1. Diseases of the Spinal Cord

2. 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

3. 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

4. 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

5. 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

6. Determining level of lesion
Manifestations
Cervical
Horner’s syndrome at any level
Upper cervical: quadriplegia, diaphragm weakness
Thoracic
Localized by the sensory level on the trunk or midline back pain, if present
Beevor’s sign (T9-T10 lesion leading to paralysis of lower abdominals, thus upward movement of umbilicus on abdominal contraction)
Lumbar
Lesions at L5-S1 abolish ankle jerk
Sacral/conus medullaris
Bilateral saddle anesthesia with urinary retention, fecal incontinence with lax anal tone, impotence

7. 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

8. Special patterns

9. 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

10. 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)

11. 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

12. Special patterns Extramedullary Intramedullary Radicular pain
Early sacral sensory loss, spastic leg weakness
Intramedullary
Sacral sparing

13. 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

14. Compressive vs noncompressive myelopathy
Compressive (mass)
Tumor
Epidural abscess or hematoma
Herniated disc
Vertebral pathology
Non-compressive (intrinsic cord lesions)
Vascular
Inflammatory
Infectious

15. Compressive myelopathies
Tumors
Extradural
Intradural
Intramedullary
Epidural abscess
Epidural hematoma
Compressive myelopathies

16. 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

17. 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

18. 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

19. 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

20. 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

21. 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

22. Spinal epidural abscess
Treatment
Decompressive laminectomy with debridement with
Empirical, then culture-guided, antibiotics for >4weeks

23. 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

24. 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

25. 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

26. 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

27. 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

28. 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)

29. 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

30. Acute infectious myelitis
Poliomyelitis, herpes zoster
Bacterial or mycobacterial myelitis (abscess)
Schistosomiasis

31. Chronic myelopathies Spondylitic myelopathy
Vascular malformations of the cord and dura
Syringomyelia
Subacute combined degeneration
Tabes dorsalis
Familial spastic paraplegia
Chronic myelopathies

32. 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

33. 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

34. 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

35. 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

36. Subacute combined degeneration
Labs: macrocytic RBCs, low B12 concentration
Therapy: replacement with intramuscular 1000ug vitamin B12 at regular intervals or subsequent oral treatement

37. 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

38. 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

39. 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

40. Thank you.