Transverse MyelitisDr/Reyad Alfaky

Transverse Myelitis•Definition• Etiology• Presentation•Diagnosis• Treatment•Prognosis

Definition• Transverse myelitis (TM) is a segmental spinal cord injury caused by

acute inflammation

DEFINITION• Transverse myelitis (TM)

• as evidence of spinal cord inflammation by an

MRI-documented enhancing lesion, or CSF pleocytosis (>10 cells), or increased

immunoglobulin G index.

• may be

1. Postinfectious

2. postvaccination, or

3. associated with multiple sclerosis.

DEFINITION acute transverse myelopathy

• is a broader term refers to any process that acutely impairs spinal cord function.

Types ; Transverse myelitis (TM)

1. Complete Transverse Myelitis

2. Partial or incomplete Transverse Myelitis

Progression ; Transverse myelitis (TM)

The progression is rapid

• time to maximal disability is more than 4 hr and fewer than 21 days.

A. Acute (over minutes or hours),

B. subacute (over days or weeks)

Small children

• Small children, 3 yr of age and younger

onset

• develop spinal cord dysfunction over hours to a few days

• clinical loss of function is often severe and may seem complete

recovery

• slow recovery (weeks to months) is common in these cases

• it is likely to be incomplete.

• independent ambulation in these small children is approximately 40%.

older children

onset

• is also rapid with a nadir in neurologic function occurring between 2 days and 2 wk

recovery

• is more rapid

• more likely to be complete.

pathophysiology

•TM is often preceded within the previous 1-3 wk by

1. mild nonspecific illness

2. minimal trauma

3. immunization.

•Postinfectious etiology largely predominates in children.

pathophysiology

•Three hypotheses have been proposed:

1. Cell mediated autoimmune response

2. autoimmune vasculitis

3. direct viral invasion of spinal cord.

Transverse Myelitis (TM)• Immune-mediated process results in neural

injury to the spinal cord

• Varying degrees of weakness, sensory

alterations and autonomic dysfunction

• Up to half of idiopathic cases will have a

preceding respiratory or gastrointestinal

illness

Multi-focal

CNS dis-ease (eg.

MS)

Systemic disease

(eg. SLE)

Idiopathic Entity

EtiologyA. Post or parainfectious

• Respiratory or gastrointestinal infections within 3 to 8 weeks

B. Direct invasion of spinal cord

C. Systemic autoimmune diseases

• Systemic Lupus Erythematosus (SLE)

•Multiple Sclerosis

EPIDEMIOLOGY

Incidence:

• 1 to 4/million per year

• affecting all ages with

• bimodal peaks between the ages of 10 and 19 years and 30 and 39 years.

• Boys and girls are affected equally

Clinical presentationCombination of ;

1. Sensory

2. Motor

3. bladder symptoms

clinical course

• The course of ATM in children proceeds through three stages:

1. initial motor loss precedes sphincter dysfunction in most patients, there is often a

sensory loss below certain levels, usually over 2 to 3 days

2. plateau phase: the mean duration of plateau is 1 week

3. recovery phase.

Clinical presentationFeatures of spinal cord lesion

1. Band like sensation (pressure, pain, numbness) over the trunk

2. Bladder symptoms (incontinence, difficulty urinating, retention)

3. Horizontal level of sensory loss

4. Unilateral posterior column loss

5. pyramidal weakness

6. contralateral spinothalamic loss

Clinical presentation

Typical presentation

• Prior history of fever (nonspecific viral illness)

• ATM, the onset of spinal cord dysfunction usually progress in 4 hours to 21 days,

the patient's signs usually plateau and evolve toward spasticity/hyperreflexia.

• Back pain, paresthesias, radicular pain in the legs

• Bilateral, asymmetric, unilateral, acute-sub acute progressive leg weakness with

any of the features of spinal cord lesion

Clinical presentation

• Urinary retention is an early finding;

• incontinence occurs later in the course.

HISTORY• Other findings may include

1. priapism

2. vision loss (neuromyelitis optica),

3. as well as spinal shock

4. subsequent autonomic dysreflexia

Physical Examination• Extreme irritability

• extent of weakness is assessed by how vigorously the child resists examination

• Fever, hypertension, tachycardia, meningeal signs may be present, in which cases CNS

infection need to be ruled out;

• point tenderness over the spine may point to trauma or infection

• Neurologic examination directed to

• visual acuity and color vision

• funduscopic examination for optic nerve head pallor (optic neuritis)

causes the pain and irritability commonly seen in children with TMPain in TM may be as a result of

1. neuropathic pain from nerve root inflammation

2. nociceptive pain from dural inflammation

3. muscle spasm from motor dysfunction

4. bladder distension from dysautonomia

5. psychological distress from loss of motor control

6. dysesthesia from demyelination of spinothalamic tract.

Physical Examination• Increased tone, spastic weakness is usually symmetric, legs more than arms

• Reflexes are usually brisk, with positive Babinski sign.

• Sensory ataxia, a sensory level (a partial level is commonly seen) that may spare

joint position and vibration, may be present.

Physical Examination• Sphincter dysfunction can lead to emergent complication of urinary

retention or incontinence;

• check for loss of anal wink

• bladder dilatation, and large volume of post void residual (>100 mL).

Laboratory Aids1. MRI with and without contrast enhancement is essential to rule out a mass lesion requiring

neurosurgical intervention

2. MRI of the brain is also indicated

3. lumbar puncture is indicated.

4. neuromyelitis optica (NMO; Devic syndrome)

• the serum of all patients should be analyzed for the NMO antibody.

5. TM, older children with the condition should have serum studies sent for autoimmune

disorders, especially systemic lupus erythematosus.

Laboratory Aids• MRI and CSF analysis are the two most important tests and are mandatory in

suspect ATM.

• Enhancing spinal cord lesion or pleocytosis or increased IgG index is required

for the diagnosis.

• If both tests are negative, repeat tests in 2 to 7 days is recommended.

Laboratory AidsA. The first priority in acute myelopathy

• is to rule out structural cause compressive myelopathy.

• spinal MRI

B. The second priority is to

• define the presence/absence of spinal cord inflammation and to rule out other CNS infection.

• LP

A. Third priority is to

• define extent of demyelination.

• Gadolinium-enhanced MRI of the brain

• evoked potential studies (e.g., visual evoked potential, somatosensory evoked potential)

Laboratory Aids Lumbar puncture is usually done after imaging, often shows

A. normal or slightly increased protein

B. mild pleocytosis with lymphocyte predominance.

C. Elevation of IgG index and presence of oligo clonal bands are indicative of MS or

other systemic inflammatory disease.

D. CSF gram stain, bacterial, viral, and fungal culture, VDRL, lyme antibodies, and PCR of

specific viruses should all be negative in ATM.

Laboratory Aids Other

A. ESR and ANA

B. RPR, Lyme titer

C. underlying metabolic disorder including VLCFA.

D. Viruses associated with ATM include the herpes viruses (EBV, VZV, HSV), CMV mumps, rubella,

influenza, hepatitis A, B, C, HIV.

E. Positive IgM or greater than fourfold increase in IgG levels on two successive tests to a specific

infectious agent suggests diagnosis of parainfectious ATM.

Differentiate FromGB Syndrome

1. Progressive lower limb weakness (proximal>distal)

2. Ascends to upper limbs & bifacial weakness

3. Arreflexia

4. Normal sensory examination

5. No bladder symptoms

6. No band like sensation

7. No level for sensory loss

Characteristics Transverse Myelitis Guillain-Barre SyndromeMotor findings Paraparesis or quadriparesis Ascending weakness LE > UE in the early

stagesSensory findings Usually can diagnose a spinal cord level Ascending sensory loss LE > UE in the

early stagesAutonomic findings Early loss of bowel and bladder control Autonomic dysfunction of the

(CV) systemCranial nerve None EOM palsies or facial weaknessElectrophysiologic findings

EMG/NCV findings may be normal or mayimplicate the spinal cord: prolonged centralconduction on somatosensory evoked potential(SEP) latencies or missing SEP in conjunctionwith normal sensory nerve action potentials

EMG/NCV findings confined to the PNS: motor

and/or sensory nerve conduction velocityreduced, distal latencies prolonged; conductionblock; reduced H reflex usually present

MRI findings Usually a focal area of increased T2 signal with or without gadohnium enhancement

Normal

CSF Usually, CSF pleocytosis and/or increased IgG index

Usually, elevated protein in the absence of CSF pleocytosis

Acute Transverse Myelopathy• The three main categories in the differential diagnosis of ATM are demyelination,

including

1. multiple sclerosis (MS)

2. neuromyelitis optica (NMO), and

3. idiopathic transverse myelitis;

DIAGNOSTIC CRITERIA FOR TRANSVERSE MYELITIS

1. Bilateral (not necessarily symmetric) sensorimotor and autonomic spinal cord dysfunction

2. Clearly defined sensory level

3. Progression to nadir of clinical deficits between 4 hours and 21 days after symptom onset

4. Demonstration of spinal cord inflammation:

1. cerebrospinal fluid pleocytosis or

2. elevated IgG index,or

3. MRI revealing a gadolinium-enhancing cord lesion

5. Exclusion of compressive, postradiation, neoplastic, and vascular causes

CRITERIA FOR DIAGNOSIS OF ACUTE TRANSVERSE MYELITIS4. Inflammation within the spinal cord demonstrated by

I. CSF pleocytosis or

II. elevated IgG index or

III. gadolinium enhancement

• (If none of the inflammatory criteria is met at symptom onset, repeat MRI

and LP evaluation between 2–7 days following symptom onset).

• IgG index = : (CSF IgG + serum IgG) (CSF albumin + serum albumin)

MANAGEMENT OF TM

1. Care of the paraplegic patient

• Multidisciplinary rehab approach is key

2. Intravenous Steroids

3. Plasma Exchange (PLEX)

Chronic Management of TM

Intravenous Steroids Corticosteroids have multiple mechanisms of action including

1. antiinflammatory activity

2. immunosuppressive properties

3. antiproliferative actions.methylprednisolone therapy

• high-dose methylprednisolone (30mg /kg (max 1 g) IV daily for 3–7 days) is

typically first-line treatment

• early in the course is effective in shortening the duration of the disease and in

improving the outcome.

Intravenous Steroidsmethylprednisolone therapy

• If there is a poor response to high-dose steroids, other

therapeutic approaches include ;

1. intravenous immunoglobulin

2. plasma exchanges

3. Rituximab

4. cyclophosphamide.

Plasma Exchange (PLEX)• PLEX is often initiated if a patient has moderate to severe TM

1. inability to walk

2. markedly impaired autonomic function

3. sensory loss in the lower extremities and

4. exhibits little clinical improvement after instituting 5 to

7days of intravenous steroids.

Natural History and PrognosisThe progression of symptoms in ATM

• often slows within 2 to 3 weeks of onset

• with a corresponding improvement in CSF and MRI abnormalities

recovery :-• Evidence of at least some recovery is expected to begin within 6 months

• most patients show some improvement in neurologic function within 8 weeks

• although recovery can take a more prolonged course of up to 2 years

Follow-UpResidual neurologic deficits include

1. fixed weakness

2. sensory, or autonomic deficits.

3. Sphincter dysfunction improves more slowly than the other deficits.

Follow-UpATM may be the presenting feature of MS, especially in patients with

1. partial ATM

2. abnormal initial brain MRI, in such cases, follow up MRIs should be considered.

Follow-Up

History of other neurologic symptoms such as

1. internuclear ophthalmoplegia

2. optic neuritis

3. focal weakness and numbness that lasted at least 24 hours to days, have now

resolved completely

4. other lesions on brain/spine MRI at the time of presentation, and subsequent new

MRI lesions

Prognosis • pediatric outcomes are better than in adults, with children often regaining

complete function.

• Spontaneous complete recovery (within weeks or months) in -40-50% of cases.

• Residual deficits (weakness of LL, bladder dysfunction) occur in the remaining

cases.

• The majority of patients with ATM have monophasic disease without recurrence.

Outcomes• 1/3 pts have a complete recovery

• 1/3 pts have some residual deficit

• 1/3 pts have no improvement from nadir

Prognosis risk factors for unfavorable outcomes at presentation, including

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

Prognosisbetter course

1. Older age

2. increased deep tendon reflexes

3. presence of the Babinski sig