Transverse Myelitis

Transverse MyelitisDr/Reyad Alfaky

Transverse MyelitisDefinitionEtiologyPresentationDiagnosisTreatmentPrognosis

DefinitionTransverse myelitis (TM) is a segmental spinal cord injury caused by acute inflammation

DEFINITION

Transverse myelitis (TM) as evidence of spinal cord inammation by anMRI-documented enhancing lesion, or CSF pleocytosis (>10 cells), or increased immunoglobulin G index. may be Postinfectiouspostvaccination, or 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) Complete Transverse MyelitisPartial 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.Acute (over minutes or hours), subacute (over days or weeks)

Small childrenSmall children, 3 yr of age and youngeronset develop spinal cord dysfunction over hours to a few days clinical loss of function is often severe and may seem completerecovery slow recovery (weeks to months) is common in these casesit 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 wkrecovery is more rapid more likely to be complete.

pathophysiologyTM is often preceded within the previous 1-3 wk by mild nonspecific illnessminimal traumaimmunization.Postinfectious etiology largely predominates in children.

pathophysiologyThree hypotheses have been proposed:Cell mediated autoimmune responseautoimmune vasculitisdirect viral invasion of spinal cord.

Transverse Myelitis (TM)Immune-mediated process results in neural injury to the spinal cordVarying degrees of weakness, sensory alterations and autonomic dysfunctionUp to half of idiopathic cases will have a preceding respiratory or gastrointestinal illness

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EtiologyPost or parainfectious Respiratory or gastrointestinal infections within 3 to 8 weeksDirect invasion of spinal cordSystemic autoimmune diseasesSystemic Lupus Erythematosus (SLE)Multiple Sclerosis

EPIDEMIOLOGYIncidence: 1 to 4/million per yearaffecting 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 ;SensoryMotorbladder symptoms

clinical courseThe course of ATM in children proceeds through three stages: initial motor loss precedes sphincter dysfunction in most patients, there is often a sensory loss below certain levels, usually over 2 to 3 daysplateau phase: the mean duration of plateau is 1 weekrecovery phase.

Clinical presentationFeatures of spinal cord lesionBand like sensation (pressure, pain, numbness) over the trunk Bladder symptoms (incontinence, difficulty urinating, retention)Horizontal level of sensory loss Unilateral posterior column losspyramidal weakness contralateral spinothalamic loss

Clinical presentationTypical presentationPrior 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 legsBilateral, asymmetric, unilateral, acute-sub acute progressive leg weakness with any of the features of spinal cord lesion

Clinical presentationUrinary retention is an early finding; incontinence occurs later in the course.

HISTORYOther findings may include priapism vision loss (neuromyelitis optica),as well as spinal shock subsequent autonomic dysreexia

Physical ExaminationExtreme irritabilityextent of weakness is assessed by how vigorously the child resists examinationFever, 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 infectionNeurologic examination directed to visual acuity and color visionfunduscopic 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 neuropathic pain from nerve root inflammationnociceptive pain from dural inflammationmuscle spasm from motor dysfunctionbladder distension from dysautonomiapsychological distress from loss of motor controldysesthesia from demyelination of spinothalamic tract.

Physical ExaminationIncreased tone, spastic weakness is usually symmetric, legs more than armsReflexes 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 ExaminationSphincter dysfunction can lead to emergent complication of urinary retention or incontinence; check for loss of anal winkbladder dilatation, and large volume of post void residual (>100 mL).

Laboratory AidsMRI with and without contrast enhancement is essential to rule out a mass lesion requiring neurosurgical interventionMRI of the brain is also indicatedlumbar puncture is indicated.neuromyelitis optica (NMO; Devic syndrome) the serum of all patients should be analyzed for the NMO antibody. TM, older children with the condition should have serum studies sent for autoimmune disorders, especially systemic lupus erythematosus.

Laboratory AidsMRI 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 AidsThe first priority in acute myelopathy is to rule out structural cause compressive myelopathy.spinal MRIThe second priority is to define the presence/absence of spinal cord inflammation and to rule out other CNS infection.LPThird priority is to define extent of demyelination. Gadolinium-enhanced MRI of the brainevoked potential studies (e.g., visual evoked potential, somatosensory evoked potential)

Laboratory AidsLumbar puncture is usually done after imaging, often shows normal or slightly increased proteinmild pleocytosis with lymphocyte predominance. Elevation of IgG index and presence of oligo clonal bands are indicative of MS or other systemic inflammatory disease. CSF gram stain, bacterial, viral, and fungal culture, VDRL, lyme antibodies, and PCR of specific viruses should all be negative in ATM.

Laboratory AidsOther ESR and ANARPR, Lyme titerunderlying metabolic disorder including VLCFA. Viruses associated with ATM include the herpes viruses (EBV, VZV, HSV), CMV mumps, rubella, influenza, hepatitis A, B, C, HIV. 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 SyndromeProgressive lower limb weakness (proximal>distal)Ascends to upper limbs & bifacial weaknessArreflexiaNormal sensory examination No bladder symptomsNo band like sensationNo level for sensory loss

CharacteristicsTransverse MyelitisGuillain-Barre SyndromeMotor findingsParaparesis or quadriparesisAscending weakness LE > UE in the early stagesSensory findingsUsually can diagnose a spinal cord levelAscending sensory loss LE > UE in the early stagesAutonomic findingsEarly loss of bowel and bladder controlAutonomic dysfunction of the (CV) systemCranial nerve NoneEOM palsies or facial weaknessElectrophysiologic findingsEMG/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 potentialsEMG/NCV findings confined to the PNS: motorand/or sensory nerve conduction velocityreduced, distal latencies prolonged; conductionblock; reduced H reflex usually presentMRI findingsUsually a focal area of increased T2 signal with or without gadohnium enhancementNormalCSFUsually, CSF pleocytosis and/or increased IgG indexUsually, elevated protein in the absence of CSF pleocytosis

Acute Transverse MyelopathyThe three main categories in the differential diagnosis of ATM are demyelination, including multiple sclerosis (MS)neuromyelitis optica (NMO), and idiopathic transverse myelitis;

DIAGNOSTIC CRITERIA FOR TRANSVERSE MYELITISBilateral (not necessarily symmetric) sensorimotor and autonomic spinal cord dysfunctionClearly defined sensory levelProgression to nadir of clinical deficits between 4 hours and 21 days after symptom onsetDemonstration of spinal cord inflammation: cerebrospinal fluid pleocytosis or elevated IgG index,or MRI revealing a gadolinium-enhancing cord lesionExclusion of compressive, postradiation, neoplastic, and vascular causes

CRITERIA FOR DIAGNOSIS OF ACUTE TRANSVERSE MYELITISInflammation within the spinal cord demonstrated byCSF pleocytosis orelevated IgG index orgadolinium enhancement (If none of the inflammatory criteria is met at symptom onset, repeat MRI and LP evaluation between 27 days following symptom onset).IgG index = : (CSF IgG + serum IgG) (CSF albumin + serum albumin)

MANAGEMENT OF TMCare of the paraplegic patientMultidisciplinary rehab approach is keyIntravenous SteroidsPlasma Exchange (PLEX)

Chronic Management of TM

Intravenous Steroids Corticosteroids have multiple mechanisms of action includingantiinflammatory activityimmunosuppressive propertiesantiproliferative actions.methylprednisolone therapy high-dose methylprednisolone (30mg /kg (max 1 g) IV daily for 37 days) is typically first-line treatmentearly 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 ;intravenous immunoglobulinplasma exchangesRituximabcyclophosphamide.

Plasma Exchange (PLEX)PLEX is often initiated if a patient has moderate to severe TM inability to walkmarkedly impaired autonomic functionsensory loss in the lower extremities and 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 onsetwith a corresponding improvement in CSF and MRI abnormalitiesrecovery :-Evidence of at least some recovery is expected to begin within 6 monthsmost patients show some improvement in neurologic function within 8 weeksalthough recovery can take a more prolonged course of up to 2 years

Follow-UpResidual neurologic deficits include fixed weaknesssensory, or autonomic deficits. Sphincter dysfunction improves more slowly than the other deficits.

Follow-UpATM may be the presenting feature of MS, especially in patients with partial ATM abnormal initial brain MRI, in such cases, follow up MRIs should be considered.

Follow-UpHistory of other neurologic symptoms such as internuclear ophthalmoplegiaoptic neuritisfocal weakness and numbness that lasted at least 24 hours to days, have now resolved completelyother 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.

Outcomes1/3 pts have a complete recovery1/3 pts have some residual deficit1/3 pts have no improvement from nadir

Prognosisrisk factors for unfavorable outcomes at presentation, including rapid progression to maximal neurologic deficit (