Myelitis

Aka: Myelitis, Transverse Myelitis, Acute Transverse Myelitis

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II. Definitions

  1. Myelitis
    1. Spinal Cord Inflammation of various causes including viral infections, toxins, autoimmune or vascular conditions
    2. Findings include motor weakness, sensory changes (Paresthesias, numbness, pain) and Urinary Incontinence
  2. Transverse Myelitis
    1. Spinal cord inflammation of a transverse segment of the cord with demyelination and necrosis
    2. Myelitis findings occur below the segment of spinal cord involvement

III. Epidemiology

  1. Incidence (rare): 1.3 to 4.6 per million people
  2. Ages
    1. Most common in children and young adults

IV. Pathophysiology

  1. Spinal cord inflammation due to autoimmune, inflammatory, vascular or infectious causes
  2. Myelitis may be unilateral or bilateral, and may effect only some long tracts but not others
    1. The terms Myelitis, Transverse Myelitis and Acute Transverse Myelitis are used interchangeably
    2. The adjective transverse only describes the distribution, but the causes and management are similar

V. Findings: Symptoms and Signs

  1. See Spinal Cord Syndrome
  2. Timing
    1. Onset over hours to days
    2. Progression over days to weeks
  3. Distribution
    1. Myelitis is typically bilateral but may be unilateral
    2. Myelitis may only cause sensory and motor changes at a few spinal levels (sparing lower cord levels)
    3. Myelitis may effect only some long tracts but not others
  4. Motor weakness
    1. Leg flexors and arm extensors are preferentially affected (pyramidal distribution)
  5. Sensory changes
    1. Paresthesias
      1. Typically initial sensory symptom
      2. Paresthesias ascend from the feet proximally
    2. Numbness
    3. Pain
  6. Autonomic Dysfunction
    1. Urinary Incontinence
    2. Stool Incontinence
    3. Temperature dysregulation
    4. Hypertension
  7. Associated Symptoms
    1. Back pain at or near the level of Myelitis (variably present)

VI. Labs

  1. Cerebrospinal fluid (Lumbar Puncture)
    1. Indicated after structural causes are excluded by neuroimaging
    2. Cerebrospinal fluid (CSF) is normal in 50% of cases

VII. Imaging

  1. MRI Spine with gadolinium contrast
    1. Imaging to include well above the level of involvement
      1. May require combined spine MRIs (e.g. cervico-thoracic MRI or thoracolumbar MRI)
    2. Exclude structural compressive causes (e.g. Epidural Abscess, Epidural Hematoma, acute disc Herniation)
      1. Surgical emergencies if present
    3. May also demonstrate inflammatory cord changes
  2. CT Myelogram
    1. Alternative if MRI contraindicated

VIII. Differential Diagnosis

  1. See Acute Motor Weakness Causes
  2. See Paresthesia Causes
  3. See Spinal Cord Syndrome
  4. See Floppy Infant
  5. Peripheral Neuropathy
    1. Symmetric Peripheral Neuropathy (Polyneuropathy, includes Guillain Barre Syndrome)
    2. Asymmetric Peripheral Neuropathy (Mononeuropathy)
    3. Botulism
  6. Structural spinal cord disorders
    1. Cervical Radiculopathy
    2. Lumbar Radiculopathy
    3. Cauda Equina Syndrome
    4. Spinal Epidural Abscess
    5. Epidural Hematoma
  7. Spinal Cord Injury
    1. Cervical Spine Injury
    2. Thoracic Spine Injury
    3. Lumbar Spine Injury

IX. Causes: Myelitis

  1. See Acute Flaccid Paralysis
  2. See Acute Flaccid Myelitis
  3. Precautions
    1. Do NOT miss Compressive Neuropathy (e.g. Cauda Equina Syndrome) which is curable with early surgical intervention
  4. Demyelinating Disease (most common cause)
    1. Acute Disseminated Encephalomyelitis (ADEM)
    2. Multiple Sclerosis
    3. Neuromyelitis optica
    4. Post-Vaccination Myelitis
  5. Autoimmune or Inflammatory Myelitis
    1. Ankylosing Spondylitis
    2. Antiphospholipid Antibody Syndrome
    3. Behcet Disease
    4. Celiac Disease
    5. Graft-vs-host disease
    6. Mixed Connective Tissue Disease (MCTD)
    7. Neurosarcoidosis
    8. Scleroderma
    9. Sjogren Syndrome
    10. Systemic Lupus Erythematosus
  6. Bacterial Myelitis
    1. Bartonella Henselae (Cat Scratch Disease)
    2. Borrelia Burgdorferi (Lyme Disease)
    3. Brucellosis melitensis (Brucellosis)
    4. Campylobacter jejuni (e.g. Acute Diarrhea)
    5. Chlamydia psittaci (Psittacosis)
    6. Chlamydia pneumoniae (Chlamydia Pneumonia)
    7. Coxiella Burnetii (Q Fever)
    8. Legionella pneumonia
    9. Leptospira (Leptospirosis)
    10. Mycobacterium tuberculosis
    11. Orientia Tsutsugamushi (Scrub Typhus)
    12. Salmonella paratyphi B (Paratyphoid)
    13. Streptococcus (Group A and B)
    14. Treponema pallidum (Syphilis)
  7. Viral Myelitis
    1. Coxsackievirus (A and B)
    2. Cytomegalovirus (Mono-Like Illness)
    3. DengueVirus
    4. Echoviruses
    5. Enterovirus (70 and 71)
    6. Epstein-Barr Virus (Mononucleosis)
    7. Hepatitis A Virus
    8. Hepatitis CVirus
    9. Herpes Simplex Virus Type 2 (Genital Herpes)
    10. HIV Infection (see HIV related Myelitis)
    11. Influenza A Virus (includes H1N1)
    12. Japanese Encephalitis Virus
    13. MeaslesVirus
    14. Mumps Virus
    15. Poliovirus (1, 2, and 3)
    16. St. Louis EncephalitisVirus
    17. Varicella Zoster Virus (Chicken Pox, Shingles)
    18. West Nile Virus
  8. Parasitic Myelitis
    1. Acanthamoeba (Granulomatous Amebic Encephalitis)
    2. Echinococcus Granulosus (Echinococcosis)
    3. Gnathostoma Angiostrongylus (Eosinophilic Meningitis)
    4. Paragonimus westermani fluke (Paragonimiasis)
    5. Schistomosoma (Schistosomiasis)
    6. Taenia solium (Neurocysticercosis)
    7. Toxoplasma gondii (Toxoplasmosis)
    8. Trypanosoma brucei (African trypanosomiasis, African Sleeping Sickness)
  9. Fungal Myelitis
    1. Actinomyces (Actinomycosis)
    2. Aspergillus (Aspergillosis)
    3. Blastomyces (Blastomycosis)
    4. Cryptococcus (Cryptococcosis)
    5. Coccidioides immitis (Coccidioidomycosis, Valley Fever)
  10. Paraneoplastic Conditions (Antibody mediated)
    1. Various antibodies (e.g. ANNA-2, GAD65, NMDAR)

X. Management

  1. Condition specific management
    1. Consult neurology
    2. Consider infectious disease Consultation if infectious Myelitis is suspected (12% of cases)
  2. Corticosteroids may be indicated in some cases
  3. Compressive neureopathy causes of acute Myelitis are surgical emergencies
    1. Emergent Consultation with spine surgery or neurosurgery

XI. Prognosis

  1. Moderate to severe residual deficits remain in two thirds of Transverse Myelitis patients

XII. Resources

  1. Transverse Myelitis Fact Sheet (NINDS)
    1. https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Transverse-Myelitis-Fact-Sheet

XIII. References

  1. West (2013) Discov Med 16(88):167-77 +PMID: 24099672 [PubMed]

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Ontology: Myelitis (C0026975)

Definition (MSHCZE) Zánět míchy, (srov. encefalomyelitida), ve složenině i dřeně (srov. osteomyelitida). (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (NCI_CTCAE) A disorder characterized by inflammation involving the spinal cord. Symptoms include weakness, paresthesia, sensory loss, marked discomfort and incontinence.
Definition (NCI) An inflammatory process affecting the spinal cord. Causes include viral infections, autoimmune disorders, vascular disorders, and toxic agents. Symptoms include weakness, paresthesia, sensory loss, pain, and incontinence.
Definition (MSH) Inflammation of the spinal cord. Relatively common etiologies include infections; AUTOIMMUNE DISEASES; SPINAL CORD; and ischemia (see also SPINAL CORD VASCULAR DISEASES). Clinical features generally include weakness, sensory loss, localized pain, incontinence, and other signs of autonomic dysfunction.
Concepts Disease or Syndrome (T047)
MSH D009187
SnomedCT 192682002, 267682000, 41370002
English Myelitides, MYELITIS, INFLAMM SPINAL CORD, MYELOPATHY INFLAMM, SPINAL CORD INFLAMM, Myelitis, Myelitis NOS, Inflammation, Spinal Cord, Inflammations, Spinal Cord, Spinal Cord Inflammation, Spinal Cord Inflammations, Inflammatory Myelopathies, Inflammatory Myelopathy, Myelopathies, Inflammatory, Myelopathy, Inflammatory, Myelitis [Disease/Finding], spinal cord inflammation, myelitis (diagnosis), myelitis, Myelitis (disorder), inflammation; spinal cord, spinal cord; inflammation, Myelitis, NOS
French MYELITE, Myélite SAI, Myélopathie inflammatoire, Myélite, Inflammation de la moelle épinière
Portuguese MIELITE, Mielite NE, Inflamação da Medula Espinal, Inflamação da Medula Espinhal, Mielite, Mielopatia Inflamatória
Spanish MIELITIS, Mielitis NEOM, mielitis (trastorno), mielitis, Mielitis, Inflamación de la Médula Espinal, Mielopatía Inflamatoria
German MYELITIS, Myelitis NNB, Myelitis, Entzündung des Rückenmarks, Myelopathie, entzündliche, Rückenmarkentzündung
Dutch myelitis NAO, ontsteking; ruggenmerg, ruggenmerg; ontsteking, myelitis, Myelitis, Myelopathie, inflammatoire, Ruggenmergontsteking
Italian Mielite NAS, Infiammazione del midollo osseo, Mielopatia infiammatoria, Infiammazione del midollo spinale, Mielite
Japanese 脊髄炎ＮＯＳ, ｾｷｽﾞｲｴﾝ, ｾｷｽﾞｲｴﾝNOS, 脊髄炎
Swedish Ryggmärgsinflammation
Czech myelitida, mícha - zánět, Myelitida NOS, Myelitida, zánět míchy
Finnish Myeliitti
Russian MIELIT, МИЕЛИТ
Croatian MIJELITIS
Polish Zapalenie rdzenia kręgowego, Zapalenie rdzenia
Hungarian Myelitis, Myelitis k.m.n.
Norwegian Betennelse i ryggmargen, Myelitt, Ryggmargsbetennelse
Derived from the NIH UMLS (Unified Medical Language System)

Ontology: Myelitis, Transverse (C0026976)

Definition (MSH) Inflammation of a transverse portion of the spinal cord characterized by acute or subacute segmental demyelination or necrosis. The condition may occur sporadically, follow an infection or vaccination, or present as a paraneoplastic syndrome (see also ENCEPHALOMYELITIS, ACUTE DISSEMINATED). Clinical manifestations include motor weakness, sensory loss, and incontinence. (Adams et al., Principles of Neurology, 6th ed, pp1242-6)
Concepts Disease or Syndrome (T047)
MSH D009188
SnomedCT 192682002, 16631009
LNC LP128698-0, MTHU040639
English Myelitis, Transverse, MYELITIS TRANSVERSE, Myelitis transverse, Transverse Myelitis, Transverse myelitis NOS, TRANSVERSE MYELITIS ACUTE OR SUBACUTE, transverse myelitis (diagnosis), transverse myelitis, Transverse Myelopathy Syndrome, Transverse Myelopathy Syndromes, Myelitis, Transverse [Disease/Finding], myelitis transverse, Transverse myelopathy syndrome, Transverse myelitis, Transverse myelopathy syndrome (disorder), myelitis; transverse, transverse; myelitis
Portuguese MIELITE TRANSVERSA, Mielite Transversa Aguda em Doença Desmielinizante do Sistema Nervoso Central, Mielite transversa, Mielite Transversa
Spanish MIELITIS TRANSVERSA, Mielitis Transversa Aguda en Enfermedad Desmielinizante del Sistema Nervioso Central, mielitis transversa, síndrome de mielopatía transversa (trastorno), síndrome de mielopatía transversa, Mielitis transversa, Mielitis Transversa
German MYELITIS TRANSVERSA, Myelitis transversa, Querschnittsmyelitis
Swedish Myelit, transversell
Japanese ｵｳﾀﾞﾝｾｲｾｷｽﾞｲｴﾝ, 横断脊髄炎, 脊髄炎-横断, 横断性脊髄炎
Finnish Selkäytimen poikittaistulehdus
Russian MIELIT POPERECHNYI, МИЕЛИТ ПОПЕРЕЧНЫЙ
French MYELITE TRANSVERSE, Myélite transverse
Czech Myelitida transverzální, transverzální myelitida, myelitida z příčné léze
Italian Mielopatia trasversale, Mielite trasversa
Polish Zapalenie rdzenia poprzeczne
Hungarian Myelitis transversa
Norwegian Transversell myelitt, Myelitt, transversell
Dutch myelitis; transversa, transversa; myelitis, myelitis transversa, Myelitis transversa
Derived from the NIH UMLS (Unified Medical Language System)

Ontology: Myelitis, Acute Transverse (C0270627)

Concepts Disease or Syndrome (T047)
MSH D009188
ICD9 341.20, 341.2
ICD10 G37.3
SnomedCT 47000000
English Acute (transverse) myelitis NOS, Acute (transverse) myelitis, Acute Transverse Myelitis, Transverse Myelitis, Acute, Acute myelitis NOS, Acute transverse myelitis NOS, acute myelitis transverse, acute transverse myelitis, acute transverse myelitis of central nervous system, acute transverse myelitis of central nervous system (diagnosis), acute transverse myelitis of CNS, Acute transverse myelitis, Acute transverse myelitis (disorder), Myelitis, Acute Transverse
French Myélite aigüe transverse, Myélite transverse aigüe, Myélite transverse aiguë, Myélite aiguë transverse
Norwegian Akutt transversell myelitt
Czech akutní transverzální myelitida
Spanish mielitis transversa aguda (trastorno), mielitis transversa aguda
Derived from the NIH UMLS (Unified Medical Language System)

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