DISEASE OF THE SPINAL CORD

NEURO2

 spinal cord
Contents
 thin, tubular extension of the CNS contained within the
I. Approach to the Patient
bony spinal canal
II. Acute and Subacute Spinal Cord Diseases
 originates at the medulla and continues caudally to the
a. Compressive Myelopathies
conus medullaris at the lumbar level; its fibrous extension,
i. Neoplastic Spinal Cord Compression
the filum terminale, terminates at the coccyx
ii. Spinal Epidural Abscess
 adult spinal cord:
iii. Spinal Epidural Hematoma
- ~46 cm (18 in.) long
iv. Hematomyelia
- oval in shape
b. Noncompressive Myelopathies
- enlarged in the cervical and lumbar regions, where
i. Spinal Cord Infarction
neurons that innervate the upper and lower
ii. Inflammatory and Immune Myelopathies
extremities, respectively, are located
(Myelitis)
 white matter: contain ascending sensory and descending
iii. High-Voltage Electrical Injury
motor pathways are located peripherally
III. Chronic Myelopathies
 gray matter
a. Spondylotic Myelopathy
 inner region
b. Vascular Malformations of the Cord and Dura
 shaped like a four-leaf clover that surrounds the central
c. Retrovirus-Associated Myelopathies
canal (anatomically an extension of the fourth ventricle)
d. Syringomyelia
 where nerve cell bodies are clustered
e. Chronic Myelopathy of Multiple Sclerosis
 meninges:
f. Subacute Combined Degeneration (Vitamin B12
 pia, arachnoid, and dura
Deficiency)
 membranes that cover the spinal cord
g. Hypocupric Myelopathy
 continuous with those of the brain
h. Tabes Dorsalis
 CSF is contained within the subarachnoid space between
i. Hereditary Spastic Paraplegia
the pia and arachnoid
j. Adrenomyeloneuropathy
 spinal cord has 31 segments, each defined by an exiting
k. Other Chronic Myelopathies
ventral motor root and entering dorsal sensory root
 during embryologic development, growth of the cord lags
APPROACH TO THE PATIENT: SPINAL CORD DISEASE 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

Because of the presence of eight cervical spinal cord segments

but only seven cervical vertebrae:
 first seven pairs of cervical spinal nerves: exit above the
same-numbered vertebral bodies
 all subsequent nerves: exit below the same-numbered
vertebral bodies

 the relationship between spinal cord segments and the

corresponding vertebral bodies are important for localization
of lesions that cause spinal cord compression
 e.g., sensory loss below the circumferential level of the
umbilicus corresponds to the T10 cord segment but
indicates involvement of the cord adjacent to the T7 or T8
vertebral body
 at every level, the main ascending and descending tracts
are somatotopically organized with a laminated
distribution that reflects the origin or destination of nerve
fibers

SPINAL CORD ANATOMY RELEVANT TO CLINICAL SIGNS

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Determining the Level of the Lesion
 sensory level:
 horizontally defined level below which sensory, motor, and
autonomic function is impaired
 hallmark of a lesion of the spinal cord
 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
- unilateral spinal cord lesion: 1-2 segments higher
(because second-order sensory fibers, which originate
in the dorsal horn, ascend for one or two levels as they
 lesions 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  lower (not upper) abdominal muscles
paralysis  upward movement of the umbilicus when the
abdominal wall contracts (Beevor’s sign)
Lumbar Cord
LESION MANIFESTATION
L2-L4 paralysis of flexion and adduction of the thigh

cross anterior to the central canal to join the opposite weakness of leg extension at the knee
absent patellar reflex
spinothalamic tract
L5-S1 paralysis of the foot and ankle, flexion at the
- bilateral lesion: at the same level
knee, and extension of the thigh
 lesions that transect the descending corticospinal and other absent ankle jerks (S1)
motor tracts (upper motor neuron syndrome):
 paraplegia or quadriplegia Sacral Cord/ Conus Medulalris
 heightened deep tendon reflexes  conus medullaris: tapered caudal termination of the spinal
 Babinski signs cord, comprising the sacral and single coccygeal segments
 spasticity  conus syndrome
 transverse damage to the cord also produces autonomic  bilateral saddle anesthesia (S3-S5)
disturbances:  prominent bladder and bowel dysfunction (urinary
 absent sweating below the implicated cord level retention and incontinence with lax anal tone)
 bladder, bowel, and sexual dysfunction  impotence
 the uppermost level of a spinal cord lesion can also be  bulbocavernosus (S2-S4) and anal (S4-S5) reflexes are
localized by attention to the segmental signs corresponding to absent
disturbed motor or sensory innervation by an individual cord  muscle strength is largely preserved
segment  lesions of the cauda equina
 a band of altered sensation (hyperalgesia or hyperpathia)  low back and radicular pain
at the upper end of the sensory disturbance, fasciculations  asymmetric leg weakness and sensory loss
or atrophy in muscles innervated by one or several  variable areflexia in the lower extremities
segments, or a muted or absent deep tendon reflex may  relative sparing of bowel and bladder function
be noted at this level  mass lesions in the lower spinal canal often produce a mixed
 these signs also can occur with focal root or peripheral clinical picture with elements of both cauda equina and conus
nerve disorders; thus, they are most useful when they medullaris syndromes
occur together with signs of long tract damage
 spinal shock
 severe and acute transverse lesions causing the limbs to be
initially flaccid rather than spastic
 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
 Horner’s syndrome (miosis, ptosis, and facial hypohidrosis)
may accompany a cervical cord lesion at any level

LESION UPPERMOST LEVEL OF WEAKNESS AND

REFLEX LOSS
C5-C6 biceps
C7 finger and wrist extensors and triceps
C8 finger, and wrist flexion

Special Patterns of Spinal Cord Disease

Thoracic Cord

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 most fiber tracts—posterior columns and the spinocerebellar  lie outside the cord and compress the spinal cord or its
and pyramidal tracts—are situated on the side of the body vascular supply
they innervate  radicular pain is often prominent
 however, afferent fibers mediating pain and temperature  there is early sacral sensory loss and spastic weakness in
sensation ascend in the spinothalamic tract contralateral to the the legs with incontinence due to the superficial location
side they supply of the corresponding sensory and motor fibers in the
 the anatomic configurations of these tracts produce spinothalamic and corticospinal tracts
characteristic syndromes that provide clues to the underlying  further distinction is made between extradural and
disease process intradural masses
- extradural masses: generally malignant
Brown-Sequard Hemicord Syndrome - intradural masses: benign (neurofibroma being a
 ipsilateral weakness (corticospinal tract) common cause)  long duration of symptoms
 ipsilateral loss of joint position and vibratory sense (posterior  intramedullary lesions
column)  arise within the substance of the cord
 contralateral loss of pain and temperature sense  produce poorly localized burning pain rather than radicular
(spinothalamic tract) one or two levels below the lesion pain
 segmental signs (radicular pain, muscle atrophy, or loss of a  spare sensation in the perineal and sacral areas (“sacral
deep tendon reflex) are unilateral sparing”), reflecting the laminated configuration of the
 partial forms are more common than the fully developed spinothalamic tract with sacral fibers outermost;
syndrome corticospinal tract signs appear later

Central Cord Syndrome ACUTE AND SUBACUTE SPINAL CORD DISEASES

 results from selective damage to the gray matter nerve cells
and crossing spinothalamic tracts surrounding the central
canal
 central cord syndrome in the cervical cord:
 arm weakness out of proportion to leg weakness
 “dissociated” sensory loss: 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
 main causes:
 spinal trauma
 syringomyelia
 intrinsic cord tumors
Anterior Spinal Artery Syndrome
 occlusion or diminished flow in this artery  infarction of the
cord  bilateral tissue destruction at several contiguous levels
that spares the posterior column
 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 Lesion
 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= “around the
clock” pattern that may begin in any of the four limbs
 weakness of the ipsilateral shoulder and arm  weakness
of the ipsilateral leg  weakness of contralateral leg 
weakness of contralateral arm
 there is typically suboccipital pain spreading to the neck and
shoulders
Intramedullary and Extramedullary Syndromes
 extramedullary lesions
 symptoms of the cord diseases that evolve over days or weeks
are focal neck or back pain, followed by various combinations
of paresthesias, sensory loss, motor weakness, and sphincter
disturbance
 there may be mild sensory symptoms only or a devastating
functional transection of the cord
 when paresthesias begin in the feet and then ascend a
polyneuropathy is often considered, and in such cases the
presence of bladder disturbances and a sharply demarcated
spinal cord level provide important clues to the spinal cord
origin of the disease
 in severe and abrupt cases, areflexia reflecting spinal shock
may be present, but hyperreflexia supervenes over days or
weeks; persistent areflexic paralysis with a sensory level usually
indicates necrosis over multiple segments of the spinal cord
Approach to the Patient: Distinguishing Compressive from
Noncompressive Myelopathy
 first priority is to exclude treatable compression of the cord by
a mass lesion
 common mass lesions compressing the cord:
- tumor
- epidural abscess or hematoma
- herniated disk
- spondylitic vertebral pathology
 epidural compression due to malignancy or abscess:
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: more likely to produce
myelopathy without antecedent symptoms
 magnetic resonance imaging (MRI) with gadolinium:
 initial diagnostic procedure if it is available
 centered on the clinically suspected level

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 image the entire spine (cervical through sacral regions) to
search for additional clinically silent lesions
 once compressive lesions have been excluded,
noncompressive causes of acute myelopathy that are intrinsic
to the cord are considered, primarily vascular, inflammatory,
and infectious etiologies
COMPRESSIVE MYELOPATHIES
NEOPLASTIC SPINAL CORD COMPRESSION
 etiology
 in adults, most neoplasms are epidural in origin, resulting
from metastases to the adjacent vertebral column
 high proportion of bone marrow located in the axial
skeleton  propensity of solid tumors to metastasize to
the vertebral column
 almost any malignant tumor can metastasize to the spinal
column, with breast, lung, prostate, kidney, lymphoma, and
myeloma being particularly frequent
 thoracic spinal column is most commonly involved
 metastases from prostate and ovarian cancer occur
disproportionately in the sacral and lumbar vertebrae,
probably from spread through Batson’s plexus (network of
veins along the anterior epidural space)
 retroperitoneal neoplasms (especially lymphomas
sarcomas) enter the spinal canal laterally through the
intervertebral foramina and produce radicular pain with
signs of weakness that corresponds to the level of involved
nerve roots
 manifestations
 pain
- usually the initial symptom of spinal metastasis
- 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
- a recent onset of persistent back pain, particularly if in
the thoracic spine (which is uncommonly involved by
spondylosis), should prompt consideration of vertebral
metastasis
- rarely, pain is mild or absent
 diagnosis
 imaging should be obtained promptly if spinal cord
compression is suspected
 (+) radicular symptoms, (-) myelopathy: defer imaging for
24–48 h
 imaging of the entire length of the spine is important to
define the extent of disease (up to 40% of patients who
present with cord compression at one level are found to
have asymptomatic epidural metastases elsewhere)
 plain radiographs of the spine and radionuclide bone
scans: limited role in diagnosis because they do not
identify 15–20% of metastatic vertebral lesions and fail to
detect paravertebral masses that reach the epidural space
through the intervertebral foramina
 MRI:
- provides excellent anatomic resolution of the extent of
spinal tumors (Fig. 434-2)
- can distinguish between malignant lesions and other
masses—epidural abscess, tuberculoma, lipoma, or
epidural hemorrhage, among others—that present in a
similar fashion
 vertebral metastases: usually hypointense relative
to a normal bone marrow signal on T1-weighted
MRI; after the administration of gadolinium,
contrast enhancement may deceptively
“normalize” the appearance of the tumor by
increasing its intensity to that of normal bone
marrow
 infections of the spinal column (osteomyelitis and
related disorders): often cross the disk space to
involve the adjacent vertebral body
or
 intradural mass lesions:
 most are slow-growing and benign
 meningiomas and neurofibromas account for most of the
cases, with occasional cases caused by chordoma, lipoma,
dermoid, or sarcoma
- meningiomas (Fig. 434-3): 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:
 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
 uncommon
 manifestations
- present as central cord or hemicord syndromes, often
in the cervical region
- there may be poorly localized burning pain in the
extremities and sparing of sacral sensation
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 etiology in adults, lesions are ependymomas,  management of cord compression includes (1) glucocorticoids
hemangioblastomas, or low-grade astrocytomas (Fig. 434- to reduce cord edema, (2) local radiotherapy (initiated as early
4) as possible) to the symptomatic lesion, and (3) specific therapy
 treatment for the underlying tumor type
- complete resection of an intramedullary ependymoma  glucocorticoids (typically dexamethasone, 10 mg
is often possible with microsurgical techniques intravenously)
- debulking of an intramedullary astrocytoma can also  can be administered before an imaging study if there is
be helpful, as these are often slowly growing lesions clinical suspicion of cord compression and continued at a
- value of adjunctive radiotherapy and chemotherapy is lower dose (4 mg every 6 h orally) until definitive
uncertain treatment with radiotherapy (generally 30–40 Gy
 secondary (metastatic) intramedullary tumors also occur, administered in 8–10 fractions) and/or surgical
especially in patients with advanced metastatic disease, decompression is completed
although these are not nearly as frequent as brain metastases  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
 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
 although most patients do not experience recurrences in
the months following radiotherapy, with survival beyond 2
years recurrence becomes increasingly likely and can be
managed with additional radiotherapy
 newer techniques such as stereotactic radiosurgery can
deliver high doses of focused radiation with similar rates of
response compared to traditional radiotherapy, and these
are increasingly being used, particularly for patients with
traditionally radioresistant tumors or those requiring re-
irradiation
 biopsy of the epidural mass is unnecessary in patients with
known primary cancer, but it is indicated if a history of
underlying cancer is lacking
 surgery
 either decompression by laminectomy or vertebral body
resection
 indications:
- when signs of cord compression worsen despite
radiotherapy
- when the maximum-tolerated dose of radiotherapy
has been delivered previously to the site
- when a vertebral compression fracture or spinal
instability contributes to cord compression

SPINAL EPIDURAL ABSCESS

 manifestations
Treatment  include:
- midline back or neck pain

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 aching pain is almost always present, either over - level of the puncture should be planned to minimize
the spine or in a radicular pattern the risk of meningitis due to passage of the needle
 duration of pain prior to presentation is generally through infected tissue; a high cervical tap is
≤2 weeks but may on occasion be several months sometimes the safest approach
or longer - CSF abnormalities in epidural and subdural abscess:
- fever  pleocytosis with a preponderance of PMNs
 typically but not invariably present, accompanied  elevated protein level
by elevated WBC count, ESR, and CRP  reduced glucose level
- progressive limb weakness - responsible organism is not cultured unless there is
 prompt recognition of this distinctive process may prevent associated meningitis
permanent sequelae
 as the abscess expands, further spinal cord damage results Treatment
from venous congestion and thrombosis  decompressive laminectomy with debridement combined with
 once weakness and other signs of myelopathy appear, long-term antibiotic treatment
progression may be rapid and irreversible  surgical management remains the treatment of choice unless
 a more chronic sterile granulomatous form of abscess is the abscess is limited in size and causes few or no neurologic
also known, usually after treatment of an acute epidural signs
infection  surgical evacuation
 etiology  prevents development of paralysis
 risk factors:  may improve or reverse paralysis in evolution, but is
- impaired immune status (HIV, diabetes mellitus, renal unlikely to improve deficits of more than several days in
failure, alcoholism, malignancy) duration
- intravenous drug abuse  broad-spectrum antibiotics should be started empirically
- infections of the skin or other tissues before surgery and then modified on the basis of culture
 mechanism: results; medication is generally continued for 6–8 weeks
- hematogenous spread of bacteria from the skin  vancomycin 15–20 mg/kg q12h (staphylococcus including
(furunculosis), soft tissue (pharyngeal or dental MRSA, streptococcus)
abscesses; sinusitis), or deep viscera (bacterial  ceftriaxone 2 gm q24h (gram-negative bacilli)
endocarditis): two-thirds of epidural infections  metronidazole 30 mg/kg/d divided into q6h intervals
- direct extension of a local infection to the subdural (anaerobes)
space: remaining one-third  if surgery is contraindicated or if there is a fixed paraplegia or
 examples of local predisposing conditions are: quadriplegia that is unlikely to improve following surgery,
 vertebral osteomyelitis long-term administration of systemic and oral antibiotics can
 decubitus ulcers be used; in such cases, the choice of antibiotics may be guided
 lumbar puncture by results of blood cultures
 epidural anesthesia  with prompt diagnosis and treatment of spinal epidural
 spinal surgery abscess, up to two-thirds of patients experience significant
 most cases are due to: recovery
- Staphylococcus aureus
- gram-negative bacilli SPINAL EPIDURAL HEMATOMA
- Streptococcus  hemorrhage into the epidural (or subdural) space causes acute
- anaerobes focal or radicular pain followed by variable signs of a spinal
- fungi cord or conus medullaris disorder
 Methicillin resistant Staphylococcus aureus (MRSA) is an  predisposing conditions:
important consideration, and therapy should be tailored to  therapeutic anticoagulation
this possibility  trauma
 tuberculosis from an adjacent vertebral source (Pott’s  tumor
disease) remains an important cause in the developing  blood dyscrasias
world  rare cases complicate lumbar puncture or epidural anesthesia
 diagnosis  MRI and computed tomography (CT): confirm the clinical
 MRI (Fig. 434-5): localizes the abscess and excludes other suspicion and can delineate the extent of the bleeding
causes of myelopathy  treatment
 blood cultures: positive in more than half of cases, but  prompt reversal of any underlying clotting disorder and
direct aspiration of the abscess at surgery is often required surgical decompression
for a microbiologic diagnosis  surgery may be followed by substantial recovery, especially
 lumbar puncture in patients with some preservation of motor function
- only required if encephalopathy or other clinical signs preoperatively
raise the question of associated meningitis, a feature
that is found in <25% of cases

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 because of the risk of hemorrhage, lumbar puncture should be

avoided whenever possible in patients with severe
thrombocytopenia or other coagulopathies

HEMATOMYELIA
 hemorrhage into the substance of the spinal cord is a rare
result of trauma, intraparenchymal vascular malformation,
vasculitis due to polyarteritis nodosa or systemic lupus
erythematosus (SLE), bleeding disorders, or a spinal cord
neoplasm
 manifestations
 acute painful transverse myelopathy
 large lesions  extension into the subarachnoid space 
subarachnoid hemorrhage
 diagnosis: MRI or CT
 treatment
 supportive
 surgical intervention is generally not useful; an exception is
hematomyelia due to an underlying vascular malformation,
for which spinal angiography and endovascular occlusion
may be indicated, or surgery to evacuate the clot and
remove the underlying vascular lesion SPINAL CORD INFARCTION
 blood supply of the cord:
NONCOMPRESSIVE MYELOPATHIES  single anterior spinal artery
 most frequent causes of noncompressive acute transverse - originates in paired branches of the vertebral arteries
myelopathy: at the cranciocervical junction
 spinal cord infarction - fed by additional radicular vessels that arise at C6, at
 systemic inflammatory disorders, including SLE and an upper thoracic level, and, most consistently, at T11-
sarcoidosis L2 (artery of Adamkiewicz)
 demyelinating diseases, including multiple sclerosis (MS) - at each spinal cord segment, paired penetrating
 neuromyelitis optica (NMO) vessels branch from the anterior spinal artery to supply
 postinfectious or idiopathic transverse myelitis, which is the anterior two-thirds of the cord
presumed to be an immune condition related to acute  paired posterior spinal arteries
disseminated encephalomyelitis - often become less distinct below the midthoracic level
 infectious (primarily viral) causes - supply the posterior columns
 after spinal cord compression is excluded, the evaluation  presence of the artery of Adamkiewicz below, and the anterior
generally requires a lumbar puncture and a search for spinal artery circulation above, creates a region of marginal
underlying systemic disease (Table 434-3) blood flow in the upper thoracic segments
 with hypotension or cross-clamping of the aorta, cord
infarction typically occurs at the level of T3-T4, and also at
boundary zones between the anterior and posterior spinal
artery territories
 the latter may result in a rapidly progressive syndrome
over hours of weakness and spasticity with little sensory
change
 manifestations
 anterior spinal artery territory infraction
- anterior cord syndrome:
 paraplegia or quadriplegia
 dissociated sensory loss affecting pain and
temperature sense but sparing vibration and
position sense
 loss of sphincter control
- onset may be sudden but more typically is progressive
over minutes or a few hours, unlike stroke in the
cerebral hemispheres
- sharp midline or radiating back pain localized to the
area of ischemia is frequent

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- areflexia due to spinal shock is often present initially;
with time, hyperreflexia and spasticity appear
 posterior spinal arteries territory infarction
- less common
- resulting in loss of posterior column function either on
one side or bilaterally
 etiology
 aortic atherosclerosis
 dissecting aortic aneurysm
 vertebral artery occlusion or dissection in the neck
 aortic surgery
 profound hypotension from any cause
 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
- typically manifests as back pain followed by an anterior
cord syndrome with progressive paralysis and loss of
sphincter control, and is likely vascular in origin
 cardiogenic emboli
 vasculitis
 collagen vascular disease (particularly SLE, Sjögren’s
syndrome, antiphospholipid antibody syndrome)
 embolism of nucleus pulposus material into spinal vessels,
usually from local spine trauma
 in a substantial number of cases, no cause can be found,
and thromboembolism in arterial feeders is suspected
 diagnosis
 MRI may fail to demonstrate infarctions of the cord,
especially in the first day, but often the imaging becomes
abnormal at the affected level
 treatment
 acute anticoagulation
- not indicated in cord infarction due to presumed
thromboembolism, except for the unusual TIA or
incomplete infarction with a stuttering or progressive
course
- treatment for antiphospholipid antibody syndrome
 increasing SBP to a MAP 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
 prognosis
 influenced by the severity of the deficits at presentation
- patients with severe motor weakness and those with
persistent areflexia usually do poorly, but in one recent
large series some improvement over time occurred in
many patients, with more than half ultimately
regaining some ambulation
INFLAMMATORY AND IMMUNE MYELOPATHIES (MYELITIS)
 includes the demyelinating conditions MS, NMO, and
postinfectious myelitis, as well as sarcoidosis and systemic
autoimmune disease
 in approximately one-quarter of cases of myelitis, no
underlying cause can be identified; some will later manifest
additional symptoms of an immune-mediated disease
 recurrent episodes of myelitis: immune-mediated disease or
infection with herpes simplex virus (HSV) type 2
Multiple Sclerosis
 presentation
 acute myelitis (Asian or African ancestry)
 partial cord syndrome; rarely cause a transverse
myelopathy (i.e., attacks of bilateral sensory disturbances,
unilateral or bilateral weakness, and bladder or bowel
symptoms) (Caucasians)
 diagnosis
 MRI findings in MS-associated myelitis:
- mild swelling of the cord
- diffuse or multifocal “shoddy” areas of abnormal signal
on T2-weighted sequences
- contrast enhancement, indicating disruption in the
blood-brain barrier associated with inflammation, is
present in many acute cases
 in one study 68% of patients presenting with partial myelitis
developed MS after a mean follow-up of 4 years
 risk factors for conversion to MS:
- age <40 years
- inflammatory CSF
- >3 periventricular lesions on brain MRI
 treatment of acute episodes of MS-associated myelitis:
 methylprednisolone (500 mg qd for 3 days) followed by
oral prednisone (1 mg/kg/d for several weeks, then
gradual taper)
 plasma exchange may be indicated for severe cases if
glucocorticoids are ineffective
Neuromyelitis Optica
 immune-mediated demyelinating disorder consisting of a
severe myelopathy that is typically longitudinally extensive
(lesion spans three or more vertebral segments)
 associated with:
 optic neuritis that is often bilateral and may precede or
follow myelitis by weeks or months
- recurrent myelitis without optic nerve or other
involvement can also occur
 brainstem and, in some cases, hypothalamic or focal
cerebral white matter involvement
 SLE as well as with other systemic autoimmune diseases
 rare cases are paraneoplastic in origin
 diagnosis
 CSF studies:
- variable mononuclear pleocytosis of up to several
hundred cells per microliter
- unlike MS, oligoclonal bands are generally absent
 serum autoantibodies against the water channel protein
aquaporin-4: present in 60–70% of patients
 autoantibodies against the CNS myelin protein myelin
oligodendrocyte glycoprotein (MOG)
 treatment
 no definitive trials of therapy for NMO
 glucocorticoids: recommended treatment of acute relapses
 plasma exchange: for refractory cases
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DISEASE OF THE SPINAL CORD
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 prophylactic treatment with azathioprine, mycophenylate,  gallium scan may assist in the diagnosis
or rituximab: may protect against subsequent relapses;  treatment
treatment for ≥5 years is generally recommended  oral glucocorticoids: initial treatment
 immunosuppressant drugs (TNF-α inhibitor infliximab): for
Systemic Immune-Mediated Disorders resistant cases
 myelitis occurs in a small number of patients with SLE and
patients are at high risk of developing future episodes of Postinfectious Myelitis
myelitis and/or optic neuritis  myelitis following an infection or vaccination
 etiology of SLE-associated myelitis:  organisms implicated:
 antibodies to aquaporin-4 (satisfy diagnostic criteria for  Epstein-Barr virus (EBV)
NMO-spectrum disorder)  cytomegalovirus (CMV)
 anti-phospholipid antibodies: presence appears to be no  mycoplasma
more frequent in SLE patients with and without myelitis  influenza
 uncertain in others  measles
 treatment  varicella
 no systematic trials of therapy for SLE myelitis  mumps
 high-dose glucocorticoids followed by cyclophosphamide:  yellow fever
recommended  course: often begins as the patient appears to be recovering
 plasma exchange: for acute severe episodes of transverse from an acute febrile infection, or in the subsequent days or
myelitis that do not initially respond to glucocorticoids weeks, but an infectious agent cannot be isolated from the
 differential diagnosis nervous system or CSF
 Sjögren’s syndrome can also be associated with NMO-  pathogenesis: autoimmune disorder triggered by infection
spectrum disorder and with cases of chronic progressive and is not due to direct infection of the spinal cord
myelopathy  treatment
 other immune-mediated myelitides:  no RCT of therapy exist
- antiphospholipid antibody syndrome  glucocorticoids: usual treatment
- mixed connective tissue disease  plasma exchange: in fulminant cases
- Behçet’s syndrome
- vasculitis related to polyarteritis nodosa, perinuclear Acute Infectious Myelitis
antineutrophilic cytoplasmic (p-ANCA) antibodies, or  etiology
primary central nervous system vasculitis  viral:
 sarcoid myelopathy: may present as a slowly progressive - herpes zoster: best characterized viral myelitis
or relapsing disorder - HSV types 1 and 2
 diagnosis  HSV-2 (and less commonly HSV-1): distinctive
 difficult when systemic manifestations of sarcoid are minor syndrome of recurrent sacral cauda equina neuritis
or absent (nearly 50% of cases) or when other typical in association with outbreaks of genital herpes
neurologic manifestations of the disease—such as cranial (Elsberg’s syndrome)
neuropathy, hypothalamic involvement, or meningeal - EBV
enhancement visualized by MRI—are lacking - CMV
 MRI: - rabies virus
- edematous swelling of the spinal cord that may mimic - Zika virus
tumor - poliomyelitis: prototypic viral myelitis; restricted to the
- gadolinium enhancement of active lesions and in some anterior gray matter of the cord containing the spinal
cases nodular enhancement of the adjacent surface of motoneurons
the cord - enteroviruses (including enterovirus 71 and coxsackie),
- lesions may be single or multiple, and on axial images, Japanese encephalitis and other flaviviruses (West Nile
enhancement of the central cord is often present virus): polio-like syndrome
 typical CSF profile:  enterovirus D-68: recent cases of paralysis in
- mild lymphocytic pleocytosis children and adolescents; causal role has not been
- mildly elevated protein level established
- reduced glucose and oligoclonal bands (minority of - HIV or human T cell lymphotropic virus type 1 (HTLV-
cases) 1): chronic viral myelitic infections
 slit-lamp examination of the eye: to search for uveitis  bacterial
 chest x-ray and CT: to assess pulmonary involvement and - almost any pathogenic species may be responsible,
mediastinal lymphadenopathy including:
 serum or CSF angiotensin-converting enzyme (ACE; CSF  Borrelia burgdorferi (Lyme disease)
values elevated in only a minority of cases)  Listeria monocytogenes
- usefulness of spinal fluid ACE is uncertain  Mycobacterium tuberculosis
 serum calcium  Treponema pallidum (syphilis)

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DISEASE OF THE SPINAL CORD
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 Mycoplasma pneumoniae: may be a cause of
myelitis, but its status is uncertain because many
cases are more properly classified as postinfectious
- bacterial and mycobacterial myelitis:
 most are essentially abscesses
 less common than viral causes
 less frequent than cerebral bacterial abscess
 parasitic:
- schistosomiasis:
 important cause of parasitic myelitis in endemic
areas
 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:
 occasionally cause a focal myelopathy
 diagnosis should especially be considered in
patients with AIDS
- cysticercosis: less common than parenchymal brain or
meningeal involvement
 treatment
 in cases of suspected viral myelitis, it may be appropriate
to begin specific therapy pending laboratory confirmation
- herpes zoster, HSV, and EBV myelitis: IV acyclovir (10
mg/kg q8h) or oral valacyclovir (2 g tid) for 10–14 days
- CMV: ganciclovir (5 mg/kg IV bid) plus foscarnet (60
mg/kg IV tid) or cidofovir (5 mg/kg per week for 2
weeks)
HIGH-VOLTAGE ELECTRICAL INJURY
 spinal cord injuries are prominent following electrocution from
lightning strikes or other accidental electrical exposures
 rare injury type, and limited data incriminate a vascular
pathology involving the anterior spinal artery and its branches
in some cases
 manifestation: transient weakness acutely (often with an
altered sensorium and focal cerebral disturbances), sometimes
followed several days or even weeks later by a myelopathy that
can be severe and permanent
 treatment: supportive
CHRONIC MYELOPATHIES
SPONDYLOTIC MYELOPATHY
 one of the most common causes of chronic cord compression
and of gait difficulty in the elderly
 symptoms
 neck and shoulder pain with stiffness are early symptoms
 impingement of bone and soft tissue overgrowth on nerve
roots  radicular arm pain, most often in a C5 or C6
distribution
 compression of the cervical cord (fewer than one-third of
cases)  slowly progressive spastic paraparesis that is
sometimes asymmetric + paresthesias in the feet and
hands
 diminished vibratory sense in the legs
 Romberg sign
 sensory level for vibration or pinprick on the upper thorax
(occasional)
 coughing or straining  leg weakness or radiating arm or
shoulder pain
 dermatomal sensory loss in the arms
 atrophy of intrinsic hand muscles
 increased deep-tendon reflexes in the legs
 extensor plantar responses
 urinary urgency or incontinence in advanced cases
 diminished tendon reflex in the arms at some level, most
often at the biceps (C5-C6)
 in individual cases, radicular, myelopathic, or combined
signs may predominate
 diagnosis
 should be considered in appropriate cases of progressive
cervical myelopathy, paresthesias of the feet and hands, or
wasting of the hands
 usually made by MRI
- axial views: extrinsic cord compression and
deformation
- T2-weighted sequences: may reveal areas of high
signal intensity within the cord adjacent to the site of
compression
 may be suspected from CT images
 plain x-rays are less helpful
 treatment
 cervical collar
- may be helpful in milder cases
- likelihood of progression of medically treated
myelopathy is high, estimated at 8% over 1 year
 surgical decompression
- definitive therapy
- either posterior laminectomy or an anterior approach
with resection of the protruded disk and bony material
VASCULAR MALFORAMATIONS OF THE CORD AND DURA
 comprise ~4% of all mass lesions of the cord and overlying
dura
 treatable causes of progressive myelopathy
 most common are fistulas located within the dura or
posteriorly along the surface of the cord
 dural arteriovenous (AV) fistulas
 most are located at or below the midthoracic level, usually
consisting of a direct connection between a radicular
feeding artery in the nerve root sleeve with dural veins
 manifestation
- typical presentation is middle-aged man with a
progressive myelopathy that worsens slowly or
intermittently and may have periods of remission,
sometimes mimicking MS (typical presentation)
- acute deterioration due to hemorrhage into the spinal
cord (hematomyelia) or subarachnoid space (rare)
 in many cases, progression results from local
ischemia and edema due to venous congestion
- incomplete sensory, motor, and bladder disturbances
 motor disorder may predominate and produce a
mixture of upper and restricted lower motor
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DISEASE OF THE SPINAL CORD
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neuron signs, simulating amyotrophic lateral

sclerosis (ALS)
- pain over the dorsal spine, dysesthesias, or radicular
pain may be present
- other symptoms suggestive of AV malformation (AVM)
or dural fistula:
 intermittent claudication
 symptoms that change with posture, exertion,
Valsalva maneuver, or menses
 fever
- intramedullary AVM disorders
 less common
 one unusual disorder is a progressive thoracic
myelopathy with paraparesis developing over
weeks or months, characterized pathologically by
abnormally thick, hyalinized vessels within the cord
(subacute necrotic myelopathy, or
Alajouanine syndrome
 diagnosis
 PE
- spinal bruits: infrequent but may be sought at rest and
after exercise in suspected cases
- vascular nevus on the overlying skin: may indicate an
underlying vascular malformation as occurs with
Klippel-Trenaunay-Weber syndrome
 MR angiography and CT angiography: detect the draining
vessels of many AVMs (Fig. 434-6)
 selective spinal angiography:
- definitive diagnosis
- defines the feeding vessels and the extent of the
malformation
 treatment
 tailored to the anatomy and location of the lesion
 microsurgical resection
 endovascular embolization of the major feeding vessels
 microsurgical resection + endovascular embolization of
the major feeding vessels
Foix-
RETROVIRUS-ASSOCIATED MYELOPATHIES
 myelopathy associated with HTLV-1
 formerly called tropical spastic paraparesis
 slowly progressive spastic syndrome with variable sensory
and bladder disturbance
 manifestations
 mild back or leg pain in approximately half of patients
 neurologic signs may be asymmetric, often lacking a well-
defined sensory level
 only sign in the arms may be hyperreflexia after several
years of illness
 course of disease
 onset is usually insidious, and the tempo of progression of
the illness occurs at a variable rate (in one study, median
time for progression to cane, walker, or wheelchair
dependent state was 6, 13, and 21 years, respectively)
 progression appears to be more rapid in older patients
and those with higher viral loads
 diagnosis
 serum HTLV-1-specific antibody by enzyme-linked
immunosorbent assay (ELISA)
 radioimmunoprecipitation or Western blot analysis:
- to confirm diagnosis
- finding of HTLV-1 seropositivity in a patient with
myelopathy does not necessarily prove that HTLV-1 is
causative
 CSF/serum antibody index: may provide support by
establishing intrathecal synthesis of antibodies, including
oligoclonal antibodies, favoring HTVL-1 myelopathy over
asymptomatic carriage
 proviral DNA by polymerase chain reaction (PCR) in serum
and CSF cells: ancillary part of diagnosis
 pathogenesis of the myelopathy
 uncertain
 from an immune response directed against HTLV-1
antigens in the nervous system

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DISEASE OF THE SPINAL CORD
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 secondary autoimmunity triggered by the viral infection vertical nystagmus, episodic dizziness or vertigo, and
 progressive myelopathy can also result from HIV infection tongue weakness with atrophy
- it is characterized by vacuolar degeneration of the  diagnosis
posterior and lateral tracts, resembling subacute  MRI: accurately identifies developmental and acquired
combined degeneration syrinx cavities and their associated spinal cord
 treatment enlargement (Fig. 434-7)
 no proven effective treatment  images of the brain and the entire spinal cord should be
 use of low dose oral glucocorticoids can be tried obtained to delineate the full longitudinal extent of the
 interferon: uncertain value syrinx, assess posterior fossa structures for the Chiari
 antiviral treatment: ineffective malformation, and determine whether hydrocephalus is
 symptomatic therapy for spasticity and bladder symptoms present
may be helpful

SYRINGOMYELIA
 syringomyelia: developmental cavity of the cervical cord that
may enlarge and produce progressive myelopathy or may
remain asymptomatic
 course
 symptoms begin insidiously in adolescence or early
adulthood, progress irregularly, and may undergo
spontaneous arrest for several years
 many young patients acquire a cervical-thoracic scoliosis
 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
 pathophysiology syrinx expansion
 likely due to some interference with the normal flow of CSF
(perhaps by the Chiari malformation)
 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
 manifestations Treatment
 presents as central cord syndrome consisting of:  treatment is generally unsatisfactory
- regional dissociated sensory loss (loss of pain and  patients with few symptoms and signs from the syrinx do not
temperature sensation with sparing of touch and require surgery and are followed by serial clinical and imaging
vibration) examinations
- areflexic weakness in the upper limbs  surgery may stabilize the neurologic deficit, and some patients
 sensory deficit with a distribution that is “suspended” over improve
the nape of the neck, shoulders, and upper arms (cape  with Chiari malformations shunting of hydrocephalus precedes
distribution) or in the hands any attempt to correct the syrinx
 most cases begin asymmetrically with unilateral sensory  suboccipital craniectomy, upper cervical laminectomy, and
loss in the hands that leads to injuries and burns that are placement of a dural graft: for decompression of Chiari
not appreciated by the patient tonsillar herniation and reestablish fourth ventricular outflow
 expansion of the cavity in the gray matter of the cord   direct decompression or drainage
muscle wasting in the lower neck, shoulders, arms, and - for large syrinx cavity
hands with asymmetric or absent reflexes in the arms  added benefit uncertain
 cavity enlargement with compression of the long tracts   complications are common
spasticity and weakness of the legs, bladder and bowel - for symptomatic syrinx cavities 2° to trauma or
dysfunction, and a Horner’s syndrome appear infection, a small shunt is inserted between the cavity
 damage to the descending tract of the trigeminal nerve and subarachnoid space, or cavity can be fenestrated
(C2 level or above) facial numbness and sensory loss  resection: intramedullary spinal cord tumor
 cough-induced headache and neck, arm, or facial pain
(with Chiari malformations) CHRONIC MYELOPATHY OF MULTIPLE SCLEROSIS
 extension of the syrinx into the medulla (syringobulbia)   chronic progressive myelopathy is the most frequent cause of
palatal or vocal cord paralysis, dysarthria, horizontal or disability in both primary progressive and secondary
progressive forms of MS

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DISEASE OF THE SPINAL CORD
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 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
 facilitated by identification of earlier attacks such as optic
neuritis
 MRI, CSF, and evoked response testing: confirmatory
 treatment
 ocrelizumab
- anti-CD20 B-cell monoclonal antibody
- effective in patients with primary progressive MS
 disease modifying therapy is also indicated in patients with
secondary progressive MS who have coexisting MS
relapses
SUBACUTE COMBINED DEGENERATION (VITAMIN B12
DEFICIENCY)
 presentation:
 subacute paresthesias in the hands and feet
 loss of vibration and position sensation
 progressive spastic and ataxic weakness
 loss of reflexes due to an associated peripheral neuropathy
+ Babinski signs= important diagnostic clue
 optic atrophy and irritability or other cognitive changes in
advanced cases; may occasionally be the presenting
symptoms
 myelopathy 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
 etiology
 dietary deficiency, especially in vegans
 gastric malabsorption syndromes (pernicious anemia)
 diagnosis
 macrocytic red blood cells
 low serum B12 concentration,
 elevated serum homocysteine and methylmalonic acid
 treatment
 replacement therapy: begins with 1000 μg of IM vitamin
B12 repeated at regular intervals or by subsequent oral
treatment
HYPOURIC MYELOPATHY
 similar to subacute combined degeneration, except there is no
neuropathy, and explains cases with
 etiology
 GI procedures, particularly bariatric surgery (impaired
copper absorption)
 excess zinc from health food supplements or in the past
zinc-containing denture creams (zinc impair copper
absorption via induction of metallothionein, a copper-
binding protein)
 many cases are idiopathic: pathophysiology and pathology
are not known
 diagnosis
 normal serum levels of B12
 low levels of serum copper
 low level of serum ceruloplasmin
 microcytic or macrocytic anemia
 treatment
 reconstitution of copper stores by oral supplementation:
improvement or at least stabilization may be expected
TABES DORSALIS
 classic syphilitic syndromes of tabes dorsalis and
meningovascular inflammation of the spinal cord are now less
frequent than in the past but must be considered in the
differential diagnosis of spinal cord disorders
 diabetic polyradiculopathy may simulate this condition
 manifestations
 characteristic symptoms: fleeting and repetitive lancinating
pains, primarily in the legs or less often in the back, thorax,
abdomen, arms, and face
 loss of position sense  ataxia of the legs and gait (half of
patients)
 paresthesias, bladder disturbances, and acute abdominal
pain with vomiting (visceral crisis) (15–30%)
 cardinal signs:
- loss of reflexes in the legs
- impaired position and vibratory sense
- Romberg sign
- bilateral Argyll Robertson pupils, which fail to constrict
to light but accommodate
 treatment: penicillin G administered IV, or IM 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
 especially for the recessive and X-linked forms, a family history
of myelopathy may be lacking
 most patients present with almost imperceptibly 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
 the onset may be as early as the first year of life or as late as
middle adulthood
 only symptomatic therapies are available
ADRENOMYELONEUROPATHY
 X-linked disorder that is a variant of adrenoleukodystrophy
(ALD)
 responsible gene encodes the adrenoleukodystrophy protein
(ALDP), a peroxisomal membrane transporter involved in
carrying long-chain fatty acids to peroxisomes for degradation
 manifestations
 most affected males have a history of adrenal insufficiency
and then develop a progressive spastic (or ataxic)
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paraparesis beginning in early or sometimes middle  nitrous oxide inhalation: produce a myelopathy identical to
adulthood; some patients also have a mild peripheral subacute combined degeneration
neuropathy  SLE, Sjögren’s syndrome, and sarcoidosis may each cause a
 female heterozygotes may develop a slower, insidiously myelopathy without overt evidence of systemic disease
progressive spastic myelopathy beginning later in  cancer-related causes of chronic myelopathy:
adulthood and without adrenal insufficiency  includes:
 diagnosis: demonstration of elevated levels of very-long-chain - radiation injury
fatty acids in plasma and in cultured fibroblasts - rare paraneoplastic myelopathies
 treatment  most often associated with lung cancer and anti-
 corticosteroid replacement: indicated if w/ hypoadrenalism Hu or anti-CV2/CRMP5 antibodies or with
 allogeneic bone marrow transplantation lymphoma that causes a syndrome of destruction
- successful in slowing progression of cognitive decline of anterior horn cells
- ineffective for the myelopathy  NMO with aquaporin-4 antibodies can also rarely
 nutritional supplements (Lorenzo’s oil): no evidence of be paraneoplastic in origin
efficacy  metastases to the cord are probably more common than
either of these in patients with cancer
OTHER CHRONIC MYELOPATHIES  often, a cause of intrinsic myelopathy can be identified
only through periodic reassessment
 primary lateral sclerosis
 mid to late life onset degenerative disorder
 manifestations REHABILITATION OF SPINAL CORD DISORDERS
- progressive spasticity with weakness, eventually
accompanied by dysarthria and dysphonia
- bladder symptoms occur in approximately half of
patients
- sensory function is spared
 resembles ALS and is considered a variant of the motor
neuron degenerations, but without the characteristic lower
motor neuron disturbance and with typically a slower
progression
 some cases may represent late-onset cases of familial
spastic paraplegia, particularly autosomal recessive or X-
linked varieties in which a family history may be absent
 tethered cord syndrome
 developmental disorder of the lower spinal cord and nerve
roots
 prospects for recovery from an acute destructive spinal cord
 manifestations
lesion fade after ~6 months
- rarely presents in adulthood as low back pain
 there are currently no effective means to promote repair of
accompanied by a progressive lower spinal cord
injured spinal cord tissue
and/or nerve root syndrome
 promising but entirely experimental approaches:
- some patients have a small leg or foot deformity
 use of factors that influence reinnervation by axons of the
indicating a long-standing process
corticospinal tract, nerve and neural sheath graft bridges
- in others, a dimple, patch of hair, or sinus tract on the
 forms of electrical stimulation at the site of injury
skin overlying the lower back is the clue to a
 local introduction of stem cells
congenital lesion
 the disability associated with irreversible spinal cord damage is
 diagnosis
determined primarily by the level of the lesion and by whether
- MRI
the disturbance in function is complete or incomplete (Table
 demonstrates a low-lying conus medullaris and
434-4); even a complete high cervical cord lesion may be
thickened filum terminale
compatible with a productive life
 may also reveal diastematomyelia (division of the
 primary goals:
lower spinal cord into two halves), lipomas, cysts,
 development of a rehabilitation plan framed by realistic
or other congenital abnormalities of the lower
expectations
spine coexisting with the tethered cord
 attention to the neurologic, medical, and psychological
 treatment: surgical release
complications that commonly arise
 rare toxic causes of spastic myelopathy:
 many of the usual symptoms associated with medical illnesses,
 lathyrism due to ingestion of chickpeas containing the
especially somatic and visceral pain, may be lacking because of
excitotoxin β-N-oxalylamino-l-alanine (BOAA): primarily in
the destruction of afferent pain pathways
the developing world

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 unexplained fever, worsening of spasticity, or deterioration in  acts by facilitating γ-aminobutyric acid–mediated

neurologic function should prompt a search for infection, inhibition of motor reflex arcs
thrombophlebitis, or an intraabdominal pathology  useful for leg spasms that interrupt sleep (2–4 mg
 loss of normal thermoregulation and inability to maintain at bedtime)
normal body temperature can produce recurrent fever - tizanidine (2–8 mg tid)
(quadriplegic fever), although most episodes of fever are due  α2 adrenergic agonist that increases presynaptic
to infection of the urinary tract, lung, skin, or bone inhibition of motor neurons, is another option
 bladder dysfunction - dantrolene (25–100 mg qid)
 generally results from loss of supraspinal innervation of  direct muscle inhibitor
the detrusor muscle of the bladder wall and the sphincter  for nonambulatory patients
musculature  potentially hepatotoxic
 detrusor spasticity - in refractory cases
- anticholinergic drugs (oxybutynin, 2.5–5 mg qid)  intrathecal baclofen administered via an implanted
- tricyclic antidepressants with anticholinergic properties pump
(imipramine, 25–200 mg/d)  botulinum toxin injections,
 failure of the sphincter muscle to relax during bladder  dorsal rhizotomy
emptying (urinary dyssynergia)  chronic pain
- α-adrenergic blocking agent terazosin hydrochloride  gabapentin or pregabalin: useful in this setting
(1–2 mg tid or qid), with intermittent catheterization  epidural electrical stimulation and intrathecal infusion of
- condom catheter in men or a permanent indwelling pain medications: have been tried with some success
catheter  paroxysmal autonomic hyperreflexia
- surgical options: creation of an artificial bladder by  may occur following lesions above the major splanchnic
isolating a segment of intestine that can be sympathetic outflow at T6
catheterized intermittently (enterocystoplasty) or can  headache, flushing, and diaphoresis above the level of the
drain continuously to an external appliance (urinary lesion, as well as hypertension with bradycardia or
conduit) tachycardia, are the major symptoms
 bladder areflexia due to acute spinal shock or conus  trigger is typically a noxious stimulus—for example,
lesions: catheterization bladder or bowel distention, a urinary tract infection, or a
 bowel regimens and disimpaction are necessary in most decubitus ulcer—below the level of the cord lesion
patients to ensure at least biweekly evacuation and avoid  treatment
colonic distention or obstruction - removal of offending stimuli; ganglionic blocking
 venous thrombosis and pulmonary embolism agents (mecamylamine, 2.5–5 mg)
 calf-compression devices - short-acting antihypertensive drugs are useful in some
 anticoagulation with low-molecular-weight heparin patients
- anticoagulation should probably be continued for 3
months in cases of persistent paralysis,
 decubitus ulcers
 prophylaxis:
- frequent changes in position in a chair or bed
- use of special mattresses
- cushioning of areas where pressure sores often
develop (sacral prominence and heels)
 to prevent infection of adjacent soft tissue or bone:
- early treatment of ulcers with:
 careful cleansing
 surgical or enzyme debridement of necrotic tissue
 appropriate dressing and drainage
 spasticity
 stretching exercises to maintain mobility of joints
 drug treatment is effective but may result in reduced
function (some patients depend on spasticity as an aid to
stand, transfer, or walk)
 drugs used:
- baclofen (up to 240 mg/d in divided doses)
 effective
 acts by facilitating γ-aminobutyric acid–mediated
inhibition of motor reflex arcs
- diazepam

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