spinalcord which evolves to nadir in 4hrs to 21
days.
• All patients with transverse myelitis should be
investigated for systemic autoimmune disease
and If positive consider immunomodulatory
treatment.

44. Questions
• TM , duration of clinical deficits progress to
nadir ?
• Spinal shock consists of sensory loss T/F ?
• _____ responsible for lhermittes sign in TM ?
• In TM most recovery occurs over ?

45. References

46. Thank you