Diseases of the Spinal

Cord
• Diseases of the spinal cord are frequently
devastating.
• They produce quadriplegia, paraplegia, and
sensory deficits far beyond the damage they
would inflict elsewhere in the nervous system
because the spinal cord contains, in a small cross-
sectional area, almost the entire motor output
and sensory input of the trunk and limbs.
• Many spinal cord diseases are reversible if
recognized and treated at an early stage; thus,
they are among the most critical of neurologic
emergencies.
 APPROACH TO THE PATIENT

• The spinal cord is a thin, tubular extension of the

central nervous system contained within the bony
spinal canal.
• It originates at the medulla and continues
caudally to the conus medullaris at the lumbar
level; its fibrous extension, the filum terminale,
terminates at the coccyx.
• The adult spinal cord is ~46 cm (18 in.) long, oval
in shape, and enlarged in the cervical and lumbar
regions, where neurons that innervate the upper
and lower extremities, respectively, are located.
• The white matter tracts containing ascending
sensory and descending motor pathways are
located peripherally, whereas nerve cell bodies
are clustered in an inner region of gray matter
shaped like a four-leaf clover that surrounds the
central canal (anatomically an extension of the
fourth ventricle).
• The membranes that cover the spinal cord—the
pia, arachnoid, and dura—are continuous with
those of the brain, and 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.
• During embryologic development, growth of the
cord lags behind that of the vertebral column,
and the mature spinal cord ends at approximately
the first lumbar vertebral body.
• The lower spinal nerves take an increasingly
downward course to exit via intervertebral
foramina.
• The first seven pairs of cervical spinal nerves exit
above the same-numbered vertebral bodies,
whereas all the subsequent nerves exit below the
same-numbered vertebral bodies because of the
presence of eight cervical spinal cord segments
but only seven cervical vertebrae.
• These relationships assume particular importance
for localization of lesions that cause spinal cord
compression.
• Sensory loss below the circumferential level of
the umbilicus, for example, corresponds to the
T10 cord segment but indicates involvement of
the cord adjacent to the seventh or eighth
thoracic vertebral body
• Determining the Level of the Lesion
• The presence of a horizontally defined level below
which sensory, motor, and autonomic function is
impaired is a hallmark of a lesion of the spinal
cord
• This sensory level is sought by asking the
patient to identify a pinprick or cold stimulus
applied to the proximal legs and lower trunk and
successively moved up toward the neck on each
side.
• Sensory loss below this level is the result of
damage to the spinothalamic tract on the
opposite side, one to two segments higher in the
case of a unilateral spinal cord lesion, and at the
level of a bilateral lesion.
• The discrepancy in the level of a unilateral lesion
is the result of the course of the second-order
sensory fibers, which originate in the dorsal horn,
and ascend for one or two levels as they cross
anterior to the central canal to join the opposite
spinothalamic tract
• Lesions that transect the descending corticospinal
and other motor tracts cause paraplegia or
quadriplegia with heightened deep tendon
reflexes, Babinski signs, and eventual spasticity
(the upper motor neuron syndrome).
• Transverse damage to the cord also produces
autonomic disturbances consisting of absent
sweating below the implicated cord level and
bladder, bowel, and sexual dysfunction.
• The uppermost level of a spinal cord lesion can
also be localized by attention to the segmental
signs corresponding to disturbed motor or
sensory innervation by an individual cord
segment.
• A band of altered sensation (hyperalgesia or
hyperpathia) at the upper end of the sensory
disturbance, fasciculations or atrophy in muscles
innervated by one or several segments, or a
muted or absent deep tendon reflex may be
noted at this level.
• These signs also can occur with focal root or
peripheral nerve disorders; thus, they are most
useful when they occur together with signs of
long tract damage.
• With severe and acute transverse lesions, the
limbs initially may be flaccid rather than spastic.
• This state of “spinal shock” lasts for several days,
rarely for weeks, and may be mistaken for
extensive damage to the anterior horn cells over
many segments of the cord or for an acute
polyneuropathy.
 Cervical Cord
• Upper cervical cord lesions produce quadriplegia
and weakness of the diaphragm.
• The uppermost level of weakness and reflex loss
with lesions
– At C5-C6 is in the biceps;
– At C7, in finger and wrist extensors and triceps; and
– At C8, finger, and wrist flexion.
• Horner’s syndrome (miosis, ptosis, and facial
hypohidrosis) may accompany a cervical cord
lesion at any level.
 Thoracic Cord
• Lesions here are localized by the sensory level on
the trunk and, if present, by the site of midline
back pain.
• Useful markers of the sensory level on the trunk
are the nipples (T4) and umbilicus (T10).
• Leg weakness and disturbances of bladder and
bowel function accompany the paralysis.
• Lesions at T9-T10 paralyze the lower—but not the
upper—abdominal muscles, resulting in upward
movement of the umbilicus when the abdominal
wall contracts (Beevor’s sign).
 Lumbar Cord
• Lesions at the L2-L4 spinal cord levels paralyze
flexion and adduction of the thigh, weaken leg
extension at the knee, and abolish the patellar
reflex.
• Lesions at L5-S1 paralyze only movements of the
foot and ankle, flexion at the knee, and extension
of the thigh, and abolish the ankle jerks (S1).
 Sacral Cord/Conus Medullaris
• The conus medullaris is the tapered caudal
termination of the spinal cord, comprising the
sacral and single coccygeal segments.
• The distinctive conus syndrome consists of
bilateral saddle anesthesia (S3-S5), prominent
bladder and bowel dysfunction (urinary retention
and incontinence with lax anal tone), and
impotence.
• The bulbocavernosus (S2-S4) and anal (S4-S5)
reflexes are absent. Muscle strength is largely
preserved.
• By contrast, lesions of the cauda equina, the
nerve roots derived from the lower cord, are
characterized by 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
• Mass lesions in the lower spinal canal often
produce a mixed clinical picture with elements of
both cauda equina and conus medullaris
syndromes
Special Patterns of Spinal Cord Disease

• Most fiber tracts—including the posterior columns

and the spinocerebellar and pyramidal tracts—are
situated on the side of the body they innervate.
• However, afferent fibers mediating pain and
temperature sensation ascend in the
spinothalamic tract contralateral to the side they
supply.
 Brown-Sequard Hemicord Syndrome

• This consists of ipsilateral weakness (corticospinal

tract) and loss of joint position and vibratory
sense (posterior column), with contralateral loss
of pain and temperature sense (spinothalamic
tract) one or two levels below the lesion.
 Central Cord Syndrome

• This syndrome results from selective damage to

the gray matter nerve cells and crossing
spinothalamic tracts surrounding the central
canal.
• In the cervical cord, the central cord syndrome
produces arm weakness out of proportion to leg
weakness and a “dissociated” sensory loss,
meaning loss of pain and temperature sensations
over the shoulders, lower neck, and upper trunk
(cape distribution), in contrast to preservation
of light touch, joint position, and vibration sense
in these regions.
• Spinal trauma,syringomyelia, and intrinsic cord
tumors are the main causes.
 Anterior Spinal Artery Syndrome

• All spinal cord functions—motor, sensory, and

autonomic—are lost below the level of the lesion,
with the striking exception of retained vibration
and position sensation.
 Foramen Magnum Syndrome

• Lesions in this area interrupt decussating

pyramidal tract fibers destined for the legs, which
cross caudal to those of the arms, resulting in
weakness of the legs (crural paresis).
• Compressive lesions near the foramen magnum
may produce weakness of the ipsilateral shoulder
and arm followed by weakness of the ipsilateral
leg, then the contralateral leg, and finally the
contralateral arm, an “around the clock” pattern
that may begin in any of the four limbs.
• There is typically suboccipital pain spreading to
the neck and shoulders.
 Intramedullary and Extramedullary
Syndromes
• The differentiating features are only relative and
serve as clinical guides.
• With extramedullary lesions, radicular pain is
often prominent, and there is early sacral sensory
loss and spastic weakness in the legs with
incontinence due to the superficial location of the
corresponding sensory and motor fibers in the
spinothalamic and corticospinal tracts
• Intramedullary lesions tend to produce poorly
localized burning pain rather than radicular pain
and to spare sensation in the perineal and sacral
areas (“sacral sparing”), reflecting the laminated
configuration of the spinothalamic tract with
sacral fibers outermost; corticospinal tract signs
appear later
 DISTINGUISHING COMPRESSIVE FROM
NONCOMPRESSIVE MYELOPATHY

• The first priority is to exclude treatable

compression of the cord by a mass lesion. The
common causes are tumor, epidural abscess or
hematoma, herniated disk, and spondylitic
vertebral pathology.
• Epidural compression due to malignancy or
abscess often causes warning signs of neck or
back pain, bladder disturbances, and sensory
symptoms that precede the development of
paralysis.
• Spinal subluxation, hemorrhage, and
noncompressive etiologies such as infarction are
more likely to produce myelopathy without
antecedent symptoms.
• Magnetic resonance imaging (MRI) with
gadolinium, centered on the clinically suspected
level, is the initial diagnostic procedure if it is
available; it is often appropriate to image the
entire spine (cervical through sacral regions) to
search for additional clinically silent lesions.
 COMPRESSIVE MYELOPATHIES

• Neoplastic Spinal Cord Compression

• In adults, most neoplasms are epidural in origin,
resulting from metastases to the adjacent
vertebral column.
• The propensity of solid tumors to metastasize to
the vertebral column probably reflects the high
proportion of bone marrow located in the axial
skeleton.
• Almost any malignant tumor can metastasize to
the spinal column, with breast, lung, prostate,
kidney, lymphoma, and myeloma being
particularly frequent.
• The thoracic spinal column is most commonly
involved; exceptions are metastases from
prostate and ovarian cancer, which occur
disproportionately in the sacral and lumbar
vertebrae, probably from spread through Batson’s
plexus, a network of veins along the anterior
epidural space.
• Pain is usually the initial symptom of spinal
metastasis; it may be aching and localized or
sharp and radiating in quality and typically
worsens with movement, coughing, or sneezing
and characteristically awakens patients at night.
• MRI provides excellent anatomic resolution of the
extent of spinal tumors and is able to distinguish
between malignant lesions and other masses—
– Epidural abscess,
– Tuberculoma,
– Lipoma, or
– Epidural hemorrhage,
• among others—that present in a similar fashion.
• Infections of the spinal column (osteomyelitis and
related disorders) are distinctive in that, 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.
• Up to 40% of patients who present with cord
compression at one level are found to have
asymptomatic epidural metastases elsewhere;
thus, imaging of the entire length of the spine is
important to define the extent of disease.
• TREATMENT
• Management of cord compression includes
– Glucocorticoids to reduce cord edema,
– Local radiotherapy (initiated as early as possible) to the
symptomatic lesion, and
– Specific therapy for the underlyingtumor type
• In one trial, initial management with surgery
followed by radiotherapy was more effective than
radiotherapy alone for patients with a single area
of spinal cord compression by extradural tumor;
however, patients with recurrent cord
compression, brain metastases, radiosensitive
tumors, or severe motor symptoms of >48 h in
duration were excluded from this study.
• Radiotherapy alone may be effective even for
some typically radioresistant metastases.
• A good response to therapy can be expected in
individuals who are ambulatory at presentation.
• Treatment usually prevents new weakness, and
some recovery of motor function occurs in up to
one-third of patients.
• Motor deficits (paraplegia or quadriplegia), once
established for >12 h, do not usually improve,
and beyond 48 h the prognosis for substantial
motor recovery is poor.
• In contrast to tumors of the epidural space, most
intradural mass lesions are slow-growing and
benign.
• Meningiomas and neurofibromas account for most
of these, with occasional cases caused by
chordoma, lipoma, dermoid, or sarcoma.
• Meningiomas are often located posterior to the
thoracic cord or near the foramen magnum,
although they can arise from the meninges
anywhere along the spinal canal
• Neurofibromas are benign tumors of the nerve
sheath that typically arise from the posterior root;
when multiple, neurofibromatosis is the likely
etiology.
• Symptoms usually begin with radicular sensory
symptoms followed by an asymmetric,
progressive spinal cord syndrome.
• Therapy is surgical resection.
• Primary intramedullary tumors of the spinal cord
are uncommon.
• 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 but may on occasion be
several months or longer
• Fever is typically but not invariably present,
accompanied by elevated white blood cell count,
sedimentation rate, and C-reactive protein.
• As the abscess expands, further spinal cord
damage results from venous congestion and
thrombosis
• Once weakness and other signs of myelopathy
appear, progression may be rapid and
irreversible.
• Risk factors include an impaired immune status
(HIV, diabetes mellitus, renal failure, alcoholism,
malignancy), intravenous drug abuse, and
infections of the skin or other tissues.
• 2/3 of epidural infections result from
hematogenous spread of bacteria from the skin
(furunculosis), soft tissue (pharyngeal or dental
abscesses; sinusitis), or deep viscera (bacterial
endocarditis).
• Most cases are due to Staphylococcus aureus;
gram-negative bacilli, Streptococcus, anaerobes,
and fungi can also cause epidural abscesses.
• MRSA is an important consideration, and therapy
should be tailored to this possibility.
• Pott’s disease remains an important cause in the
developing world.
• MRI localizes the abscess and excludes other
causes of myelopathy. Blood cultures are positive
in more than half of cases
• Direct aspiration of the abscess at surgery is
often required for a microbiologic diagnosis
• TREATMENT
• Treatment is by decompressive laminectomy with
debridement combined with long-term antibiotic
treatment.
• Surgical evacuation prevents development of
paralysis and may improve or reverse paralysis in
evolution, but it is unlikely to improve deficits of
more than several days in duration.
• Broad-spectrum antibiotics (typically
– Vancomycin 15–20 mg/kg q12h (staphylococcus
including MRSA, streptococcus),
– Ceftriaxone 2 gm q24h (gram-negative bacilli), and
– when indicated Metronidazole 30 mg/kg/d divided into
q6h intervals (anaerobes)
should be started empirically before surgery for 6–
8 weeks.
• Surgical management remains the treatment of
choice unless the abscess is limited in size and
causes few or no neurologic signs.
• If surgery is contraindicated or if there is a fixed
paraplegia or quadriplegia that is unlikely to
improve following surgery, long-term
administration o systemic and oral antibiotics can
be used.
• With prompt diagnosis and treatment of spinal
epidural abscess, up to two-thirds of patients
experience significant recovery.
• 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.
• Treatment consists of prompt reversal of any
underlying clotting disorder and surgical
decompression.
• Hematomyelia
• Hemorrhage into the substance of the spinal cord
is a rare result of
– Trauma,
– Intraparenchymal vascular malformation,
– Vasculitis due to polyarteritis nodosa or SLE,
– Bleeding disorders, or
– A spinal cord neoplasm.
• Therapy is supportive, and surgical intervention is
generally not useful.
• An exception is hematomyelia due to an
underlying vascular malformation
NONCOMPRESSIVE MYELOPATHIES

• The most frequent causes of noncompressive

acute transverse myelopathy are
– Spinal cord infarction;
– Systemic inflammatory disorders, including SLE and
sarcoidosis;
– Demyelinating diseases, including MS; NMO;
– Postinfectious or idiopathic transverse myelitis, which is
presumed to be an immune condition related to acute
disseminated encephalomyelitis; and
– Infectious (primarily viral) causes.
 Spinal Cord Infarction

• Acute infarction in the territory of the anterior

spinal artery produces paraplegia or quadriplegia,
dissociated sensory loss affecting pain and
temperature sense but sparing vibration and
position sense, and loss of sphincter control
(“anterior cord syndrome”).
• Areflexia due to spinal shock is often present
initially; with time, hyperreflexia and spasticity
appear.
• Less common is infarction in the territory of the
posterior spinal arteries, resulting in loss of
posterior column function either on one side or
bilaterally.
• 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.
• A surfer’s myelopathy usually in the thoracic
region, has been associated with prolonged back
extension due to lifting the upper body off the
board while waiting for waves.
• MRI may fail to demonstrate infarctions of the
cord, especially in the first day, but often the
imaging becomes abnormal at the affected level.
• In cord infarction due to presumed
thromboembolism, acute anticoagulation is not
indicated, with the possible exception of the
unusual transient ischemic attack or incomplete
infarction with a stuttering or progressive course.
• Increasing systemic BP to a mean arterial
pressure of >90 mmHg, or lumbar drainage of
spinal fluid, was reportedly helpful in a few
published cases of cord infarction, but neither of
these approaches has been studied
systematically.
 Inflammatory and Immune
Myelopathies (Myelitis)
• MULTIPLE SCLEROSIS
• MS may present with acute myelitis
• In Caucasians, MS attacks rarely cause a
transverse myelopathy (i.e., attacks of bilateral
sensory disturbances, unilateral or bilateral
weakness, and bladder or bowel symptoms), but
it is among the 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 on
T2W sequences.
• Contrast enhancement, indicating disruption in
the blood-brain barrier associated with
inflammation, is present in many acute cases.
• Treatment of acute episodes of MS-associated
myelitis consists of intravenous
methylprednisolone (500 mg qd for 3 days)
followed by oral prednisone (1 mg/kg/d for
several weeks, then gradual taper).
• NEUROMYELITIS OPTICA
• Immune-mediated demyelinating disorder
consisting of a severe myelopathy that is typically
longitudinally extensive ( 3 or mor segments
• NMO is associated with optic neuritis that is often
bilateral , and also by brainstem and, in some
cases, hypothalamic or focal cerebral white
matter involvement
• Diagnostic serum autoantibodies against the
water channel protein aquaporin-4 are present in
60–70% of patients with NMO, and
• less commonly autoantibodies against the CNS
myelin protein myelin oligodendrocyte
glycoprotein (MOG) are found.
• Unlike MS, oligoclonal bands are generally
absent.
• NMO has also been associated with SLE as well
as with other systemic autoimmune diseases;
rare cases are paraneoplastic in origin.
• POSTINFECTIOUS MYELITIS
• Many cases of myelitis, termed postinfectious or
postvaccinal, follow an infection or vaccination
• Numerous organisms have been implicated,
including EBV, CMV, mycoplasma, influenza,
measles, varicella, mumps, and yellow fever.
• As in the related disorder acute disseminated
encephalomyelitis , postinfectious myelitis often
begins as the patient appears to be recovering
from an acute febrile infection, or in the
subsequent days or weeks, but an infectious
agent cannot be isolated from the nervous
system or CSF.
• The presumption is that the myelitis represents
an autoimmune disorder triggered by infection
and is not due to direct infection of the spinal
cord.
• Treatment is usually with glucocorticoids or, in
fulminant cases, plasma exchange.
• No definitive trials of therapy for NMO
• Recommended treatment of acute relapses is
with glucocorticoids and, for refractory cases,
plasma exchange
• Prophylactic treatment with azathioprine,
mycophenylate, or rituximab may protect against
subsequent relapses
• ACUTE INFECTIOUS MYELITIS
• Many viruses have been associated with an acute
myelitis that is infectious in nature rather than
postinfectious.
• Nonetheless, the two processes are often difficult
to distinguish.
• Herpes zoster is the best characterized viral
myelitis, but HSV types 1 and 2, EBV, CMV, and
rabies virus are other well-described causes and
Zika virus has also been recognized as a cause of
infectious myelitis.
• HSV-2 (and less commonly HSV-1) produces a
distinctive syndrome of recurrent sacral cauda
equina neuritis in association with outbreaks of
genital herpes (Elsberg’s syndrome)
• 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
causes and much less frequent than cerebral
bacterial abscess.
• Almost any pathogenic species may be
responsible, including Lyme disease, Listeria
monocytogenes, TB, and syphilis).
• 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, typically Schistosoma hematobium or
Schistosoma mansoni.
• Toxoplasmosis can occasionally cause a focal
myelopathy, and this diagnosis should especially
be considered in patients with AIDS .
• Cysticercosis is another consideration, although
myelitis from this helminth is far less common
than parenchymal brain or meningeal
involvement.
• In cases of suspected viral myelitis, it may be
appropriate to begin specific therapy pending
laboratory confirmation.
– Herpes zoster, HSV, and EBV myelitis are treated with
intravenous acyclovir (10 mg/kg q8h) or oral valacyclovir
(2 g tid) for 10–14 days;
– CMV is treated with ganciclovir (5 mg/kg IV bid) plus
foscarnet (60 mg/kg IV tid) or cidofovir (5 mg/kg per
week for 2 weeks).
 CHRONIC MYELOPATHIES

• SPONDYLOTIC MYELOPATHY
• Spondylotic myelopathy is one of the most
common causes of chronic cord compression and
of gait difficulty in the elderly.
• Neck and shoulder pain with stiffness are early
symptoms; impingement of bone and soft tissue
overgrowth on nerve roots results in radicular arm
pain, most often in a C5 or C6 distribution.
• Compression of the cervical cord produces a
slowly progressive spastic paraparesis, at times
asymmetric and often accompanied by
paresthesias in the feet and hands
• Vibratory sense is diminished in the legs, there is
a Romberg sign, and occasionally there is a
sensory level for vibration or pinprick on the
upper thorax.
• Dermatomal sensory loss in the arms, atrophy of
intrinsic hand muscles, increased deep-tendon
reflexes in the legs, and extensor plantar
responses are common.
• Diagnosis is usually made by MRI and may be
suspected from CT images; plain x-rays are less
helpful.
• Definitive therapy consists of surgical
decompression, either posterior laminectomy or
an anterior approach with resection of the
protruded disk and bony material
• RETROVIRUS-ASSOCIATED MYELOPATHIES
• The myelopathy associated with HTLV-1, formerly
called tropical spastic paraparesis, is a slowly
progressive spastic syndrome with variable
sensory and bladder disturbance.
• Approximately half of patients have mild back or
leg pain.
• The neurologic signs may be asymmetric, often
lacking a well-defined sensory level; the only sign
in the arms may be hyperreflexia after several
years of illness.
• Diagnosis is made by demonstration of HTLV-1-
specific antibody in serum by ELISA, confirmed by
radioimmunoprecipitation or Western blot
analysis.
• Measuring proviral DNA by PCR in serum and CSF
cells can be useful as an ancillary part of
diagnosis.
• There is no proven effective treatment. Based on
limited evidence, the use of chronic low dose oral
glucocorticoids can be tried.
• Antiviral treatment is ineffective.
• Symptomatic therapy for spasticity and bladder
symptoms may be helpful.
 SYRINGOMYELIA

• Syringomyelia is a developmental cavity of the

cervical cord that may enlarge and produce
progressive myelopathy or may remain
asymptomatic
• More than half of all cases are associated with
Chiari type 1 malformations in which the
cerebellar tonsils protrude through the foramen
magnum and into the cervical spinal canal.
• Acquired cavitations of the cord in areas of
necrosis are also termed syrinx cavities; these
follow trauma, myelitis, necrotic spinal cord
tumors, and chronic arachnoiditis due to
tuberculosis and other etiologies.
• The presentation is a central cord syndrome
consisting of a regional dissociated sensory loss
(loss of pain and temperature sensation with
sparing of touch and vibration) and areflexic
weakness in the upper limbs.
• The sensory deficit has a distribution that is
“suspended” over the nape of the neck,
shoulders, and upper arms (cape distribution) or
in the hands.
• Muscle wasting in the lower neck, shoulders,
arms, and hands with asymmetric or absent
reflexes in the arms reflects expansion of the
cavity in the gray matter of the cord
• As the cavity enlarges and compresses the long
tracts, spasticity and weakness of the legs,
bladder and bowel dysfunction, and a Horner’s
syndrome appear.
• Extension of the syrinx into the medulla,
syringobulbia, causes palatal or vocal cord
paralysis, dysarthria, horizontal or vertical
nystagmus, episodic dizziness or vertigo, and
tongue weakness with atrophy.
• MRI accurately identifies developmental and
acquired syrinx cavities and their associated
spinal cord enlargement
• Treatment of syringomyelia is generally
unsatisfactory.
• The Chiari tonsillar herniation may be
decompressed, generally by suboccipital
craniectomy, upper cervical laminectomy, and
placement of a dural graft.
 CHRONIC MYELOPATHY OF MULTIPLE
SCLEROSIS

• A chronic progressive myelopathy is the most

frequent cause of disability in both primary
progressive and secondary progressive forms of
MS.
• Involvement is typically bilateral but asymmetric
and produces motor, sensory, and bladder/bowel
disturbances.
• Fixed motor disability appears to result from
extensive loss of axons in the corticospinal tracts.
• Diagnosis is facilitated by identification of earlier
attacks such as optic neuritis.
• MRI, CSF, and evoked response testing are
confirmatory.
• Treatment with ocrelizumab, an anti-CD20 B-cell
monoclonal antibody, is effective in patients with
primary progressive MS, and disease modifying
therapy is also indicated in patients with
secondary progressive MS who have coexisting
MS relapses.
 SUBACUTE COMBINED DEGENERATION
(VITAMIN B12 DEFICIENCY)

• This treatable myelopathy presents with subacute

paresthesias in the hands and feet, loss of
vibration and position sensation, and a
progressive spastic and ataxic weakness.
• Loss of reflexes due to an associated peripheral
neuropathy in a patient who also has Babinski
signs is an important diagnostic clue.
• Optic atrophy and irritability or other cognitive
changes may be prominent in advanced cases
and are occasionally the presenting symptoms.
• The myelopathy of subacute combined
degeneration tends to be diffuse rather than
focal; signs are generally symmetric and reflect
predominant involvement of the posterior and
lateral tracts, including Romberg’s sign.
• Causes include dietary deficiency, especially in
vegans, and gastric malabsorption syndromes
including pernicious anemia .
• Diagnosis is confirmed by the finding of
macrocytic red blood cells, a low serum B12
concentration, and elevated serum levels of
homocysteine and methylmalonic acid.
• Treatment is by replacement therapy, beginning
with 1000 μg of intramuscular vitamin B12
repeated at regular intervals or by subsequent
oral treatment.
 HYPOCUPRIC MYELOPATHY

• This myelopathy is similar to subacute combined

degeneration , except there is no neuropathy, and
explains cases with normal serum levels of B12.
• Low levels of serum copper are found,and often
there is also a low level of serum ceruloplasmin.
• Improvement or at least stabilization may be
expected with reconstitution of copper stores by
oral supplementation.
• There is microcytic or macrocytic anemia.
 TABES DORSALIS

• The characteristic symptoms of tabes are fleeting

and repetitive lancinating pains, primarily in the
legs or less often in the back, thorax, abdomen,
arms, and face.
• Ataxia of the legs and gait due to loss of position
sense occurs in half of patients.
• Paresthesias, bladder disturbances, and acute
abdominal pain with vomiting (visceral crisis)
occur in 15–30% of patients.
• The cardinal signs of tabes are loss of reflexes in
the legs; impaired position and vibratory sense;
Romberg sign; and, in almost all cases, bilateral
Argyll Robertson pupils, which fail to constrict to
light but accommodate
• Treatment of tabes dorsalis and other forms of
neurosyphilis consists of penicillin G administered
intravenously, or intramuscularly in combination
with oral probenecid
 HEREDITARY SPASTIC PARAPLEGIA

• Many cases of slowly progressive myelopathy are

genetic in origin .
• More than 60 different causative loci have been
identified, including autosomal dominant,
autosomal recessive, and X-linked forms.
• Most patients present with almost gradual
progressive spasticity and weakness in the legs,
usually but not always symmetrical.
• Sensory symptoms and signs are absent or mild,
but sphincter disturbances may be present.
• In some families, additional neurologic signs are
prominent, including nystagmus, ataxia, or optic
atrophy.
• Only symptomatic therapies are available.
Thanks