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Diseases of the Spinal Cord. Disease description. Many conditions constitute neurologic emergencies because of Severity on presentation (quadriplegia, sensory deficits, spinal shock) Potential reversibility

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disease description
Disease description
  • Many conditions constitute neurologic emergencies because of
    • Severity on presentation (quadriplegia, sensory deficits, spinal shock)
    • Potential reversibility
  • Hallmark: presence of horizontally defined level below which motor, sensory and autonomic function is impaired
approach to a patient
Approach to a Patient
  • Determine level of lesion
  • Identify special anatomic patterns, if any
  • Determine time frame of the disease
  • Differentiate compressive from non-compessive lesions
determining level of the lesion
Determining level of the lesion
  • Sensory level
    • Using pinprick or cold stimulus
    • Level indicates damage to the spinothalamic tract 1-2 segments above a unilateral lesion or at the level of the bilateral lesion
determining level of the lesion1
Determining level of the lesion
  • Motor level
    • Damage to the descending corticospinal tract
    • Paraplegia or quadriplegia
    • Upper motor neuron syndrome
  • Autonomic abnormalities
    • Sweating
    • Bladder or bowel dysfunction
determining level of the lesion2
Determining level of the lesion
  • Upper level of the lesion may be determined with segmental signs
    • Hyperalgesia or hyperpathia
    • Fasciculations or muscle atrophy
    • Hypo- or areflexia
special patterns1
Special patterns
  • Brown-Sequard syndrome
    • Ipsilateral weakness (corticospinal), loss of joint and position sense (posterior column)
    • Contralateral loss of pain or temperature sense 1-2 levels below the lesion (spinothalamic)
    • Unilateral segmental signs
special patterns2
Special patterns
  • Central cord syndrome
    • Damge to gray matter and crossing spinothalamic tracts near central canal
    • Arm > leg weakness
    • Loss of pain and temperature sense with intact light touch, joint position and vibration sense (dissociated sensory loss)
special patterns3
Special patterns
  • Anterior spinal artery syndrome
    • Extensive bilateral deficits below the level of the lesion, with intact vibration and position sense
  • Foramen magnum syndrome
    • Damage to decussating pyramidal tract fibers of the legs
    • “Around the clock” pattern of weakness with suboccipital pain
special patterns4
Special patterns
  • Extramedullary
    • Radicular pain
    • Early sacral sensory loss, spastic leg weakness
  • Intramedullary
    • Sacral sparing
time frame of the disease
Time-frame of the disease
  • Acute or severe transverse lesions may initially present as spinal shock (flaccidity, areflexia) in the first few days or weeks, rather than upper motor neuron signs
    • May be mistaken for acute severe polyneuropathy or stratified to have more severe damage than actual
compressive vs noncompressive myelopathy
Compressive vs noncompressive myelopathy

Compressive (mass)

  • Tumor
  • Epidural abscess or hematoma
  • Herniated disc
  • Vertebral pathology

Non-compressive (intrinsic cord lesions)

  • Vascular
  • Inflammatory
  • Infectious
compressive myelopathies

Tumors

    • Extradural
    • Intradural
    • Intramedullary
  • Epidural abscess
  • Epidural hematoma
Compressive myelopathies
neoplastic spinal cord compression epidural neoplasms
Neoplastic spinal cord compression: Epidural neoplasms
  • Mostly metastatic (breast, lung, prostate, kidney, lymphoma, myeloma)
  • Thoracic cord most common except for prostate and ovarian cancers (lumbosacral)
  • Pain is an early symptom, awakens patient at night and is worse with movement
  • MRI is useful
neoplastic spinal cord compression epidural
Neoplastic spinal cord compression: Epidural
  • Therapy
    • Glucocorticoids
    • Local radiotherapy
    • Treatment of underlying tumor
  • Fixed motor deficits of >12 hours do not usually improve and > 48 hours, prognosis is poor
neoplastic spinal cord compression intradural
Neoplastic spinal cord compression: Intradural
  • Mostly slow-growing and benign
  • Meningiomas, neurofibromas, also chordoma, lipoma, dermoid, sarcoma
  • Present with radicular sensory symptoms followed by asymmetric progressive cord syndrome
  • Therapy is surgical resection
neoplastic spinal cord compression intramedullary
Neoplastic spinal cord compression: Intramedullary
  • Present as central cord or hemicord syndromes of the cervical region
  • Ependymoma, hemangioblastoma or low-grade astrocytoma in adults
  • Secondary metastatic lesions are also common
spinal epidural abscess
Spinal epidural abscess
  • Triad:
    • midline dorsal pain, fever, progressive limb weakness
  • Risk factors:
    • impaired immune status, intravenous drug abuse, infections of skin or otehr tissues
  • Causes:
    • Hematogenous spread
    • Direct extension
spinal epidural abscess1
Spinal epidural abscess
  • Causative organsims
    • Staph. Aureus
    • Tuberculosis
    • Gram-neg bacilli, Strep, anaerobes, fungi
  • Lab tests
    • MRI
    • High cervical MRI (to rule out concomittant meningitis)
    • Blood culture
spinal epidural abscess2
Spinal epidural abscess
  • Treatment
    • Decompressive laminectomy with debridement with
    • Empirical, then culture-guided, antibiotics for >4weeks
spinal epidural hematoma
Spinal epidural hematoma
  • Acute focal or radicular pain with variable cord findings
  • Risk factors: anticoagulation, trauma, tumor or blood dyscrasia
  • Labs
    • MRI, CT
  • Therapy is surgical decompression and correction of underlying problem
noncompressive myelopathies

Spinal cord infarction

  • Inflammatory and immune myelitis
    • Systemic inflammatory diseases
      • SLE
    • Demyelinating myelopathy
      • Multiple sclerosis
    • Post-infectious myelitis
    • Acute infectious myelitis
Noncompressive myelopathies
spinal cord infarction
Spinal cord infarction
  • “Watershed” infarcts
    • T3-T4 and boundary zones between anterior and posterior spinal artery territories
    • Rapidly progressive weakness and spasticity with little sensory change
    • Usually associated with hypotension
spinal cord infarction1
Spinal cord infarction
  • Anterior spinal artery infarct
    • Anterior cord syndrome
      • Paraplegia or quadriplegia, dissociated sensory loss, loss of sphincter control
    • Onset may be sudden or progressive over hours
    • Sharp midline back pain
    • Initial spinal shock
systemic inflammatory disorders
Systemic inflammatory disorders
  • Mostly due to SLE, specially with antiphopholipid antibodies
  • CSF may be normal or show mild lymphocytic pleocytosis
  • May respond to high dose steroids and cyclophosphamide
demyelinating myelopathy
Demyelinating myelopathy
  • Multiple sclerosis may present as myelitis
    • Mild swelling and edema of the cord
    • Multifocal areas of abnormal T2 signal on MRI
    • Mild pleocytosis, oligoclonal band
    • Hihg dose steroids, plasma exchange
  • Neuromyelitis optica
    • No oligoclonal bands
    • May respond to anti-CD20 (rituximab)
post infectious or post vaccinal myelitis
Post-infectious or post-vaccinal myelitis
  • Organisms implicated: EBV, CMV, mycoplasma, influenza, measles, varicella, rubeola, mumps
  • Autoimmune disorder triggered by infection and not due to direct infection of spinal cord
  • Treatment glucocorticoids,plasma exchange
acute infectious myelitis
Acute infectious myelitis
  • Poliomyelitis, herpes zoster
  • Bacterial or mycobacterial myelitis (abscess)
  • Schistosomiasis
chronic myelopathies

Spondylitic myelopathy

  • Vascular malformations of the cord and dura
  • Syringomyelia
  • Subacute combined degeneration
  • Tabes dorsalis
  • Familial spastic paraplegia
Chronic myelopathies
spondylitic myelopathy
Spondylitic myelopathy
  • Most common cause of gait problems in the elderly
  • Early neck and shoulder stiffness, later radicular pain
  • Cord compression in <1/3 of patients
    • Spastic paraparesis, paresthesia
    • Reduced vibratory sense, (+) Romberg sgin
    • Dermatomal sensory loss & decreased tendon reflex in the arms, intrinsic hand muscle atrophy
    • Hyperreflexia in the legs
vascular malformation of the cord dura
Vascular malformation of the cord & dura
  • Slowly progressive or intermittent myelopathy with incomplete sensory, motor or bladder disturbance
  • Spinal bruits
  • Labs : high resolution contrast MRI, CT myelogram, selective spinal angiography
  • Therapy: endovascular embolization
syringomyelia
Syringomyelia
  • Developmental cavitary expansion of the cervical cord, often associated with Chiari type I malformation
  • Classic presentation is central cord syndrome (dissociated sensory loss, areflexi weakness of the arms)
  • Symptoms progress as the syrinx expand
subacute combined degeneration
Subacute combined degeneration
  • Vitamin B12 deficiency
  • Subacute paresthesia in hands & feet, loss of vibration and position sense, progressive spastic & ataxic weakness
    • Diffuse, symmetric myelopathy
    • Predominant involvement of posterior and lateral tracts
    • Associated peripheral neuropathy
subacute combined degeneration1
Subacute combined degeneration
  • Labs: macrocytic RBCs, low B12 concentration
  • Therapy: replacement with intramuscular 1000ug vitamin B12 at regular intervals or subsequent oral treatement
tabes dorsalis
Tabes dorsalis
  • Complication of syphilis
  • Fleeting and lancinating pains in the legs, wtih ataxia due to loss of position sense
    • Loss of leg reflexes
    • Impaired vibratory and position sense
    • Romberg’s sign
    • Argyll Robertson pupils
  • Differential: diabetic polyradiculopathy
familial spastic paraplegia
Familial spastic paraplegia
  • Genetic defects of >20 different loci presenting as slowly progressive myelopathy
    • Progressive spasticity and leg weakness
    • Absent or mild sensory symptoms
    • Sphincter disturbances
    • + nystagmus, ataxia, optic atrophy
  • Onset maybe from infancy to middle adulthood
  • Therapy: symptomatic for spasticity
summary
Summary
  • Spinal cord conditions may present catastrophically but many have treatable causes
  • Determine the level of the lesion and identify special patterns in the presentation to derive a diagnosis
  • Address the conditions promptly