Transcript Diseases of the Spinal Cord

Diseases of the
Spinal Cord
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
1.
Determine level of lesion
2.
Identify special anatomic patterns, if any
3.
Determine time frame of the disease
4.
Differentiate compressive from non-
compessive lesions
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 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 lesion
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
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
patterns
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 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 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 patterns
Extramedullary
 Radicular
pain
 Early sacral sensory loss, spastic leg
weakness
Intramedullary
 Sacral
sparing
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 (mass)
 Tumor
 Epidural abscess or
hematoma
 Herniated disc
 Vertebral pathology
Non-compressive
(intrinsic cord lesions)
 Vascular
 Inflammatory
 Infectious
 Tumors
 Extradural
 Intradural
 Intramedullary
 Epidural
abscess
 Epidural
hematoma
Compressive
myelopathies
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
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
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
Present as central cord or hemicord
syndromes of the cervical region
Ependymoma, hemangioblastoma or lowgrade astrocytoma in adults
Secondary metastatic lesions are also
common
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 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 abscess
Treatment
 Decompressive
laminectomy with
debridement with
 Empirical, then culture-guided, antibiotics for
>4weeks
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
 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
“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 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
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
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
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
Poliomyelitis, herpes zoster
Bacterial or mycobacterial myelitis
(abscess)
Schistosomiasis
 Spondylitic
myelopathy
 Vascular
malformations of the
cord and dura
 Syringomyelia
 Subacute combined
degeneration
 Tabes dorsalis
 Familial spastic
paraplegia
Chronic
myelopathies
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
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
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
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 degeneration
Labs:
macrocytic RBCs, low B12
concentration
Therapy: replacement with intramuscular
1000ug vitamin B12 at regular intervals or
subsequent oral treatement
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
 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
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
Thank you.