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MYELOPATHY

Anatomical syndromes   Anterior cord   Cauda equina   Central cord   Hemicord (Brown-Séquard)   Inferior cord (Conus medullaris)   Posterior cord Systemic disorders Traumatic myelopathy Vascular myelopathy

From: M Al-Lozi

• Central cord
  • Etiologies
    • Trauma: Especially with
      • Cervical arthritis
        
      • Older patients
    • Spondylitic myelopathy
    • Syringomyelia
    • Neoplasm: Metastatic, glial, lymphoma
  • Sensory loss
    • Pain & temperature > posterior column sensations
      
    • Sparing: Sacral
  • Weakness: Arms > legs
  • May occur in absence of spinal fracture
• Anterior cord
  • Etiology: Anterior spinal artery territory ischemia
  • Loss: Motor function & pin & temperature sensation
  • Relative preservation: Position sense
• Posterior cord
  • Etiology
    • Posterior spinal artery damage
    • Diffuse atherosclerosis: Deficient collateral perfusion
    • B₁₂ deficiency
  • Loss: Position sense
  • Relative preservation: Motor, pain & temperature
  • Prognosis: Better than anterior syndrome
• Brown-Séquard: Hemicord injury
  • Etiologies
    • Trauma: Penetrating
    • Radiation
    • Decompression sickness
  • Ipsilateral loss
    • Weakness
    • Position sense
  • Contralateral loss
    Pain & temperature
    
• Conus medullaris: Inferior cord
  • Onset: Rapid
  • Symmetric defects
  • Pain: Inconstant; Perineum & thighs
  • Weakness: Sacral
  • Sensory
    • Saddle (sacral distribution)
    • May be dissociated (syringomyelic)
  • Tendon reflex loss: Ankle > Knee
  • Bladder involvement; Impotence: Consistent; Early
• Cauda equina
  • Clinical
    • Onset: Progressive; Slow; Unilateral
    • Exercise provocation of symptoms
      • Walking downhill exacerbates symptoms
      • Normal bicycle exercise
    • Asymmetric
    • Pain: Severe; Radicular distribution
    • Sensory loss
      • All modalities
      • Lumbar > sacral
    • Weakness: Lumbar > sacral
    • Fasciculations: Common
    • Tendon reflex loss: Knee > Ankle
    • Bladder involvement: Occasional; Late
    • Radicular distribution of lesions: Below L2
  • Common etiology: Lumbar spinal stenosis
  • Also see
    • Lumbosacral plexus lesion
    • Lumbosacral radiculopathy

Intrinsic  Skeletal

Cervical spinal stenosis

Cervical stenosis Osteophyte

• Anatomy
  • Anterior compression: Protruding disc or posterior osteophytes;
  • Anterolateral compression: Joints of Luschka
  • Lateral compression: Cervical facets
  • Posterior compression: Ligamentum flavum
• Atlanto (C1)-Axial (C2) Instability
  • Rheumatoid Arthritis
  • Trisomy 21
  • Mucopolysaccharidosis
    • Morquio's Syndrome (Mucopolysaccharidosis type IV), types A & B
    • Maroteaux-Lamy Syndrome (Mucopolysaccharidosis type VI) : Rare
      • Usual cause of cervical spinal cord syndrome: Thickening of posterior longitudinal ligament
    • Sly syndrome (Mucopolysaccharidosis type VII)
  • Skeletal dysplasias
    • Type II Collagenopathies
    • Opsismodysplasia
    • Spondyloepiphyseal dysplasia (SED) with atlantoaxial instability
    • Pseudoachondroplastic dysplasia
    • Spondylometaepiphyseal Dysplasia, Short limb-hand type
    • Dyggve-Melchior-Clausen disease
    • Shprintzen-Goldberg craniosynostosis
    • Cleidocranial dysplasia
    • Odontoid hypoplasia
  • Ankylosing spondylitis
  • CODAS
  • Faciogenital dysplasia
  • Lesch-Nyhan syndrome ⁸: Also see multisystem ataxia
  • Dystonia
  • Congenital ⁴
  • Animals
    • Horses: Arabian
    • Dogs: Small toy breeds; Yorkshire terriers
• Spinal canal stenosis
  • Familial
    • Achondroplasia
    • Coffin Lowry
    • Stickler dysplasia
    • Hunter (Mucopolysaccharidosis 2)
    • Familial intervertebral disc disease
      • 60% to 74% Heritability of disc height & bulge in Monozygotic twins
      • Dominant familial cases
        ● ? Collagen IX α2 (COL9A2) ; Chromosome 1p33-p32.2; Dominant
        • Mutation: Gln326Trp
        • Other COL9A2 mutations: Multiple epiphyseal dysplasia, Dominant, Type2
      • Predisposition
        • Lumbar
          • COL11A1
          • THBS2
          • ASPN
          • CILP
        • Other
          • COL9A3
    • Familial Lumbar Stenosis
    • sub-Saharan Africans: Increased frequency of congenital narrow lumbar canals
    • Hypophosphatemia, Vitamin D resistant rickets
    • Spondyloarthropathy, Destructive + Renal failure, Chronic
    • Posterior longitudinal ligament
      • Ossification
        • Mouse model: Twy ('tip-toe' walking Yoshimura)
      • Thickening
        • Maroteaux-Lamy Syndrome (Mucopolysaccharidosis type VI)
        • Amyloid
    • ± Acrodysostosis (lumbar)
    • Animals (Wobbler syndrome)
      • Horses: Thoroughbred horses
      • Dogs: Great Danes (Young) & Doberman Pinschers (Old)
  • Acquired
    • Spinal hyperextension
    • Vertebral collapse
    • Soft disk herniation (Central)
    • Spondylosis: Disk disease & osteophyte formation
    • Epidural lipomatosis
    • Amyloid TTRwt ¹³: Biopsy ligamentum flavum
• Thoracolumbar Junction Region Stenosis ¹⁴
  • Anatomy
    • Spine level: T10 to L1
    • Spinal cord compression: Just above conus
  • Clinical
    • Gait Disorder
    • Leg numbness
    • Weakness (70%): Especially foot dorsiflexion
    • Other
      • Plantar response: Extensor (25%)
      • Tendon reflexes brisk (30%)
      • Urinary dysfunction (35%)
      • Back pain (35%)
• Cervical Spondylotic Myelopathy
  • Anatomy
    • Spinal cord compression
      • Commonly associated with disc protrusion
      • Canal diameter < 10 mm
    • Common levels
      • C3-4, C4-5, C5-6 & C6-7
      • One or multiple levels: Up to 5
      • More multiple at lower levels
  • Clinical syndromes: May overlap
    • Spastic paraparesis
      • Spasticity: Legs > Arms; Gait disorder
      • Tendon reflexes
        • Often increased in arms & legs
        • Reduced in regions with radiculopathy
      • Bladder dysfunction: More severe cord compression
    • Cervical amyotrophy
      • Distal type ¹⁰
        • Onset age: 39 to 69 years
        • Male > Female
        • Weakness: Intrinsic hand muscles & Finger extensors
        • Sensory loss: Arms
        • Pyramidal signs: Legs in some patients
        • MRI: Compression of spinal cord anterior horn, often ≥ 2 levels
      • Proximal type
    • Gait disorder
    • Course
      • Onset
        • Ages: Usual 5th & 6th decades; Range 14 to 80 years
        • May be precipitated by trauma
        • Neck pain (30%)
        • Paresthesias or Pain (30%): More common with C5-6, C6-7 or Multiple levels
        • Weakness (30%): More common with C3-4 or C4-5; Clumsy hands; Gait disorder
        • Vasomotor (1%)
      • Progression: Stable or Slow or Step-wise
  • MRI
    • Spinal cord ¹¹
      • T2 hyperintensity, spindle-shaped
      • Pancake-like gadolinium enhancement
        • Near maximal stenosis
        • White matter predominant
        • Spares gray matter
    • Paraspinous muscles: Atrophy
  • Treatment: Surgical decompression
    • Provides variable results
    • Poor outcome: Low T1 spinal cord signal
    • Complication: C5 root lesion
  • External link: Wheeless
• Cervical vertebral fusion: Klippel-Feil syndromes
  • General
    • Congenital anomaly
      • Defect in formation or segmentation of cervical vertebrae
      • Fused appearance
    • Clinical triad: All present in only 50% of patients
      • Neck length: Short
      • Neck movement: Limited
      • Posterior hairline: Low
  • Klippel-Feil syndrome 1 (KFS1)
    ● Growth/differentiation factor 6 (GDF6) ; Chromosome 8q22.1; Dominant
    
    
  • Klippel-Feil syndrome 2 (KFS2)
    ● Mesenchyme homeobox 1 (MEOX1) ; Chromosome 17q21.31; Recessive
    
    
  • Klippel-Feil syndrome 3 (KFS3)
    ● Growth/differentiation factor 3 (GDF3) ; Chromosome 12p13.31; Recessive
    
    
  • Klippel-Feil syndrome 4 (KFS4) + Nemaline Myopathy & Facial Dysmorphism ¹²
    ● Myosin XVIIIB (MYO18B) ; Chromosome 22q12.1; Recessive
    • Epidemiology: 11 patients
    • Genetics
      • Mutations: Stop
        • Saudi founder; Ser2302Ter (c.6905C-A transversion)
        • Myopathy without KFS: Arg1294Glyfs*34
        • Other: Glu2166* (c.6496G>T); Ile38Trpfs*18
      • Allelic disorder: Loss of heterozygosity in lung cancer
      • Mouse: Embryonic lethal
    • MYO18B protein
      • Sucellular Locations
        • Z-lines & adjacent regions: On F-actin (Punctate pattern)
        • Nuclei
      • Tissues: Skeletal & cardiac muscle
      • Functions
        • ? Ancillary protein to myosin 2
        • Expression coincides with myogenic differentiation
        • Interacts with the proteasomal subunit Sug1
        • Other functions: Tumor suppressor
      • KFS4: Loss of MYO18B transcript
      • Also see: Other myosins
    • Clinical
      • Onset
        • Age: Birth to 16 months
        • Motor: Hypotonia or Delay
      • Klippel-Feil: Congenital synostosis of regions of spine
      • Myopathy
        • Weakness: Distal > Proximal
        • Bulbar: Face weak; Speech nasal
        • Respiratory weakness: Mild
        • Gait: Clumsy or Steppage with falls
        • May be present without contractures
      • Tendon reflexes: Reduced
      • Cardiomyopathy (20%)
      • Skeletal
        • Short stature
        • Distal arthrogryposis: Hand ulnar deviation
        • Scoliosis
        • Microcephaly
        • Face: Ptosis; Nose bulbous; Alae nasi hypoplastic; Posterior hairline low
      • Hands: Reduced creases
    • Laboratory
      • Serum CK: Normal
      • Skeletal: Cervical fusion; Thoracolumbar scoliosis; Bilateral acetabular dysplasia
      • Brain MRI: Normal
      • EMG: Myopathic
      • Muscle pathology
        • Fiber size: Varied; Large & Small
        • Myofibrillar: Disarray; Loss or disruption of myosin filaments
        • Cytoplasm: Rods; Subsarcolemmal globular deposits of electron-dense material
        • Loss of thick myosin filaments
    
    
  • Klippel-Feil syndrome: Otofaciocervical syndrome 2 (OFC2)
    ● Paired Box Gene (PAX1) ; Chromosome 20p11.22; Recessive
    
    
  • Otofaciocervical syndrome 1 (OFC1)
    ● Eyes absent 1 (EYA1) ; Chromosome 8q13.3; Recessive
    
    
• Myelomeningocoele
• Mass lesions: Often epidural
  • Metastasis & Lymphoma
  • Epidural lipomatosis: Steroids; Obesity
  • Gout
  • Xanthoma
  • Large nerves: CMT 1
  • Amyloid
  • Sarcoid
  • Extramedullary hematopoesis
• External links
  • Image: Spondyloepiphyseal dysplasia
  • Radiology of cervical spine

• Immuner & Connective Tissue Disease
  • Systemic Lupus Erythematosis
    • Epidemiology
      • Most common connective tissue disease associated with myelopathy
      • Myelopathy occurs in < 1% of patients with lupus
    • Clinical
      • Onset: Rapid over hours to days; Occasionally slow progression over months
      • May progress to severe paraparesis
    • Laboratory
      • Associated with anti-cardiolipin antibodies
      • CSF: Glucose reduced; Protein increased; Mild pleocytosis
    • MRI
      • Diffuse increased signal intensity on T2
      • Signal reduced over time with treatment or recovery
    • Treatment: ? Corticosteroids ± Cyclophosphamide
  • Wegener's
    • Due to: Vasculitis or Pachymeningitis
  • Sjögren's
  • Mixed Connective Tissue
  • Progressive Systemic Sclerosis
  • Behçet syndrome ²
    • Onset: Subacute
    • Clinical syndromes
      • Transverse myelitis with paraplegia
      • Brown–Séquard syndrome
      • Sensory changes, Tendon reflexes increased, Sphincter disorders, No weakness
    • CSF: Pleocytosis
    • MRI: Multiple contrast enhancing intramedullary lesions ³
  • Sarcoid: Subpial enhancement on MRI
• Embolism
  • Decompression Sickness
  • Atherosclerotic
• Ischemic
  • Liver disease: Portosystemic shunting
  • Aortic Aneurysm
  • Waldenström's macroglobulinemia (Hyperviscosity syndromes)
• Paraneoplastic
• Infectious
  • HIV: Posterior columns
  • HTLV 1: Spastic paraparesis
• Vitamin & Nutritional
  • E deficiency
  • B₁₂ deficiency
  • Cuban epidemic neuropathy
• Toxic
• Amyloid
• Hepatic
• Idiopathic: Vernant's disease

Clinical features Epidemiology Late complications Partial cord injury syndromes   Anterior cord   Brown-Séquard   Cauda Equina   Conus medullaris   Hemicord   Posterior cord Treatment

• Male > Female: ~ 4:1
• Age groups
  • 16 to 30: 60%
  • 31 to 45: 20%
• Causes
  • Motor vehicle (especially alcohol-related): 50%
  • Violence (gunshot & stab wounds)
  • Falls: Especially young < 16 and old > 45
  • Sporting accidents: Diving > Football & Horseback riding
  • Also see: Surfer myelopathy

• Regions involved: With most spine mobility
  • C5 to C7
  • T10 to L2
• Mortality: 1 year
  • Paraplegia: 6% to 10%
  • Quadriplegia: 30% to 40%
• Late complications
  • CSF leak
  • Infections
  • Syringomyelia
  • Painful spasms: Especially with intercurrent infections
• Treatment
  • Acute
    • Mechanical stabilization of whole spine in neutral position
    • Airway maintenance
    • Blood pressure
    • Skin care
  • Hospital
    • Airway & shock management
    • Spine stabilization
    • ?? Agents to reduce cord damage
      ? Corticosteroids; Gangliosides; Naloxone....
    • Radiological & neurosurgical work-up

• Anterior spinal: Major artery
  • Originates from vertebral arteries
  • Discontinuous course
  • Input: 6 to 9 radicular arteries
• Artery of Adamkiewicz
  • Major radicular artery
  • Origin: Variable; Usually T9 to T12; Left > Right
• Other vascular supply
  • Circumflex anastomoses: From anterior & posterior spinals
  • Hypovascular region: T4 to T8

• Level
  • Lower thoracic: 50%
• Syndromes depend on location of lesion

• Aortic disease: Most common
  • Surgery; Thrombosis; Dissection; Trauma
• Arteriovenous malformation
• Systemic vascular disease
  • Atherosclerosis
  • Emboli
  • Cardiac arrest & hypotension
  • Lupus erythematosis
  • Coagulopathy
  • Syphilis
  • Anemia
  • Decompression sickness
• Local vascular compromise
  • Anterior spinal
    • Atlanto-axial dislocation
    • Epidural hematoma
    • Cervical spondylosis
    • ? Cocaine
  • Thoraco-Lumbar
    • Aortic disease
    • Spinal surgery & anesthesia
    • Liver disease: Porto-systemic shunting

• Functional Improvement: 33%
• Stable: 23%
• Death: 20%

SPINAL ARTERIOVENOUS MALFORMATIONS

Dural:Type I; Extramedullary 5 Anatomy & Physiology Location Thoracic (Lower) & Lumbar: 90% Sacral: 4% Cervical 3% Left > Right: 2:1 Feeding vessels Location: Often lumbar Number: Usually 1; Occasionally 2 or 3 Fistula in dorsolateral root sleeve High venous pressure in spinal cord Reduced spinal cord perfusion Epidemiology Male:Female = 4:1 Age of onset: Mean 58 years; Range 21 to 78 75% of all AVMs Clinical General Lumbar or sacral signs most common Poor correlation between lesion & level of symptoms Similar syndrome: Associated with remote pelvic AVM Initial symptom Gait disorder Sensory symptoms: Numbness or Paresthesias Pain (low back, or radicular) Leg weakness: Often asymmetric No change with Valsalva Hemorrhage: 5% to 25% Sensory Loss: Sacral 1st; Spinal level in 20% Paresthesias Pain: Back or Legs; 25% to 50% Motor Weakness Legs: Mild paraparesis most frequent May be asymmetric Progression to flaccid Lower motor neuron only in 35% Wasting: Proximal - buttocks & thighs Gait disorder Early in course: Exacerbated by exercise Later in course: Fixed Reflexes Tendon: Reduced or increased Plantar: Upgoing Anal & cremasteric: Reduced Bladder dysfunction Frequency: 80% More severe with conus AVM Vascular Bruit: Rare Cutaneous angioma More prominent with Valsalva ? Related to dural or intradural AVM Progression Gradual over months to years Short term exacerbations: Related to exercise Stepwise exacerbations: 20%; Related to ischemia or hemorrhage Full symptom complex (motor, sensory, bladder) after 1 year in 2/3 Severe gait disorder: 90% by 3 years Treatment 6 Endovascular Surgery Cut intradural venous connection to parenchyma Improvement in 60% to 80% Symptom recurrence may be 2° to new collaterals to AVM Benefit Motor & gait improvement most likely: Rarely reverts to normal Pain often reduced More improvement with lower thoracic AVMs Diagnosis Myelography: 90% positive Supine and prone films MRI 7 Use small field of view Eliminate motion artifact T2 signal or flow voids: Present in most Perform T1 sequence after gadolinium: Detects enhancement 86% positive Detects other lesions Spinal angiography7 Localizes fistula May be only positive diagnostic test (30% to 50%)

From M Al-Lozi Arteriogram: Tortuous vessels

MRI T2: Flow voids on dorsal cord surface

MRI T1: High signal in cord

• Types
  • II: Dural sinus drainage; Retrograde subarachnoid vein
  • III: Subarachnoid venous drainage without dural connection
• 10% of spinal AVMs
• Anatomy: Cervical 46%; Thoraco-lumbar 44%
• Onset
  • 75% < 40 years
  • Hemorrhage: Common; Aneurysms in 20%
• Prognosis: Progressive myelopathy in 60%
• Treatment: Surgery

• Sporadic or von Hippel-Lindau
• Onset: Average 35 years

• 5% to 12% of Spinal AVMs
• Onset: Hemorrhage 0.25% to 0.8% per year
• MRI: Mixed signal T1 & T2; Hemosiderin

• Associated with
  • Renal failure
  • Dialysis for > 72 months
• ? Related to
  • Rheumatoid arthritis
  • Amyloidosis: Dialysis-associated
• Clinical
  • Location: Usually cervical
  • Onset: Neck & shoulder pain
  • Progression: Myelopathy within 2 years
• Pathology: Disk-space narrowing; Vertebral subluxation

• Early or mild symptoms (Type I disease)
  • Joint pain
  • Skin: Rash or Marbling
  • Edema: Localized
• More severe (Type II disease)
  • Myelopathy
    • Onset: 10 to 30 minutes after surfacing; Malaise
    • Progression: Paresthesias & Paralysis; Lower > Upper Extremity
    • May be partial Brown-Sequard syndrome
    • Prognosis: Recovery over weeks in 60% to 80%
    • Pathophysiology: ? Congestion of spinal epidural veins

• Associations
  • Initial manifestation of malignancy (20%)
    • Lung (Adenocarcinoma); Unknown primary; Multiple myeloma; Non-Hodgkin's lymphoma
    • Pediatric: Neuroblastoma; non-Hodgkin's lymphoma
    • Systemic signs suggestive of malignancy (weight loss): Only 20%
  • Established malignancy (80%)
    • Breast; Prostate
    • Less common: Renal; Liver; Colorectal; Sarcoma; Thyroid; Myeloma; Lymphoma
• Presenting neurologic feature
  • Back pain (20%)
    • > 2 weeks
    • Worse with recumbency
    • Thoracic
  • Radiculopathy (40%)
  • Myelopathy (40%)
• Duration of symptoms before diagnosis: 1 to 4 months
• Diagnosis
  • Needle biopsy of vertebral lesion ± diagnostic laminectomy
  • Chest & Abdominal CT
  • Marrow biopsy; Serum immunofixation
• Survival: Mean 6.5 months
• Treatment: Depends on tumor type
  • Radiation
  • Vertebral corpectomy: For spinal instability; high complication rate

• Epidemiology
  • Associated with: Obesity (80%); Corticosteroids; Cushing's
  • Male (80%) > Female
  • Age: 14 to 75 years
• Level: Thoracic (earlier age) & Lumbar spine
• Onset: Pain (Low back & radiating; 80%) & Weakness (64%)
• Weakness (65%): Legs; Symmetric or asymmetric
• Sensory loss (50%): Spinal level; Radicular
• Incontinence (16%)
• Progression: Gradual; 1 week to 6 months
• Anatomy of compression: Thoracic - Anterior; Lumbar - Circumferential
• Pathogenesis: Spinal stenosis
  • Direct cord & root compression
  • Vascular insufficiency & venous engorgement
• Treatment: Surgical decompression; ? Weight loss

• Withdraw > 10 mL of CSF
• Process CSF immediately
• Obtain CSF from a site of known leptomeningeal disease
• Repeat procedure at least once if the initial cytology is negative

• Vascular procedure: Most often cross clamping of aorta above L subclavian artery
• Levels
  • Upper: C6 to L1; Usually lower than T8
  • Lesion length: 4 to 11 segments
• Clinical: Incomplete central cord lesion
  • Sensory loss: Pain & temperature; Spared touch & position
  • Motor: Mild leg weakness
  • Bladder: Acontractile (50%)

• Epidemiology
  • Location: French West Indies
  • Female
  • Onset: Mean 31 yrs; Range 17 to 53; 62% < 30
• Clinical
  • Optic neuropathy (88%)
  • Myelopathy
    • Weakness: Paraplegia; Amyotrophy
    • Sensory loss: Cervicothoracic; Suspended; Dissociated pain & temperature
  • Autonomic (50%)
    • Piloerection
    • Sweating: Increased or Decreased
    • Horner's
  • Endocrine
    • Amenorrhea-Galactorrhea: Hyperprolactinemia
    • Hypothyroidism
    • Diabetes insipidus
    • Hyperphagia with obesity
• Lab
  • CSF: Pleocytosis (6 to 200); Protein & IgG increased
  • MRI: Enlarged pituitary; Optic nerve enhancement; Cervical spinal cord cavitation
• Course: Acute or subacute; Recurrent; Duration 2 to 11 years
• Etiology: ? Quinoline neurotoxicity from A. muricata tea

Transverse Myelitis

Transverse Myelitis

Myelitis in ADEM

• Epidemiology
  • Incidence: 1-8 per 10⁶ per year
  • Male = Female
  • Prodrome: Febrile illness 37%; Vaccination 15%
• Causes
  • Immune
    • Lupus
    • Post-Infectious
    • Sjögren
    • Behcet
  • Infections
    • Mycoplasma pneumoniae: Possible association with myelitis ⁹
      • Associated infection
        • Respiratory
        • Timing: ~10 days before
        • Age: Often young adults
      • Age: 8 to 43 years
      • Clinical
        • Paraplegia: Flaccid; May evolve to spasticity
        • Sensory level
        • Level: Thoracic spine, some cervical; May be diffuse
        • Sphincter dysfunction
        • Poliomyelitis syndrome: 1 patient
        • Associated CNS features: Some patients with mental status changes
        • Course
          • Progression: Over 1 to 3 days
          • Recovery: Days to Months; Residual disability in some
      • Laboratory
        • CSF: Cells in 50% (10 to 200)
        • MRI: T2 spinal signal
      • Diagnosis
        • Detection of M. pneumoniae by PCR/culture in CSF and/or throat specimens
        • Positive M. pneumoniae serology: IgM antibodies may persist for 6 months
        • Other: Cold agglutinins in serum
      • Other syndromes
        • Encephalitis (ADEM)
        • Polyneuropathy
        • GBS
        • Optic neuritis
    • Lyme
    • Viral
      • Frequency: 20% to 40%
      • Types
        • Varicella zoster
        • Herpes simplex
        • LCM
        • Enterovirus 71
  • Idiopathic
  • Multiple sclerosis
  • Children: Acute demyelinating encephalomyelitis (ADEM)
  • Rule out: Spinal cord infarction; Lymphoma
• Onset
  • Course: Days; Progression < 4 weeks
  • Symptoms: Leg weakness (40%); Back pain (50%); Paresthesias
  • Age range: 4 to 83 years; Mean 32 to 44 years
• Clinical
  • Spinal cord
    • Isolated defect: Other parts of CNS intact
    • Levels: Most common T2 to T6; Cervical up to C1
  • Sensory loss (96%): Segmental and below lesion
  • GU
    • Bladder dysfunction: 40%
    • Erectile dysfunction: 90%
  • Maximal defect
    • 1 Day: 37%
    • 1 to 10 days: 45%
  • Prognosis
    • Good outcome in 42%: Better prognosis with
      • Retained tendon reflexes
      • Retained posterior column function
    • Worse prognosis
      • Shorter time to maximal defect
      • Spinal shock
      • Severe weakness
      • Denervation on EMG
    • Death: 5%
    • Recurrence: 5% to 15%
    • Development of MS: 7%; ? More common in females
• CSF
  • Cells increased in 33%
  • Protein increased in 50%
• Treatment: IV Corticosteroids, ? benefit in children