Diseases of the Spinal Cord

• originates at the medulla and continues

caudally to the conus medullaris at the lumbar
level
• its fibrous extension, the filum terminale,
cauda equinam, terminates at the coccyx
• The adult spinal cord is ∼46 cm long and
enlarged in the cervical and lumbar regions
• white matter tracts containing ascending sensory and
descending motor pathways are located peripherally
• nerve cell bodies are in an inner region of gray matter
• The membranes that cover the spinal cord—the pia,
arachnoid, and dura—are continuous with those of the
brain
• the cerebrospinal fluid is contained within the
subarachnoid space between the pia and arachnoid.
• The spinal cord has 31 segments, each defined by an
exiting ventral motor root and entering dorsal sensory
root.
• first 7 pairs of cervical spinal nerves exit above
the same-numbered vertebral bodies
• all subsequent nerves exit below the same-
numbered vertebral bodies
• the presence of eight cervical spinal cord With
only seven cervical vertebrae
 Major pathwathways pass through Spinal
cord
Corticospinal tract….motor pathway
• Precentral gyrus ( mainly).. mid brain …pons …crossed at
pyramidal decussation…at medulla and spinal cord jn.
• Pass through posteriolateral aspect …., with CTLS distribution,
S being the most lateral one
Spinothalamic tract…fibers of pain and temperature
• unmylienated short fibers
• Enter through dorsal horn, cross to the opposite side at the
same level, then ascends to thalamus
• Located on anteriolateral area
• with CTLS distribution, S being the most lateral one
Large myleinated fibers for vibration and
proprioception
• Enter through dorsal horn then ascends upward
with crossing over the other side at Pyramidal
decussation
• Pass through the posterior column
• with SLTC distribution, C being the most lateral
one
• Determining the Level of the Lesion
• Lesions that transect the descending corticospinal and
other motor tracts cause paraplegia or quadriplegia
• with UMNL syndrome below the affected cord level
• Transverse damage to the cord also produces autonomic
disturbances consisting of absent sweating below the
implicated cord level and bladder, bowel, and sexual
dysfunction
• At the level of the lesion, it involve nerve roots, which is
lower motor lesion so give us lower motor neuron lesion
weakness with flaccid, mm atrophy…)
main features of transverse damage at each level of the
spinal cord are summarized below.
• Cervical Cord Upper cervical cord lesions produce
• quadriplegia and weakness of the diaphragm
• lesions at C5–C6… reflex loss in biceps
• C7…in finger and wrist extensors and triceps
• C8…finger and wrist flexion.
• Horner’s syndrome (miosis, ptosis, and facial
hypohidrosis)
Thoracic Cord
• Lesions here are localized by the sensory level on the
trunk and, if present, by the site of midline back pain
• Lumbar Cord
• Lesions at the L2–L4 spinal cord levels
– paralyze flexion and adduction of the thigh,
– weaken leg extension at the knee
– abolish the patellar reflex.
• Lesions at L5–S1
– paralyze only movements of the foot and ankle,
– flexion at the knee
– extension of the thigh
– abolish the ankle jerks (S1)
• Sacral Cord/Conus Medullaris
• conus syndrome consists of
– bilateral saddle anesthesia (S3–S5),
– Early onset prominent bladder and bowel
dysfunction (urinary retention and incontinence
with lax anal tone)
– impotence.
– The bulbocavernosus (S2–S4) and anal (S4–S5)
reflexes are absent
• cauda equina syndromes
• low back and radicular pain
• asymmetric leg weakness and sensory loss,
• variable areflexia in the lower extremities, and
relative sparing of bowel and bladder
function.
Special Patterns of Spinal Cord Disease
• Brown-Sequard Hemicord Syndrome
– ipsilateral weakness (corticospinal tract)
– loss of joint position and vibratory sense (posterior
column)
– contralateral loss of pain and temperature sense
(spinothalamic tract)
• one or two levels below the lesion.
• Segmental signs, such as radicular pain,
muscle atrophy, or loss of a deep tendon reflex
• Central Cord Syndrome
• selective damage to the
– gray matter nerve cells
– crossing spinothalamic tracts surrounding the central canal.
• produces arm weakness out of proportion to leg weakness
• a “dissociated” sensory loss, meaning loss of pain and
temperature sensations over the shoulders, lower neck, and
upper trunk (cape distribution),
• preservation of light touch, joint position, and vibration sense
• Cause, Spinal truama, syringomyelia, and intrinsic cord tumors
• Anterior Spinal Artery Syndrome
• Infarction of the cord is b/c of occlusion or
diminished flow in this artery.
• All spinal cord functions —motor, sensory, and
autonomic—are lost below the level of the
lesion,
• with retained vibration and position sensation.
• Posterior column syndrome
• Loss of sensation of vibration and
proprioception
• Intact pain and temperature
• No motor weakness
• Cbl/ copper deficiency, HIV myelopathy,
extrinsic compression
• Intramedullary and Extramedullary Syndromes
• intramedullary processes, arising within the
substancen of the cord
• extramedullary ones that lie outside the cord and
compress the spinal cord or its vascular supply.
– extradural Vs intradural masses,
– generally malignant Vs benign (neurofibroma,
Meningiomas being a common cause)
– Shorted Vs long duration of symptoms
extramedullary lesions
• radicular pain is often prominent
• early sacral sensory loss and spastic weakness in
the legs with incontinence
Intramedullary lesions
• poorly localized burning pain rather than
radicular pain
• spare sensation in the perineal and sacral areas
(“sacral sparing”)
 COMPRESSIVE MYELOPATHIES

• 1, Neoplastic Spinal Cord Compression

• most neoplasms are epidural in origin, resulting from
metastases to the adjacent vertebral column
– breast,
– lung,
– prostate,
– kidney,
– Lymphoma
– myeloma being particularly frequent
• thoracic spinal column is most commonly involved
• aching and localized or sharp and radiating
• Pain is usually the initial symptom of spinal
metastasis
• spine X ray and radionuclide bone scans do not
identify 15–20% of met
• MRI provides excellent anatomic resolution
• Infections of the spinal column (e.g,osteomyelitis )
unlike tumor, they often cross the disk space to
involve the adjacent vertebral body
• If spinal cord compression is suspected,
imaging should be obtained promptly…
nuerologic emergency
• radicular symptoms but no evidence of
myelopathy, it may be safe to defer imaging
for 24–48 hr
• Primary intramedullary tumors of the spinal
cord are uncommon.
• 2, Spinal Epidural Abscess
• Spinal epidural abscess presents with
• midline back or neck pain, fever, and progressive limb
weakness.
• Prompt recognition of this distinctive process may
prevent permanent sequelae.
• The duration of pain prior to presentation is generally
≤2 weeks
• elevated white blood cell count, sedimentation rate
and C-reactive protein
• Risk factors include an impaired immune status
(HIV, DM, renal failure, alcoholism, malignancy),
• intravenous drug abuse, and infections of the
skin or other tissues.
• Two-thirds of epidural infections result from
hematogenous spread of bacteria from
– the skin (furunculosis),
– soft tissue (pharyngeal or dental abscesses; sinusitis),
– deep viscera (bacterial endocarditis).
• The remainder arises from direct extension of a local infection
to the subdural (osteomyelitis, decubitus ulcers, LP, epidural
anesthesia, or spinal surgery)
Most cases are due to
• Staphylococcus aureus;
• gram-negative bacilli,
• Streptococcus, anaerobes
• fungi
• Methicillin-resistant Staphylococcus aureus (MRSA) is an
important consideration
• Tuberculosis from an adjacent vertebral source (Pott’s disease)
• Blood cultures are positive in >1/2 cases, but
direct aspiration of the abscess at surgery is
often required.
• Lumbar puncture is only required if
encephalopathy or meningitis suspected a
feature that is found in <25% of cases.
• The level of the puncture should be planned
to minimize the risk of Meningitis, high
cervical tap is may be safest approach
• Spinal Epidural Hematoma
• Hemorrhage into the epidural (or subdural)
space causes acute focal or radicular pain
followed by variable signs of a spinal cord or
conus medullaris disorder.
• Therapeutic anticoagulation, trauma, tumor,
or blood dyscrasias are predisposing
conditions
• NONCOMPRESSIVE MYELOPATHIES
– Vascular
– ischemic
– Inflammatory
– infectious causes
• Ischemia is likely in hyperacute presentations with back
or neck pain , with anterior cord syndrome
• inflammation is more likely in cases that develop
subacutely, or when systemic symptoms, CSF
oligoclonal bands or multiple discrete spinal cord MRI
lesions are present.
• The most frequent inflammatory causes of
acute myelopathy are
– multiple sclerosis (MS);
– neuromyelitis optica (NMO);
– sarcoidosis;
– systemic inflammatory diseases such as SLE and
Behcet’s disease
– postinfectious or idiopathic transverse myelitis
• Spinal Cord Infarction The cord is supplied by
three arteries that
• a single anterior spinal artery and paired
posterior spinal arteries.
• The anterior spinal artery is branches of the
vertebral arteries
• Acute infarction in the territory of the anterior
spinal artery produces paraplegia or
quadriplegia
• Present with anterior cord syndrome
• typically is progressive over minutes or a few
hours, may be sudden
• Sharp midline or radiating back pain localized
to the area of ischemia is frequent.
• Areflexia due to spinal shock is often present
initially
Causes of spinal cord infarction include
• aortic atherosclerosis,
• dissecting aortic aneurysm,
• vertebral artery occlusion or dissection in the neck,
• aortic surgery, or profound hypotension from any
cause
• Cardiogenic emboli, vasculitis and collagen vascular
disease (particularly SLE, Sjögren’s syndrome, the
antiphospholipid antibody syndrome)
• Inflammatory and Immune Myelopathies
(Myelitis) includes the demyelinating
conditions
• MS, NMO,
• postinfectious myelitis,
• sarcoidosis, systemic autoimmune disease
• Transverse myelitis
• MULTIPLE SCLEROSIS
• present with acute myelitis
• most common causes of a partial cord
syndrome.
• MRI findings in MS-associated myelitis
typically consist of
– mild swelling of the cord and diffuse or multifocal
“shoddy” areas of abnormal signal MRI.
• NEUROMYELITIS OPTICA, NMO
• is an immune-mediated demyelinating disorder
• severe myelopathy that is typically longitudinally
extensive, meaning that the lesion spans three or
more vertebral segments.
• NMO is associated with optic neuritis that is often
bilateral and may precede or follow myelitis by
weeks or months.
• CSF studies reveal pleocytosis (higher than in
typical MS) with polymorphonuclear
predominant pattern
• Diagnostic serum autoantibodies against the
water channel protein aquaporin-4 (AQP-4)
are present in 90% of patients with NMO
• POSTINFECTIOUS MYELITIS
• follow an infection or vaccination.
• Numerous organisms have been implicated, including
Epstein-Barr virus (EBV), cytomegalovirus (CMV),
mycoplasma, influenza, measles, varicella, mumps, and
yellow fever
• an infectious agent cannot be isolated from the nervous
system or CSF.
• presumption is that the myelitis represents an
autoimmune disorder triggered by infection and is not due
to direct infection of the spinal cord.
• ACUTE INFECTIOUS MYELITIS
• Herpes zoster is the best characterized viral myelitis,
but
• HSV types 1 and 2, EBV, CMV, and rabies virus are well
described Causes
• Poliomyelitis is the prototypic viral myelitis, but it is
more or less restricted to the anterior gray matter of
the cord containing the spinal motoneurons
• Bacterial and mycobacterial myelitis (most are
essentially abscesses) are less common than viral
• Schistosomiasis is an important cause of
parasitic myelitis in endemic areas.
• The process is intensely inflammatory and
granulomatous, caused by a local response to
tissue-digesting enzymes from the ova of the
parasite
• High-Voltage Electrical Injury
• Spinal cord injuries are prominent following
electrocution from lightning strikes or other
accidental electrical exposures.
• The syndrome consists of
– transient weakness acutely (often with an altered
sensorium and focal cerebral disturbances),
– sometimes lasted several days or even weeks
– myelopathy could be permanant
• CHRONIC MYELOPATHIES
• Neck and shoulder pain with stiffness are early
symptoms in cervical radiculopathy
• Radiating leg pain and numbness in case of
lumbosacral
• impingement of bone and soft tissue overgrowth on
nerve roots results in radicular arm pain, most often
in a C5 or C6 distribution
• progression of medically treated myelopathy is high,
estimated at 8% over 1 year
• SYRINGOMYELIA
• Syringomyelia is a developmental cavity in the cervical
cord that may enlarge and produce progressive
myelopathy or may remain Asymptomatic
• The presentation is a central cord syndrome
• More than half of all cases are associated with Chiari
type 1 malformations
• MRI accurately identifies developmental and acquired
syrinx cavities and their associated spinal cord
enlargement
• SUBACUTE COMBINED DEGENERATION (VITAMIN B12
• DEFICIENCY)
• treatable myelopathy
• presents with
– subacute paresthesias in the hands and feet,
– loss of vibration and position sensation
– a progressive spastic and ataxic weakness
– Romberg sign.
– Optic atrophy and irritability or
– other cognitive changes may be prominent in advanced cases
– myelopathy tends to be diffuse rather than focal
• signs are generally symmetric and reflect
predominant involvement of the posterior and
lateral tracts
• The diagnosis is confirmed by the finding of
macrocytic red blood cells, a low serum B12
concentration
• elevated serum levels of homocysteine and
methylmalonic acid
• HYPOCUPRIC MYELOPATHY
• similar to subacute combined degeneration
except normal B12 levels
• low level of serum copper and serum
ceruloplasmin.
• bariatric surgery with impaired copper
absorption
• excess zinc from health food supplements
• TABES DORSALIS
• The classic syphilitic syndromes of tabes dorsalis are now less
Frequent
• The characteristic symptoms
– repetitive lancinating pains, primarily in the legs
– Ataxia of the legs and gait due to loss of position sense
– Paresthesias,
– bladder disturbances
– cardinal signs of tabes are loss of reflexes in the legs
– impaired position and vibratory sense
– Romberg sign
• in almost all cases, bilateral Argyll Robertson pupils, which fail to
constrict to light but accommodate
• HEREDITARY SPASTIC PARAPLEGIA
• Many cases of slowly progressive myelopathy are genetic in
origin
• Most present with progressive spasticity and weakness in
the legs
• usually symmetrical.
• Sensory symptoms and signs are absent or mild
• but sphincter disturbances may be present
• The onset may be as early as the first year of life or as late as
middle adulthood.
• Only symptomatic therapies are available.
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