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Anatomy and localization of spinal cord disorders

INTRODUCTION — Because it is the primary pathway of communication between the


brain and peripheral nervous system, diseases that affect the spinal cord are clinically eloquent.
Many of these disease processes have a predilection for targeting specific areas or tracts within
the spinal cord. As a result, knowledge of spinal cord anatomy and recognition of typical common
spinal cord syndromes are useful in the evaluation of a patient with a myelopathy and can allow
for a more directed diagnostic evaluation.

SPINAL CORD ANATOMY — There are 31 spinal cord segments, each with a pair of ventral
(anterior) and dorsal (posterior) spinal nerve roots, which mediate motor and sensory function,
respectively. The ventral and dorsal nerve roots combine on each side to form the spinal nerves as
they exit from the vertebral column through the neuroforamina

Longitudinal organization — The spinal cord is divided longitudinally into four regions: the
cervical, thoracic, lumbar, and sacral cord. The spinal cord extends from the base of the skull and
terminates near the lower margin of the first lumbar vertebral body (L1). Below that level, the
spinal canal contains the lumbar, sacral, and coccygeal spinal nerve roots that comprise the cauda
equina.

Because the spinal cord is shorter than the vertebral column, vertebral and spinal cord segmental
levels are not necessarily the same. The C1 through C8 spinal cord segments lie between the C1
through C7 vertebral levels. The T1 through T12 cord segments lie between T1 through T8. The
five lumbar cord segments are situated at the T9 through T11 vertebral levels, and the S1 through
S5 segments lie between T12 to L1. The C1 through C7 nerve roots emerge above their respective
vertebrae; the C8 nerve root emerges between the C7 and T1 vertebral bodies. The remaining
nerve roots emerge below their respective vertebrae

Cervical cord — The first cervical vertebra (the atlas) and the second cervical vertebra (the axis),
upon which the atlas pivots, support the head at the atlanto-occiput junction. The interface
between the first and second vertebra is called the atlanto-axis junction.

Cervical spinal segments innervate the skin and musculature of the upper extremity and
diaphragm

 C3 through C5 innervate the diaphragm, the chief muscle of inspiration, via the phrenic
nerve
 C4 through C7 innervate the shoulder and arm musculature
 C6 through C8 innervate the forearm extensors and flexors
 C8 through T1 innervate the hand musculature

Thoracic cord — The thoracic vertebral segments are defined by those that have an attached rib.
The spinal roots form the intercostal nerves that run along the inferior rib margin and innervate
the associated dermatomes, as well as the intercostal abdominal wall musculature. These muscles
are the main muscles of expiration. The thoracic cord also contains the sympathetic nerves that
innervate the heart and abdominal organs.

Lumbosacral cord — The lumbosacral spinal cord contains the segments that innervate the
muscles and dermatomes of the lower extremity, as well as the buttocks and anal regions Sacral
nerve roots S3 through S5 originate in the narrow terminal part of the cord, called the conus
medullaris.

 L2 and L3 mediate hip flexion


 L3 and L4 mediate knee extension
 L4 and L5 mediate ankle dorsiflexion and hip extension
 L5 and S1 mediate knee flexion
 S1 and S2 mediate ankle plantar flexion

Sacral nerve roots also provide parasympathetic innervation of pelvic and abdominal organs,
while lumbar nerve roots L1 and L2 contain sympathetic innervation of some pelvic and
abdominal organs.

Cauda equina — In adults, the spinal cord ends at the level of the first or second lumbar vertebral
bodies. The filum terminale, a thin connective tissue filament that descends from the conus
medullaris with the spinal nerve roots, is connected to the third, fourth, and fifth sacral vertebrae;
its terminal part is fused to the periosteum at the base of the coccygeal bone.

Pathology at the T12 and L1 vertebral level affects the lumbar cord. Injuries to L2 frequently
damage the conus medullaris. Injuries below L2 usually involve the cauda equina and represent
injuries to spinal roots rather than to the spinal cord

Cross-sectional anatomy — The spinal cord contains the gray matter, the butterfly-shaped central
region, and the surrounding white matter tracts. The spinal cord gray matter, which contains the
neuronal cell bodies, is made up of the dorsal and ventral horns, each divided into several laminae
[1,2].

Dorsal horn — The dorsal horn is the entry point of sensory information into the central nervous
system. It is divided into six layers or laminae that process sensory information. More than a
relay station for the transmission of sensory information, the dorsal horn also modulates pain
transmission through spinal and supraspinal regulatory circuits. Three major categories of sensory
input that are important to the clinical examination of spinal cord pathology include:

 Afferents from muscle spindles that participate in spinal cord reflexes.


 Axons, mostly small and unmyelinated, mediating sensory modalities of pain and
temperature. These can travel up and down a few segments before synapsing with the
second order neurons, which then cross the midline of the cord in the anterior
commissure, just anterior to the central canal, and then enter the contralateral anterior or
lateral spinothalamic tract.
 Axons mediating the sensory modalities of proprioception, vibration, and touch
discrimination. These large myelinated fibers pass through the dorsal horn to enter the
ipsilateral dorsal column.

Ventral horn — The motor nuclei of the spinal cord are contained within the ventral horn, which
also contains interneurons mediating information from other descending tracts of the pyramidal
and extrapyramidal motor systems. These ultimately synapse on the alpha and gamma motor
neurons, which subsequently leave the ventral horn via the ventral nerve root to terminate at the
neuromuscular junction.
White matter tracts — The major white matter tracts of clinical importance in the assessment of
spinal cord disease include:

 The dorsal or posterior columns, the fasciculus gracilis, and the fasciculus cuneatus.
These contain sensory information regarding joint position and vibration. They are
organized anatomically such that cervical sections lie most laterally and sacral segments
most medially (figure 7). These pathways will cross in the medulla; hence, in the spinal
cord, these tracts contain ipsilateral sensory representation.
 The anterior and lateral spinothalamic tracts contain sensory information regarding pain,
temperature, and touch. These axons have crossed in the ventral commissure and
therefore contain contralateral sensory representation. This tract is somatotopically
organized with cervical inputs located most medially and sacral inputs most laterally
(figure 7).
 The corticospinal tracts contain the upper motor neurons that originate in M1 of the
primary motor cortex. These axons synapse either directly or indirectly on the anterior
horn cells, and as such have distinct sites of anatomic origin within M1 [3]. A single
corticomotoneuronal axon synapses with many anterior horn cells of its own motor
neuron pool and also with those of agonists and antagonists, allowing for coordination of
highly skilled movements.

The lateral corticospinal tract contains the majority (80 to 85 percent) of these fibers, which have
previously decussated (crossed) at the cervicomedullary junction and therefore provide input to
the ipsilateral musculature. Fibers are somatotopically organized within the tract such that fibers
destined for upper extremity motor control lie most medially, while fibers controlling the lower
extremity lie more laterally (figure 7). The anterior corticospinal tract contains undecussated
fibers, some of which will subsequently cross at the spinal level through the anterior commissure.

Other descending tracts include:

 The tectospinal tract originates in the superior colliculus and mediates reflex postural
movements of the head in response to visual and/or acoustic input
 The rubrospinal tract originates in the red nucleus and controls tone of ipsilateral flexor
muscle groups
 The vestibulospinal tracts arise from the vestibular nuclei and facilitate spinal cord
reflexes and muscle tone to maintain posture
 The reticulospinal tracts originate in the brainstem and provide facilitatory input to
muscle groups as well as input to spinal autonomic fibers

Other ascending tracts include:

 The dorsal and ventral spinocerebellar tracts carry inputs mediating unconscious
proprioception directly to the cerebellum
 The spinoreticular tract carries deep pain input to the reticular formation of the brainstem

Autonomic fibers — Autonomic fibers of hypothalamic and brainstem origin descend in the
lateral aspect of the spinal cord but not in a well-defined tract. These synapse with cell bodies in
the intermediolateral columns of the central gray matter of the spinal cord. Sympathetic fibers
exit between T1 and L2, and parasympathetic fibers exit between S2 and S4.
The sympathetic neurons lie in the lateral horn of the central gray matter at spinal levels T1-L3.
The preganglionic fibers exit via the ventral root, spinal nerve, and ventral ramus to reach the
paravertebral ganglion. Many will synapse at the paravertebral ganglion, others pass through it to
terminate on postganglionic neurons (eg, coeliac, superior mesenteric, and inferior mesenteric
ganglia) more proximate to their end organ.

Parasympathetic neurons originate in the sacral spinal cord and exit the spinal cord with other
efferents to the ventral ramus. After leaving the ventral ramus, they may subsequently join with
sympathetic nerves to reach the viscera. These preganglionic fibers then synapse with a diffuse
network of terminal ganglion cells that affect organs in the pelvis.

Autonomic dysfunction is an important determinant of site, extent, and severity of spinal cord
pathology. Many autonomic functions can be affected by spinal cord pathology, but for clinical
evaluation, the most useful symptoms relate to bladder control.

Autonomic bladder control is primarily parasympathetic, and is unaffected by isolated injury to


the sympathetic fibers. Voluntary bladder control is under somatomotor control, mediated by
motor fibers originating from the anterior horn cells at levels S2-S4. A spinal cord lesion that
interrupts descending motor and autonomic tracts above the S2 level produces an "automatic
bladder" that cannot be emptied voluntarily, but empties reflexly when expanded to a certain
degree, the so-called neurogenic bladder [4-7]. Loss of descending inhibition of segmental reflex
control leads to urinary urgency and incontinence. Injury to S2-S4 spinal levels interrupts the
bladder reflex circuit; the bladder becomes flaccid, and fills beyond capacity with overflow
incontinence.

Other autonomic functions are disturbed by spinal cord pathology. The effects of spinal cord
injury on the colon and rectum are similar to those on the bladder. Spinal cord transections
interrupt voluntary control of the external sphincter and produce constipation. Sacral lesions
cause a loss of the anal reflex and rectal incontinence. Impotence can result from spinal cord
lesions at any level. Spinal cord injuries can also affect cardiovascular function, most
dramatically with lesions above T6 which can produce a phenomenon of autonomic dysreflexia.

Blood supply — A single anterior and two posterior spinal arteries supply the spinal cord (figure
8). The anterior spinal artery supplies the anterior two-thirds of the cord [8-12]. The posterior
spinal arteries primarily supply the dorsal columns. The anterior and posterior spinal arteries arise
from the vertebral arteries in the neck and descend from the base of the skull. Various radicular
arteries branch off the thoracic and abdominal aorta to provide additional blood supply to the
spinal arteries. The largest and most consistently present of these radicular branches is the great
ventral radicular artery or the artery of Adamkiewicz, which supplies the anterior spinal artery
[13]. This artery enters the spinal cord anywhere between T5 and L1 (usually between T9 and
T12).

In most people, the anterior spinal artery passes uninterrupted along the entire length of the spinal
cord; in others, it is discontinuous, usually in its midthoracic segment, making these individuals
more susceptible to vascular injury. The primary watershed area of the spinal cord in most people
is in the midthoracic region.

CLINICAL LOCALIZATION — A spinal cord lesion may be suspected when there are
bilateral motor and sensory signs or symptoms that do not involve the head or face. Motor deficits
are manifest by weakness and long tract signs (spasticity, increased reflexes, Babinski sign) [5,14-
16]. When the pathology is localized or segmental, these findings will be present in muscle
groups innervated below that level and will be normal above. A sensory level, with normal
sensation above and reduced or absent below, can also often be defined and should be specifically
sought. Other so-called segmental signs include lower motor neuron findings (atrophy, flaccid
weakness, loss of reflexes) in a myotomal distribution at the specific level of involvement;
however, these are usually not elicitable in thoracic lesions

As well as longitudinal localization within the spinal cord, it can also be helpful to distinguish
specific areas of functional loss with a spinal cord level (or across spinal cord levels for
nonsegmental pathologies). Some disorders affecting the spinal cord preferentially affect different
structures, and therefore careful testing of all spinal cord functions, including motor, reflex, and
all sensory modalities, and sphincter function is important for clinical localization.

Several distinct spinal cord syndromes are recognized. These are useful in the clinical evaluation,
as they often correspond to distinct pathologies. These are summarized in the table and are
discussed below (table 1).

Segmental syndrome — Pathologies that affect all functions of the spinal cord at one or more
levels produce a segmental syndrome. Loss of function may be total or incomplete. A total cord
transection syndrome results from the cessation of function in all ascending and descending
spinal cord pathways and results in the loss of all types of sensation and loss of movement below
the level of the lesion. Less profound injuries produce a similar pattern of deficits, which are less
severe: ie, weakness rather than paralysis and decreased sensation rather than anesthesia.

Acute transection can cause spinal shock, with a flaccid paralysis, urinary retention, and
diminished tendon reflexes. This is usually temporary, and increased tone, spasticity, and
hyperreflexia will usually supervene in days or weeks after the event.

Transverse injuries above C3 involve cessation of respiration and are often fatal if acute. Cervical
cord lesions that spare the phrenic nerve but impair intercostal nerve function can produce
respiratory insufficiency. Lesions above the L2 cord level will cause impotence and spastic
paralysis of bladder. There is loss of voluntary control of the bladder, which will empty
automatically by reflex action.

Causes of a cord segmental syndrome include acute myelopathies, such as traumatic injury and
spinal cord hemorrhage. Epidural or intramedullary abscess, tumors, and transverse myelitis may
have a more subacute presentation.

Dorsal (posterior) cord syndrome — Dorsal cord syndrome results from the bilateral involvement
of the dorsal columns, the corticospinal tracts, and descending central autonomic tracts to bladder
control centers in the sacral cord (figure 9). Dorsal column symptoms include gait ataxia and
paresthesias. Corticospinal tract dysfunction produces weakness that, if acute, is accompanied by
muscle flaccidity and hyporeflexia and, if chronic, by muscle hypertonia and hyperreflexia.
Extensor plantar responses and urinary incontinence may be present.

Causes of a dorsal cord syndrome include multiple sclerosis (more typically the primary
progressive form), tabes dorsalis, Friedreich ataxia, subacute combined degeneration, vascular
malformations, epidural and intradural extramedullary tumors, cervical spondylotic myelopathy,
and atlantoaxial subluxation.
Ventral (anterior) cord syndrome — Ventral cord or anterior spinal artery syndrome usually
includes tracts in the anterior two-thirds of the spinal cord, which include the corticospinal tracts,
the spinothalamic tracts, and descending autonomic tracts to the sacral centers for bladder control
(figure 10). Corticospinal tract involvements produce weakness and reflex changes. A
spinothalamic tract deficit produces the bilateral loss of pain and temperature sensation. Tactile,
position, and vibratory sensation are normal. Urinary incontinence is usually present.

The causes of a ventral cord syndrome include spinal cord infarction, intervertebral disc
herniation, and radiation myelopathy

Brown-Sequard (hemi-cord) syndrome — A lateral hemisection syndrome, also known as the


Brown–Sequard syndrome, involves the dorsal column, corticospinal tract, and spinothalamic
tract unilaterally (figure 11). This produces weakness, loss of vibration, and proprioception
ipsilateral to the lesion and loss of pain and temperature on the opposite side. The unilateral
involvement of descending autonomic fibers does not produce bladder symptoms. While there are
many causes of this syndrome, knife or bullet injuries and demyelination are the most common.
Rarer causes include spinal cord tumors, disc herniation, infarction, and infections.

Central cord syndromes — The central cord syndrome is characterized by loss of pain and
temperature sensation in the distribution of one or several adjacent dermatomes at the site of the
spinal cord lesion caused by the disruption of crossing spinothalamic fibers in the ventral
commissure (figure 12). Dermatomes above and below the level of the lesion have normal pain
and temperature sensation, creating the so-called "suspended sensory level." Vibration and
proprioception are often spared.

As a central lesion enlarges, it may encroach on the medial aspect or the corticospinal tracts or on
the anterior horn gray matter, producing weakness in the analgesic areas. Fibers mediating the
deep tendon reflexes are interrupted as they pass from the dorsal to the ventral horn, thus causing
tendon reflex loss in the analgesic areas. There are usually no bladder symptoms.

The classic causes of a central cord syndrome are slow-growing lesions such as syringomyelia or
intramedullary tumor. However, central cord syndrome is most frequently the result of a
hyperextension injury in individuals with long-standing cervical spondylosis. This form of central
cord syndrome is characterized by disproportionately greater motor impairment in upper
compared with lower extremities, bladder dysfunction, and a variable degree of sensory loss
below the level of injury [

Pure motor syndrome — A pure motor syndrome produces weakness without sensory loss or
bladder involvement. This may involve only the upper motor neurons, producing hyperreflexia
and extensor plantar responses, or only the lower motor neuron bilaterally, producing muscle
atrophy and fasciculations. Other disorders involve both the upper and lower motor neurons and
produce mixed signs.

The causes of a pure motor syndrome include chronic myelopathies such as HTLV-I myelopathy,
hereditary spastic paraplegia, primary lateral sclerosis, amyotrophic lateral sclerosis, progressive
muscular atrophy, post-polio syndrome, and electric shock-induced myelopathy.

Conus medullaris syndrome — Lesions at vertebral level L2 often affect the conus medullaris.
There is early and prominent sphincter dysfunction with flaccid paralysis of the bladder and
rectum, impotence, and saddle (S3-S5) anesthesia. Leg muscle weakness may be mild if the
lesion is very restricted and spares both the lumbar cord and the adjacent spinal and lumbar nerve
roots.

Causes include disc herniation, spinal fracture, and tumors [4,20].

Cauda equina syndrome — Though not a spinal cord syndrome, cauda equina syndrome is
considered here because its location within the spinal canal subjects it to many of the same
disease processes that cause myelopathy. The syndrome is caused by the loss of functions of two
or more of the 18 nerve roots constituting the cauda equina. Deficits usually affect both legs but
are often asymmetric. Symptoms include [21-23]:

 Low back pain accompanied by pain radiating into one or both legs. Radicular pain
reflects involvement of dorsal nerve roots and may have localizing value [21].
 Weakness of plantar flexion of the feet with loss of ankle jerks occurs with mid cauda
equina lesions, involving S1, S2 roots. Involvement of progressively higher levels leads
to corresponding weakness in other muscles (figure 5).
 Bladder and rectal sphincter paralysis usually reflect involvement of S3-S5 nerve roots
[21,22].
 Sensory loss of all sensory modalities occurs in the dermatomal distribution of the
affected nerve roots (figure 6).

Many etiologies can cause a cauda equina syndrome, including intervertebral disc herniation,
epidural abscess, epidural tumor, intradural extramedullary tumor, lumbar spine spondylosis, and
a number of inflammatory conditions including spinal arachnoiditis, chronic inflammatory
demyelinating polyneuropathy, and sarcoidosis [20,24-29]. The cauda equina can also be the
primary site of involvement in carcinomatous meningitis and a number of infections (eg,
cytomegalovirus, herpes simplex virus, herpes zoster virus, Epstein Barr virus, Lyme disease,
mycoplasma, and tuberculosis).

Lhermitte's sign — This well-described sign describes a sensation of electric shock-like


sensations that run down the back and/or limbs during flexion of the neck. This generally occurs
with pathologies involving the cervical spinal cord, but is not specific to etiology, occurring in
patients with cervical spondylotic myelopathy [30], multiple sclerosis, radiation myelopathy, and
vitamin B12 deficiency, among others. It can also occur with cervical nerve root pathology.

DIAGNOSIS — The differential diagnosis of myelopathy is wide, but can be significantly


narrowed by the clinical syndrome (table 1). Other features in the examination and history also
limit the differential diagnosis and tailor the diagnostic work-up. Clinical features of some of the
more common causes of myelopathy are outlined in the Table (table 2). These are discussed in
detail separately

For patients with a clinical syndrome that suggests a localized process within the spinal cord (eg,
transection syndrome, central cord syndrome, ventral cord syndrome, etc), an imaging study,
usually magnetic resonance imaging (MRI), of the relevant section of the spinal cord is usually
required [16,31]. Administration of gadolinium contrast is often helpful. When an infectious or
inflammatory disorder is suspected, cerebrospinal fluid examination may be helpful.

In general, the pace at which spinal cord deficits appear dictate the urgency of the neurologic
evaluation. Even when the deficits are not severe, acute myelopathic signs need to be evaluated
urgently because neurologic deterioration can occur abruptly, and the clinical deficit at the time of
intervention often dictates the chances of recovery. This is true particularly for compressive
etiologies such as spinal cord metastases and epidural spinal abscess.

1. SUMMARY — Disorders that affect the spinal cord often target specific structural and
functional anatomic regions, producing distinct clinical syndromes. The spinal cord
syndromes are summarized in the table (table 1). The clinical syndrome along with other
features in the examination and history usually significantly limits the differential
diagnosis and tailors the diagnostic work-up (table 2).

Important causes of spinal cord dysfunction*


Age Course Clinical features Diagnosis
Moderate-severe cases
Cervical demonstrate gait and leg
Usually >60 Progressive or
spondylotic spasticity and MRI cervical spine
years stepwise course
myelopathy amyotrophy of hand or
arms
Transverse Children, young
Subacute Segmental cord syndrome MRI and CSF
myelitis adults
Pure motor syndrome or
Viral myelitis Any age Acute-subacute MRI and CSF
Segmental cord syndrome
Epidural Subacute; may
Any age Segmental cord syndrome MRI
abscess worsen abruptly
Usually >60 MRI with diffusion
Infarction Abrupt onset Anterior cord syndrome
years weighted sequences
>40 years (dural
fistula)
Vascular Acute and/or MRI, spinal
Radicuomyelopathy
malformation 20's stepwise angiography
(intramedullary
AVM)
Subacute
Slowly
combined Any age Dorsal cord syndrome Vitamin B12 levels
progressive
degeneration
Slowly
progressive;
beginning 6-12 Segmental cord syndrome MRI, clinical
Radiation Any age
months after or Ventral cord syndrome history
radiation
therapy
Children, young Slowly
Syringomyelia Central cord syndrome MRI
adults progressive
Epidural Usually >50 Subacute, may
Segmental cord syndrome MRI
metastasis years worsen abruptly
MRI with
Intramedullary Slowly
Young adults Central cord syndrome gadolinium
tumor progressive
enhancement
Usually >60 Slowly
ALS Pure motor syndrome Electromyography
years progressive