ID
Myelitis
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Myelitis
, Transverse Myelitis, Acute Transverse Myelitis
See Also
Acute Flaccid Paralysis
Acute Flaccid Myelitis
Spinal Cord Syndrome
Cauda Equina Syndrome
Acute Motor Weakness Causes
Paresthesia Causes
Peripheral Neuropathy
Symmetric Peripheral Neuropathy
(
Polyneuropathy
)
Asymmetric Peripheral Neuropathy
(
Mononeuropathy
)
Guillain Barre Syndrome
Cervical Radiculopathy
Lumbar Radiculopathy
Spinal Epidural Abscess
Epidural Hematoma
Cervical Spine Injury
Thoracic Spine Injury
Lumbar Spine Injury
Floppy Infant
Definitions
Myelitis
Spinal Cord Inflammation of various causes including
Viral Infection
s, toxins, autoimmune or vascular conditions
Findings include weakness, sensory changes (
Paresthesia
s, numbness),
Autonomic Dysfunction
(e.g.
Urinary Incontinence
)
Transverse Myelitis
Spinal cord inflammation of a transverse segment of the cord with demyelination and necrosis
Myelitis findings occur below the segment of spinal cord involvement
Epidemiology
No gender or familial predisposition
Incidence
(rare): 1.3 to 4.6 per million people
U.S. 1400 new cases per year
Ages: Bimodal distribution
Most common in children and young adults (peak age 10 to 19 years old)
Further bimodal distribution in children (age <3 years and age 5 to 17 years)
Affects 0.2 per 100,000 children per year
Accounts for 20% of acute demyelinating syndrome in children
Associated with
Viral Infection
or
Vaccination
in 60% of cases
Second peak at age 30 to 39 years old
Pathophysiology
Spinal cord inflammation due to autoimmune, inflammatory, vascular or infectious causes
Lymphocyte
and
Monocyte
infiltration
Associated with demyelination and axonal injury
Myelitis may be unilateral or bilateral, and may effect only some long tracts but not others
May affect both ascending (sensory) and descending (motor) pathways
The terms Myelitis, Transverse Myelitis and Acute Transverse Myelitis are used interchangeably
The adjective transverse only describes the distribution, but the causes and management are similar
Causes
Myelitis
See
Acute Flaccid Paralysis
See
Acute Flaccid Myelitis
Precautions
Do NOT miss
Compressive Neuropathy
(e.g.
Cauda Equina Syndrome
) which is curable with early surgical intervention
Demyelinating Disease (most common cause)
Acute Disseminated Encephalomyelitis
(ADEM)
Multiple Sclerosis
Neuromyelitis Optica Spectrum Disorder
(
NMOSD
)
Myelin Oligodendrocyte
Glycoprotein
Antibody
Disease
Autoimmune or Inflammatory Myelitis
Ankylosing Spondylitis
Antiphospholipid Antibody Syndrome
Behcet Disease
Celiac Disease
Graft-vs-host disease
Mixed Connective Tissue Disease
(MCTD)
Neurosarcoidosis
Scleroderma
Sjogren Syndrome
Systemic Lupus Erythematosus
Bacteria
l Myelitis
Bartonella Henselae
(
Cat Scratch Disease
)
Borrelia Burgdorferi
(
Lyme Disease
)
Brucellosis
melitensis (
Brucellosis
)
Campylobacter jejuni
(e.g.
Acute Diarrhea
)
Chlamydia psittaci
(
Psittacosis
)
Chlamydia pneumoniae
(
Chlamydia Pneumonia
)
Coxiella Burnetii
(
Q Fever
)
Legionella pneumonia
Leptospira
(
Leptospirosis
)
Mycobacterium tuberculosis
Orientia Tsutsugamushi (
Scrub Typhus
)
Salmonella
paratyphi B (Paratyphoid)
Streptococcus
(Group A and B)
Treponema pallidum
(
Syphilis
)
Viral Myelitis
Coxsackievirus (A and B)
Cytomegalovirus
(Mono-Like Illness)
Dengue
Virus
Echoviruses
Enterovirus (70 and 71)
Epstein-Barr Virus
(
Mononucleosis
)
Hepatitis A Virus
Hepatitis C
Virus
Herpes Simplex Virus
Type 2 (
Genital Herpes
)
Elsberg Syndrome
is bilateral lumbosacral radiculitis with lower cord Myelitis due to HSV reactivation
HIV Infection
(see
HIV related Myelitis
)
Influenza
A
Virus
(includes H1N1)
Japanese Encephalitis Virus
Measles
Virus
Mumps
Virus
Polio
virus (1, 2, and 3)
St. Louis
Encephalitis
Virus
Varicella Zoster Virus
(
Chicken Pox
,
Shingles
)
West Nile Virus
Parasitic Myelitis
Acanthamoeba (
Granuloma
tous Amebic
Encephalitis
)
Echinococcus
Granulosus (
Echinococcosis
)
Gnat
hostoma Angiostrongylus (
Eosinophil
ic
Meningitis
)
Paragonimus westermani fluke (Paragonimiasis)
Schistomosoma (
Schistosomiasis
)
Taenia solium
(
Neurocysticercosis
)
Toxoplasma gondii
(
Toxoplasmosis
)
Trypanosoma brucei (African trypanosomiasis, African
Sleep
ing Sickness)
Fungal Myelitis
Actinomyces (
Actinomycosis
)
Aspergillus
(
Aspergillosis
)
Blastomyces (
Blastomycosis
)
Cryptococcus (Cryptococcosis)
Coccidioides immitis
(
Coccidioidomycosis
,
Valley Fever
)
Paraneoplastic Conditions (
Antibody
mediated)
Various antibodies (e.g. ANNA-2, GAD65,
NMDA
R)
Toxins or Drugs
Poisoning due to Brown Recluse Spider Venom
Heroin
abuse
Post-
Vaccination
Myelitis
Findings
Symptoms and Signs
See
Spinal Cord Syndrome
Timing
Onset over hours to days
Progression over days to weeks
Distribution
Myelitis typically affects a full triad of sensory, motor and
Autonomic Dysfunction
once disease has progressed
However, at onset, findings may be more subtle and the full triad may be absent
Myelitis is typically bilateral but may be unilateral in early or atypical cases (partial Transverse Myelitis)
Myelitis may only cause sensory and motor changes at a few spinal levels (sparing lower cord levels)
Myelitis may effect only some long tracts but not others
Motor weakness
Leg flexors and arm extensors are preferentially affected (pyramidal distribution)
Lower extremity weakness and diminished
Muscle
tone are frequent initial presenting symptoms
Patients may present with acute flaccid limb weakness and hyporeflexia (
Spinal Shock
)
Distinguish from
Guillain Barre Syndrome
With progression over time, half of patients develop an inability to move their legs
Chronically develop
Upper Motor Neuron Deficit
s (hyperreflexia, positive babinski, increased tone)
Sensory changes
Sensory findings may follow a
Dermatomal Distribution
Distinguish from
Peripheral Neuropathy
or neuropathic pain
Paresthesia
s
Typically initial sensory symptom
Paresthesia
s ascend from the feet proximally
Numbness
Pain
Autonomic Dysfunction
Urinary Incontinence
Urine Urgency
Urine Retention
Stool Incontinence
Constipation
Tenesmus
Temperature
dysregulation
Hypertension
Sexual Dysfunction
Associated Symptoms
Back pain at or near the level of Myelitis (variably present)
Labs
Cerebrospinal fluid (
Lumbar Puncture
)
Indicated after structural causes are excluded by neuroimaging
Cerebrospinal fluid (CSF) may be normal in up to 50% of cases (esp. early in course)
CSF
Pleocytosis
(>5 white cells/uL) is present in a majority of cases
CSF White Blood Cell
s >100/uL in >50% of cases
CSF IgG Index
Often increased in Transverse Myelitis
Obtain other standard CSF labs (e.g.
CSF Protein
,
CSF Glucose
,
CSF Culture
, CSF viral panel, CSF VRDL, oligoclonal bands)
Exclude alternative diagnoses
Exclude alternative diagnoses
Serum
Vitamin B12
Level (and consider methylmalonic acid level)
Thyroid Stimulating Hormone
Syphilis Serology
HIV Test
Consider specific organism testing (e.g.
Mycoplasma
)
Consider other testing per neurology (e.g. ANA, Neuomyelitis optica Ig)
Imaging
MRI Spine with gadolinium contrast
Imaging the entire spine (cervical, thoracic and
Lumbar Spine
) is typically recommended to avoid
False Negative
testing
At minimum, obtain imaging to include well above the level of involvement
May also obtain combined spine MRIs (e.g. cervico-thoracic MRI or thoracolumbar MRI)
Exclude structural compressive causes (e.g.
Epidural Abscess
,
Epidural Hematoma
, acute disc
Hernia
tion)
May indicate surgical emergency
May also demonstrate inflammatory cord changes (gadolinium enhancement)
Findings consistent with Transverse Myelitis
T2 weighted images with high intensity signals at lesions
Transverse Myelitis lesions typically span at least 2
Vertebra
l levels
Multiple Sclerosis
related lesions typically span only one level
Neuromyelitis Optica Spectrum Disorder
(
NMOSD
) typically spans 3 or more levels
CT Myelogram
Alternative if MRI contraindicated
MRI Brain
Consider to exclude other demyelinating disease
Differential Diagnosis
See
Acute Motor Weakness Causes
See
Paresthesia Causes
See
Spinal Cord Syndrome
See
Floppy Infant
Peripheral Neuropathy
Symmetric Peripheral Neuropathy
(
Polyneuropathy
, includes
Guillain Barre Syndrome
)
Asymmetric Peripheral Neuropathy
(
Mononeuropathy
)
Botulism
Structural spinal cord disorders
Cervical Radiculopathy
Lumbar Radiculopathy
Cauda Equina Syndrome
Spinal Epidural Abscess
Epidural Hematoma
Acute vascular
Myelopathy
with ischemia (e.g.
Anterior Spinal Artery
thrombosis)
Spinal Cord Injury
Cervical Spine Injury
Thoracic Spine Injury
Lumbar Spine Injury
Diagnosis
Idiopathic Acute Transverse Myelitis
Progression to nadir (low point) with 4 hours to 21 days from symptom onset in Acute Transverse Myelitis
Nadir <4 hours from onset suggests acute vascular
Myelopathy
with ischemia (e.g.
Anterior Spinal Artery
thrombosis)
Nadir >21 days from onset suggests chronic progressive
Myelopathy
(e.g. AV fistula, MS)
Sensory, Motor or
Autonomic Dysfunction
attributable to spinal cord lesion (all 3 are typically present at nadir)
Sensory deficits have a clearly defined level
Findings are bilateral (but may be asymmetric)
Spinal cord inflammation
CSF
Pleocytosis
or IgG Index elevated or MRI Gadolinium enhancement
Repeat MRI and LP again in 2 to 7 days if inflammation not present on initial testing
Exclusion Criteria
Not due to extra-axial compression on MRI or CT myelography
Spinal radiation in last 10 years
Deficits consistent with alternative diagnosis
Anterior Spinal Artery
thrombosis
Spinal cord
Arteriovenous Malformation
Demyelinating disease (e.g.
Multiple Sclerosis
,
Optic Neuritis
)
Connective Tissue Disorder
(e.g.
Sarcoidosis
, Behcet Disease,
Sjogren Syndrome
,
Systemic Lupus Erythematosus
)
CNS Disorders (e.g. HIV, Tertiary
Lyme Disease
,
Neurosyphilis
, HTLV1,
Viral Encephalitis
)
Management
Supportive Care
Admit patients
ABC Management
Keep patient NPO if
Dysarthria
or
Dysphagia
Consider
Advanced Airway
indications
Significant
Urinary Retention
may require urine catheterization
Condition specific management
Consult neurology early
Consider infectious disease
Consultation
if infectious Myelitis is suspected (12% of cases)
High dose
Corticosteroid
s
Consult neurology for indications based on presentation and findings
Methylprednisolone
1000 mg IV daily in divided doses for 3 to 5 days
Plasma exchange
Indications
Corticosteroid
s contraindicated
Not effective if not
Corticosteroid
responsive
Life threatening complications (e.g. respiratory compromise)
Consider as adjunctive therapy (per neurology recommendations)
Dosing
Exchange transfusion every other day for 10 to 14 days (total of 5 to 7 exchanges)
Intravenous Immunoglobulin
(IVIG)
Indications
Adjunct to
Corticosteroid
s (consult neurology for indications)
Dosing
IVIG 2 g/kg IV for 2 to 5 days
Compressive neureopathy causes of acute Myelitis are surgical emergencies
Emergent
Consultation
with
Spine Surgery
or neurosurgery
Prognosis
Idiopathic Transverse Myelitis
Gradual improvement over 3 months (and often further improvement over the next year)
Partial or complete resolution in 50 to 70% of patients
Episodes tend to be monophasic (non-recurring)
Secondary Transverse Myelitis
Variable outcomes depending on cause
Demyelinating disease are at risk of recurrence
Multiple Sclerosis
is associated with significant recovery with episodes (full in some cases)
Neuromyelitis Optica Spectrum Disorder
(
NMOSD
) is associated with significant residual deficits
Overall poor prognostic signs
Severe weakness
Hypotonia
Areflexia
Spinal Shock
Pseudoexacerbation (recrudescence)
Neurologic deficits may recur transiently with acute infections or metabolic disturbances
Similar to recrudescence seen after
Cerebrovascular Accident
Findings abate with treatment with the acute trigger
Differentiate from recurrence as seen with demyelinating diseases (e.g. MS,
NMOSD
)
Resources
Transverse Myelitis Fact Sheet (NINDS)
https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Transverse-Myelitis-Fact-Sheet
References
Lindquist and Stephanos in Swadron (2023) EM:Rap 37(3): 4-12
West (2013) Discov Med 16(88):167-77 +PMID: 24099672 [PubMed]
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