Neurology in Practice
THE BARE ESSENTIALS
Correspondence to
Professor Lionel Ginsberg,
Department of Neurology, Royal
Free Hospital, Pond Street,
London NW3 2QG, UK;
Lionel.Ginsberg@nhs.net
2011;11:259–267. doi:10.1136/practneurol-2011-000069
Disorders of the spinal cord
and roots
Lionel Ginsberg
INTRODUCTION
The distinctive patterns of myelopathy (disorder
of the spinal cord) and radiculopathy (disorder of
spinal roots) are a direct consequence of the strik-
ing anatomy of the spinal cord:
▶ its near cylindrical, segmental structure of
great length (42–45 cm in adults)
▶ the marked proximity of ascending and
descending long tracts within the confi nes of
a narrow cross sectional area (the maximum
circumference of the cervical enlargement of
the cord is approximately 38 mm)
▶ enclosure by meninges and vertebral column
▶ vulnerable blood supply.
Having established that a patient’s clinical pre-
sentation localises to the spinal cord and/or roots,
clues to the pathological diagnosis emerge from
the timing of the symptoms (table 1), as is usually
the case in neurology.
NEUROANATOMY AND SPECIFIC SYNDROMES
Spinal cord
The relationships of the white matter tracts to one
another and to the central grey matter are most
easily appreciated in transverse section (figure 1).
Within the major pathways, there is a relatively
orderly somatotopic arrangement:
▶ In the spinothalamic tracts, the most super-
ficial fibres are related to the sacral der-
matomes. These overlie the lumbar fibres,
with thoracic still deeper in the tracts, and
the cervical fibres closest to the central grey
matter. Pain and temperature sensation are
conveyed in the lateral spinothalamic tracts
whereas touch and pressure pathways are
anterior (ventral).
▶ In the posterior (dorsal) columns, fi bres from
the lower limbs ascend medially in the grac-
ile tracts, those from the upper limbs are lat-
eral in the bulkier cuneate tracts. Pathways
for position and touch sensation generally
lie deep to those for vibration and pressure.
▶ In the lateral corticospinal tracts, descending
fibres to the upper limbs are medial to those
to the lower limbs.
The organisation is similar in the central grey
matter of the cord where the neuronal cell
bodies are arranged in 10 layers or ‘nuclei’ (Rexed’s
laminae):
▶ The laminae in the dorsal horns are where the
primary cutaneous afferent fibres terminate
having reached the cord via the dorsal roots
of the spinal nerves. They then synapse with
second order neurons in the substantia gelati-
nosa of the dorsal horns, directly or through
interneurons. These second order neurons
decussate in the ventral white commissure and
ascend in the contralateral spinothalamic tract
(this pathway may be contrasted with that for
proprioceptive and exteroceptive information
ascending in the dorsal columns where the
central axons of the primary afferents remain
ipsilateral and may not synapse until reaching
the gracile and cuneate nuclei in the medulla).
▶ The lateral horns of the central grey matter are
small projections in the thoracolumbar cord
(T1 to L2) which contain the cell bodies of
preganglionic sympathetic neurons.
▶ The ventral (anterior) horns are much larger
and contain the cell bodies of lower motor
neurons. Anterior horn cells are arranged so
those whose axons are destined to innervate
flexor muscles lie dorsal to those supplying
the extensors; lower motor neurons to the
limb muscles originate lateral to those des-
tined for the truncal musculature in the ven-
tral horns.
These microscopic arrangements and relation-
ships all have clinical relevance when specific cord
syndromes are considered:
Extrinsic compression
A lesion gradually compressing the spinal cord
can produce weakness below the level of the
lesion (spastic paraparesis, spastic tetraparesis, or
upper motor neuron signs in the legs with a com-
bination of upper and lower motor neuron signs
in the arms), sphincter disturbance and sensory
loss, ultimately to all modalities. There may be
local or radicular pain. The area of cutaneous
sensory impairment typically includes the sacral
dermatomes because the fibres from this area lie
closest to the surface of the cord in the spinotha-
lamic tracts and are therefore most vulnerable to
extrinsic compression.
259
 Neurology in Practice
Table 1 Speed of onset and likely cause of myeloradiculopathy
Onset Examples of pathophysiology
Hyperacute ...seconds, minutes, hours... Traumatic
Vascular (haemorrhagic or ischaemic)
Acute ...hours, days, weeks... Compressive (abscess, tumour, intervertebral disc)*
Inflammatory (including infective and post-
infective)†
Subacute ...days, weeks, months... Metabolic (eg, B12 deficiency)
Compressive
Inflammatory
Chronic ...months, years, decades... Compressive (eg, spondylotic)
Inflammatory (eg, HTLV-1 infection, primary
progressive multiple sclerosis)
Heredo-degenerative
Syringomyelia
*Compression from abscess, tumour or disc may also be subacute or acute-on-chronic.
†
Non-infective inflammatory lesions are usually subacute or chronic in onset, but can occasionally strike
abruptly, as in ‘stroke-like’ presentations of multiple sclerosis.
Figure 1 Transverse section of the spinal cord in the mid-cervical region. Many
ascending and descending tracts have been omitted to emphasise the most clinically
relevant.
This pattern is the opposite of that in intrinsic
cord lesions, where there may be sacral sparing.
The upper limit of the impaired sensation—the
sensory level—may correspond to the level of the
compressive lesion, but not always. Thus a sensory
level at T10, for example, implies that the lesion is
at T10 or above. The explanation is again related
to the lamination of the spinothalamic tracts.
Early in its evolution, a compressive lesion at the
cervicothoracic junction (C8/T1), for example,
may only cause relatively mild cord impingement,
damaging the outermost layers of the spinotha-
lamic tracts—that is, those relating to the sacral
and lumbar dermatomes. In time, however, as the
compression worsens and deeper fibres within the
tracts become affected, the sensory level ascends
towards where the lesion actually is—at T1. The
upper level of the sensory disturbance therefore
depends on the stage of evolution of the lesion.
Indeed patients may describe this process in their
history, saying that the numbness began in their
feet, gradually ascended to the knees and even-
tually to their trunk. This evolving pattern is of
great practical importance in the investigation
and management of acute cord lesions. If a patient
with a sensory level at T10 has only the lower
260
thoracic cord imaged, a surgically treatable lesion
further up the spine may be missed.
Acute complete transverse lesions
Acute complete transverse lesions produce an
extreme version of the pattern seen with extrin-
sic cord compression of more gradual onset; com-
plete paralysis and sensory loss to all modalities
below the level of the lesion, and loss of sphincter
control. Immediately after the injury, however,
there is a period of spinal shock typically lasting
1–2 weeks or sometimes longer when the motor
signs are paradoxical, with fl accid paralysis and
absent tendon reflexes. This is why acute cord
lesions enter the differential diagnosis of the
Guillain–Barré syndrome. Upper motor neuron
signs only develop after spinal shock has resolved.
A similar sequence of events is seen with sphinc-
ter function after severe spinal cord injury, with
an initially atonic bladder and bowel, later becom-
ing spastic. Acute complete transverse lesions can
be traumatic or non-traumatic (eg, vascular).
Central cord syndrome
Some patients with limited trauma to the cer-
vical cord may have disproportionate damage
to the central grey matter, with relative sparing
of the long tracts; the dominant clinical fi nd-
ings are then of lower motor neuron signs in the
arms, with variable sensory loss. Mild, reversible
trauma, often in the context of pre-existing cervi-
cal spinal stenosis, may result in numb, clumsy or
‘burning’ hands without weakness or long tract
features, the pathology presumably residing in or
near the dorsal horns.
A similar pattern, albeit much more chronic,
is seen in syringomyelia where a cavity develops
within the cord, usually starting close to the cer-
vicothoracic junction and gradually extending ros-
trally and caudally, in association with a Chiari
malformation. The cavity affects the decussating
spinothalamic fibres in the ventral white com-
missure, producing a characteristic suspended or
cape-like loss of pain and temperature sensation,
with upper and lower sensory levels. Eventually,
the long tracts are involved, so the patient has a
combination of lower motor neuron signs in the
upper limbs and upper motor neuron signs in the
lower limbs, but a degree of dissociated anaesthe-
sia persists—ie, with sparing of dorsal column
function.
A syrinx may also occur in association with
intramedullary spinal tumours and after trauma
and other causes of extrinsic cord compression.
Ventral cord syndrome
The blood supply of the spinal cord is vulnerable
to hypotension. The anterior spinal artery supplies
the anterior two-thirds of the cord and is a func-
tional end artery. The cord’s longitudinal arteries
(one anterior and two posterior) are insufficient to
provide its entire transverse or longitudinal needs
2011;11:259–267. doi:10.1136/practneurol-2011-000069
 Neurology in Practice

and are supplemented by segmental medullary
feeder vessels. Of these, the largest is the artery of
Adamkiewicz which arises from a segmental ves-
sel of the aorta, usually between T9 and L2 on the
left, and this can be responsible for most of the
supply to the lower cord. The upper to mid tho-
racic cord is distant from this and other feeders,
hence it is at particular risk of ischaemia at times
of hypotension. Anterior spinal artery infarction
produces paraplegia or tetraplegia, loss of pain and
temperature sensation below the lesion, with rel-
ative sparing of dorsal column function (because
the posterior one-third of the cord is supplied by
the posterior spinal arteries).
cervical cord lesions sometimes produce physical
signs in the arms which suggest lower motor neu-
ron involvement, with distal wasting and weak-
ness, and depressed tendon reflexes.
Spinal roots
The ventral and dorsal roots at each spinal level
unite to form mixed (sensory and motor) spinal
nerves. The point of union is in or close to the
intervertebral foramen. Spinal nerves and roots
are numbered according to the adjacent vertebrae
(figure 2). Thus in the cervical region, the nerve is
the same number as the vertebra below it (eg, the
right and left C7 nerves emerge from the spine

Dorsal cord syndrome

While the dorsal columns can sometimes be
selectively damaged by vascular occlusion, the
more usual pathology (in combination with that
of the lateral corticospinal tracts) is metabolic or
inflammatory (see below).

Hemicord syndrome
The typical features of the Brown–Séquard syn-
drome, when the lesion is confi ned to one side
of the cord, arise because of the decussation of
spinothalamic fibres in the ventral white com-
missure. As a result, corticospinal tract and dor-
sal column function are impaired on the side of
the lesion whereas pain and temperature sen-
sation are affected on the opposite side of the
body. Patients may also have a narrow band of
spinothalamic sensory loss, sometimes accompa-
nied by pain, on the side of the lesion and close to
its level, caused by damage to fibres which have
not yet decussated to join the contralateral spi-
nothalamic tract. Originally described (and still
seen) in the context of trauma, any asymmetrical
cord lesion, such as a plaque of demyelination,
can cause this, albeit often partial.

Conus lesion
A lesion at the lower end of the spinal cord can
produce a mixture of upper and lower motor
neuron signs in the legs, typically absent ankle
reflexes (due to damage to the reflex arc) with
upgoing plantar responses (due to damage to the
corticospinal tract). Weakness in the lower limbs
is mild or absent but there is profound sphincter
disturbance and saddle anaesthesia.

Foramen magnum lesion

Lesions of the high cervical cord can produce con-
fusing symptoms and signs. Weakness may begin
in one arm and then spread in a clockwise or anti-
clockwise fashion to the ipsilateral leg, then the
contralateral leg and fi nally to the contralateral
arm. Lower cranial nerve palsies may be pres-
ent, along with downbeating nystagmus. Pain Figure 2 Diagram showing the relationship of the
and paraesthesiae may affect the upper limb fi rst spinal cord segments and spinal nerves to the vertebrae.
showing motor involvement. For poorly under- (Reproduced with permission from Ginsberg L. Lecture
stood reasons, possibly vascular in basis, high notes on neurology, 9th Edn. Wiley-Blackwell, 2010.)

2011;11:259–267. doi:10.1136/practneurol-2011-000069 261

 Neurology in Practice
at C6/7). The C8 nerves lie between the C7 and
T1 vertebrae so spinal nerves below this level
exit below the vertebrae with which they share
a number. However, a disc prolapse in the lumbar
region will still generally impinge on the root(s)
with the same number as the vertebra below
(because of the three-dimensional arrangement
of the roots in the lower spinal canal). A lateral
disc prolapse at L4/5, for example, will generally
cause an L5 root lesion. It will only involve the
L4 nerve if there is a far lateral protrusion.
A spinal cord segment is defi ned by the zone
of attachment of the rootlets of a pair of spinal
nerves (right and left, dorsal and ventral roots,
6–8 rootlets per root). As the cord occupies only
the upper two-thirds of the spinal canal, there will
necessarily be non-correspondence between the
cord segments and the vertebrae (figure 2). This
divergence becomes more marked the more caudal
the segment, so that the lower roots have a longer
intraspinal course than those arising more rostrally.
Ultimately, with the spinal cord ending around
L1/2, the neural elements in the lowest part of the
theca consist solely of the roots forming the cauda
equina, crowded around the fi lum terminale.
Monoradiculopathies
Monoradiculopathies are usually compressive
but can be infl ammatory or infective. Their
clinical features comprise radicular pain, der-
matomal sensory loss, weakness and some-
times loss of reflex(s). Radicular pain may be
perceived in the relevant myotome, rather than
the dermatome—a useful practical point. For
example, C5 pain may localise medial to the
scapula, in the rhomboids. Testing the strength
of particular muscles may indicate which root is
affected—segment-pointer muscles (table 2).
Polyradiculopathies
Polyradiculopathies may be compressive, inflam-
matory or infi ltrative. A compressive lesion of the
cauda equina (caused by central intervertebral
disc prolapse or tumour) may be indistinguishable
Table 2 Segment-pointer muscles*
Root Muscle(s)
C3 Diaphragm
C4 Diaphragm
C5 Deltoid, biceps
C6 Brachioradialis, extensor carpi radialis longus
C7 Triceps, extensor carpi ulnaris, extensor digitorum
C8 Wrist and finger flexors
T1 Intrinsic muscles of the hand
L3 Quadriceps femoris
L4 Quadriceps femoris, tibialis anterior
L5 Extensor hallucis longus
S1 Gastrocnemius
*Most muscles are innervated by fibres from more than one root.
However, in these examples, only one or two roots predominate for a
particular muscle, aiding lesion localisation. The root values assume
that the plexus is neither pre- nor post-fi xed.
262
from a conus lesion but there will be no upper
motor neuron signs. Cauda equina syndrome is
also typically more painful than a conus lesion,
and more likely to cause lower limb weakness.
CAUSES OF MYELOPATHY
(AND RADICULOPATHY)
Spinal cord and root disease may fi rst be divided
into extrinsic (usually compressive) and intrinsic
causes, then the familiar ‘surgical sieve’ can be
applied (table 3).
Genetic
▶ Hereditary spastic paraplegia is a neurode-
generative process primarily affecting upper
motor neurons, rather than a disease of the
spinal cord per se. It can resemble a ‘true’
myelopathy clinically, however, even in its
relatively uncomplicated forms where there
may be sphincter and sensory involvement.
The pattern of inheritance is most commonly
autosomal dominant but can be autosomal
recessive or sex linked.
▶ Adrenomyeloneuropathy is one presentation
of X linked adrenoleukodystrophy. Males
(and occasionally female manifesting carriers)
develop a slowly progressive spastic parapare-
sis in young adulthood, followed by sensory
and sphincter involvement. Adrenal failure
need not occur. Raised very long chain fatty
acid levels are diagnostic in males but muta-
tion analysis may be required in females.
▶ Other leukodystrophies, for example, metach-
romatic leukodystrophy, can have prominent
upper motor neuron features, but the pres-
ence of cognitive deterioration and brain MRI
changes usually help clarify the diagnosis.
▶ Some of the hereditary ataxias may occasion-
ally present with a predominant spastic para-
paresis. In particular, variant patients with
Friedreich’s ataxia may have lower limb spastic-
ity, pyramidal weakness and retained reflexes.
Similarly, some patients with spinocerebellar
ataxia type 3 (Machado–Joseph disease) have
clinical features resembling hereditary spastic
paraplegia early in the course of their illness.
▶ Genetic disorders can also cause extrinsic
compressive cord lesions, for example, in the
mucopolysaccharidoses where the metabolic
defect may lead to spinal dysostosis, dural
thickening or atlanto-axial instability.
Congenital (ie, starting during fetal development)
▶ Syringomyelia is probably congenital, in asso-
ciation with a Chiari malformation.
▶ Spinal dysraphism varies in severity from the
chance radiographic fi nding of spina bifida
occulta to a full blown lumbar myelomenin-
gocoele with consequent lower cord/cauda
equina syndrome and neonatal hydrocephalus.
Adults presenting with a partial conus or cauda
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 Neurology in Practice

Table 3 Causes of myelopathy Infective

Extrinsic Mixed or indeterminate Intrinsic
Extrinsic infective processes compressing the cord or
cauda equina include epidural abscess, usually due
Genetic Mucopolysaccharidoses Hereditary spastic to Staphylococcus aureus (sometimes streptococci
paraplegia
Adrenomyeloneuropathy and anaerobes). The clinical presentation is with
Other spinocerebellar the triad of pain (local, with tenderness and/or
degenerations and radicular), fever and signs of myeloradiculopathy.
leukodystrophies
Tuberculous spinal osteomyelitis (figure 3A) is
Congenital Arachnoid cyst Syringomyelia
Dysraphism encountered particularly in at risk groups (those
Traumatic Vertebral fracture and/or in or from developing countries, or immunocom-
dislocation promised individuals) and leads to neural com-
Missile and stab injuries pression which may be associated with spinal
Infective Epidural abscess Acute viral myelitis deformity, ultimately the angular gibbus of Pott’s
Tuberculous osteomyelitis Tuberculosis
Syphilis disease of the spine.
Schistosomiasis Intrinsic infective myelopathies can be acute or
HIV chronic:
HTLV-1
▶ Acute infective myelitis is less common in
Inflammatory Rheumatoid arthritis Multiple sclerosis
Ankylosing spondylitis Neuromyelitis optica developed countries than a post-infective pro-
Post-infective cess (see below). It may be viral (zoster, her-
Sarcoid, Behçet’s pes simplex, HIV at seroconversion), bacterial
Connective tissue
disorders (tuberculosis, secondary syphilis) or, rarely,
Neoplastic Extradural tumours Intramedullary tumours parasitic (schistosomiasis).
Extramedullary intradural Paraneoplastic ▶ Chronic infective myelopathies include the
tumours vacuolar myelopathy of advanced HIV infec-
Vascular Epidural haematoma Dural arteriovenous Haematomyelia
fistula Ischaemic infarction
tion. Another chronic retroviral myelopathy
Metabolic Paget’s disease Vitamin B12 deficiency
is associated with human T cell lymphotropic
Copper deficiency virus infection (tropical spastic paraparesis,
Liver failure human T lymphotropic virus type 1 associ-
Toxic Myodil induced arachnoiditis Radiation Nitrous oxide ated myelopathy) which is endemic in the
Intrathecal methotrexate Electrical injury Clioquinol
Lathyrism
Caribbean, sub-Saharan Africa and the Far
Konzo East. Transmission is vertical, sexual or
Degenerative Spondylosis Amyotrophic lateral through blood transfusion. Only a small
Disc prolapse sclerosis minority of infected individuals develop neu-
Primary lateral sclerosis rological problems following an incubation
period of many years. The clinical picture is
equina syndrome in association with devel- of a progressive spastic paraparesis (usually
opmental lumbosacral skin changes (dimples, slow) with prominent sphincter involvement
pits, sinuses, lipomas, haemangiomas or tufts and painful paraesthesiae in the legs.
of hair) may have a variety of underlying spi- Infective radiculopathies include herpes zoster
nal defects, for example, tethering of the cord, and also the meningoradiculopathy of Lyme dis-
or a lumbar syrinx or lipoma. Further up the ease. A polyradiculitis of the cauda equina caused
spine, a bony spur can separate the cord into by cytomegalovirus occurs in AIDS patients with
two halves—diastematomyelia. very low CD4 cell counts.
▶ Arachnoid cysts are also probably congenital.
Although usually asymptomatic, they can Inflammatory and demyelinating
occasionally cause chronic cord compression, Multiple sclerosis (MS) is the most common cause
sometimes with secondary syrinx formation. of a spastic paraparesis in young adults in tem-
perate zones. Although a complete transverse
Traumatic myelitis may occur, the clinical features of a cord
Missile and stab injuries to the spine are encoun- relapse of MS are typically asymmetrical, reflect-
tered in civilian life but the more common trauma ing the likely irregular disposition of the causative
is crushing of the cord consequent on fracture– plaques. The Lhermitte phenomenon is helpful
dislocation of the spine, potentially exacerbated by in the diagnosis of MS, but not pathognomonic,
hyperflexion/hyperextension and by any pre-exist- because it also occurs in compressive cervical cord
ing narrowing of the spinal canal. Such injuries usu- lesions and vitamin B12 deficiency. The Uhthoff
ally transect the cord with instantly fatal results if phenomenon is also useful, but may be more subtle
the lesion is in the high cervical region, unless there than the patient simply complaining of worsening
is immediate respiratory support (the respiratory symptoms after a hot bath. Thus patients with
muscles are innervated by motor fibres arising from spinal cord demyelination may describe exercise
C3 to the lower thoracic cord). More caudally, the induced deterioration in their walking, mimicking
degree of paralysis depends on the level of the lesion vascular or compressive spinal lesions.
but there will generally be a phase of spinal shock Theoretically, there is no limit to the length of a
followed by the development of hyperreflexia. spinal plaque of MS. However, very long intrinsic

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 Neurology in Practice

Figure 3 Spinal MRI. (A) Tuberculous spinal osteomyelitis and discitis—sagittal T2 weighted image of the thoracic spine showing destruction of
the T8 vertebral body and extradural compression of the spinal cord. (B) Neuromyelitis optica—sagittal T2 weighted image of the cervicothoracic
spine showing longitudinally extensive transverse myelitis, involving much of the cord caudally from C5. (C) Spinal meningioma—sagittal T1 weighted
image of the thoracic spine post-gadolinium. The lesion was isointense with the spinal cord on the pre-contrast images but can be seen to enhance
avidly post-gadolinium. (D) Spondylotic compression at C3/4—sagittal T2 weighted image of the cervicothoracic spine. There is anterior and posterior
cord impingement, resulting in damage to its parenchyma (myelomalacia), shown as high signal within cord substance at the point of maximal
compression. (Figure 3C is reproduced with permission from Ginsberg L. Lecture notes on neurology, 9th Edn. Wiley-Blackwell, 2010.)

cord lesions identified on MRI are increasingly Neoplastic

being associated with other causes. This has led
to the emergence of the awkwardly named but
clinically useful concept of longitudinally exten-
sive transverse myelitis (extending at least three
vertebral segments, figure 3B). This can be seen
in neuromyelitis optica (NMO, Devic’s disease)
where the clinical cord syndrome is usually much
more severe than in MS.
Post-infective or post-immunisation transverse
myelitis may be a forme fruste of acute disseminated
encephalomyelitis. The typical presentation is with
a monophasic acute or subacute transverse myelopa-
thy a few days or weeks after an infection or immu-
nisation. The initial trigger may be relatively trivial,
for example, an upper respiratory tract infection.
Often, no specific organism is identified but recog-
nised associations include Epstein–Barr virus, vari-
cella zoster virus and Mycoplasma. Spinal MRI may
show a longitudinally extensive transverse myelitis
and the CSF is usually moderately lymphocytic.
Various systemic infl ammatory and autoim-
mune diseases have occasionally been linked to
cord syndromes that resemble MS. The diagnosis
is relatively straightforward if the patient already
has clinical and/or paraclinical features of the
systemic disease, as may occur in sarcoidosis and
(rarely) Behçet’s disease. A diagnosis of sarcoi-
dosis is harder to reach if there are no systemic
features. The situation is more controversial for
systemic lupus erythematosus and Sjögren’s syn-
drome. Again, there is some diagnostic security
if there are systemic features of the autoimmune
disease. However, if the only evidence of lupus
or Sjögren’s is serological, the patient may in fact
have NMO with non-pathogenic antibodies.
Systemic infl ammatory disorders may also
cause extrinsic cord lesions. The most notorious is
atlanto-axial subluxation, a rare complication of
rheumatoid arthritis, where the combination of a
build-up of infl ammatory tissue and weakening of
ligaments puts the patient at risk of a catastrophic
compressive foramen magnum lesion.
Spinal tumours are classified as extradural,
extramedullary–intradural and intramedullary:
▶ Extradural tumours include metastases to the
vertebrae (typically breast, lung, prostate, thy-
roid and kidney cancers) and other bony malig-
nancies (myeloma, lymphoma). These lesions
impinge on the cord, roots or cauda equina
by invading the epidural space, or by causing
vertebral collapse. Patients present with acute
cord compression, back pain and tenderness,
but the history can often be traced back days
or even weeks. Sometimes, malignant dis-
ease can spread through the epidural space,
‘picking off’ roots and compressing the cord
at multiple levels, without involving the ver-
tebrae. This applies particularly to lymphoma
and prostatic carcinoma. The result can be a
confusing mixture of upper and lower motor
neuron signs, even mimicking motor neuron
disease. Non-malignant extradural tumours
include chordomas and lipomas.
▶ Extramedullary–intradural tumours lie on the
surface of the cord, having arisen from the
meninges or a root, mainly meningiomas and
neurofibromas. Spinal meningiomas (figure
3C) have a much more marked female prepon-
derance than their intracranial counterparts.
Neurofibromas may occur in isolation or in the
context of neurofibromatosis. They are more
likely to generate radicular symptoms than
meningiomas.
▶ Intramedullary tumours lie within the spinal
cord, sometimes with a syrinx. They are very
rare and include astrocytomas, ependymomas,
haemangioblastomas and metastases.
An acute necrotising myelopathy may occur as
a paraneoplastic phenomenon, most commonly
with small cell lung cancer. The cord syndrome
may antedate the discovery of the cancer by many
months. Antineuronal antibodies (anti-Hu) are
usually present.

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Vascular
Like the brain, the cord can be affected by both
haemorrhagic and ischaemic vascular events.
Bleeding into the epidural or subdural space,
for example, through anticoagulant excess, will
cause an acute compressive transverse myelopa-
thy. Haemorrhage within the cord (haematomy-
elia) may be due to an underlying arteriovenous
or cavernous malformation.
Ischaemic cord infarction most commonly
affects the anterior spinal artery territory, produc-
ing a ventral cord syndrome of abrupt and pain-
ful onset. Causes include atherosclerosis of feeder
vessels (in particular, the artery of Adamkiewicz),
aortic dissection, and aortic surgery (especially if
complicated by hypotension). Rarer possibilities
are thrombosis of the anterior spinal artery itself,
vasculitis, infective endocarditis, decompression
sickness and fibrocartilaginous embolism.
Spinal dural arteriovenous fi stula is uncom-
mon but potentially treatable. Patients are usu-
ally male and middle-aged or elderly, presenting
with a subacute or chronic, stepwise, fluctuating
deterioration in gait, with pain in the back or leg,
sensory disturbance and ultimately sphincter
involvement. Exercise may trigger deterioration.
On examination, a combination of upper and
lower motor neuron signs may be seen. Although
the fi stula is usually in the lower cord, the sensory
level may be several segments higher because of
ascending venous congestion. Spinal MRI may
show only subtle signal change in the lower cord
and conus or an enlarged draining vein, although
spinal MR angiography may be more informative.
Confi rmation of the diagnosis ultimately requires
selective catheter angiography. The fistula may be
amenable to embolisation or surgical occlusion.
Metabolic and toxic
As the name implies, subacute combined degen-
eration of the cord, secondary to vitamin B12 defi-
ciency, is characterised by a combination of dorsal
column and corticospinal tract dysfunction, usu-
ally evolving over the course of a few weeks.
There is also a significant component of peripheral
neuropathy (hence the coexistence of lower motor
neuron with the upper motor neuron signs from
the myelopathy—absent ankle reflexes and exten-
sor plantar responses) and, later, optic neuropathy
and cognitive impairment. Vitamin B12 deficiency
is remarkable as one of the few causes of periph-
eral neuropathy where the hands may be affected
before the feet, but this is largely because of the
coexistent myelopathy; spinal MRI shows signal
change in the dorsal columns of the cervical cord.
The neurological complications of vitamin B12
deficiency may occur in the absence of any hae-
matological disorder, even with a normal mean
corpuscular volume. Investigation should include
measurement of serum methylmalonic acid and
homocysteine as well as vitamin B12 levels.
Copper deficiency has been recognised recently
as a cause of a subacute combined degeneration
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Neurology in Practice
picture. At risk patients include those who have
undergone partial gastrectomy or gastric bypass
surgery (hence impairing copper absorption) and
individuals with excessive zinc intake (zinc com-
peting with copper for absorption in the proximal
duodenum). The diagnosis is confi rmed by very
low serum copper and caeruloplasmin levels. The
haematological fi ndings of copper deficiency are
different from vitamin B12 deficiency, sideroblastic
anaemia with neutropenia.
Chronic liver failure may lead to a range of neu-
rological deficits, including a slowly progressive
myelopathy.
Toxic causes of myelopathy include:
▶ Nitrous oxide inhalation producing clinical fea-
tures resembling subacute combined degenera-
tion. Patients having multiple anaesthetics are
at risk, as are dentists and veterinary surgeons
who self-administer for recreational purposes.
▶ Oil based contrast medium (Myodil), previ-
ously used for myelography, causes spinal
arachnoiditis with consequent cord and cauda
equina damage. Arachnoiditis can also develop
after spinal surgery.
▶ Radiation—a slowly progressive myelopathy
may occur months or years after
radiotherapy.
▶ Dietary toxins—lathyrism from consumption
of the chickling vetch Lathyrus sativus, konzo
from cassava.
Degenerative
Cervical spondylotic myelopathy is the most
common cause of a spastic paraparesis in elderly
patients. The extrinsic compression arises from a
combination of degenerative disc disease, osteo-
phyte formation and ligamentous hypertrophy,
leading to myelomalacia (figure 3D). A single
level in the lower or mid cervical region may be
affected (as shown in figure 3) but often there is
multilevel disease. The clinical features are of a
spastic paraparesis, with or without sphincter
and sensory involvement, in association—but not
always—with neck pain and stiffness, and radicu-
lar arm pain. Upper limb signs may be a mixture
of upper and lower motor neuron. It is often possi-
ble to discern the site of the lesion clinically from
the presence of a ‘reflex level’. Thus an absent or
inverted biceps reflex, with brisk reflexes below,
implies myeloradiculopathy at C5.
Lumbar spondylosis, frequently superimposed
on a congenitally stenotic spinal canal, may cause
chronic cauda equina compression. Patients pres-
ent with back and leg pain, with sensory and motor
symptoms in the lower limbs, which may be exer-
cise induced—intermittent claudication of the
cauda equina. Neurogenic claudication is typically
triggered by walking and relieved by sitting or lean-
ing forwards for 5–15 min, considerably longer than
is required to relieve claudication from peripheral
vascular disease. This relationship of symptoms to
posture is thought to reflect the cross sectional area
of the lumbar canal, which is least on standing and
265
 Neurology in Practice
walking, hence resulting in maximal compression
of the cauda equina and its blood supply.
Disc protrusions usually occur spontane-
ously, in the context of disc degeneration, rather
than being linked to a specific traumatic event.
Lateral disc protrusions typically cause a cervi-
cal or lumbar monoradiculopathy. Central disc
prolapse in the lumbar region may cause an
acute cauda equina syndrome, a neurosurgical
emergency.
Motor neuron disease (amyotrophic lateral
sclerosis) occasionally presents as a spastic
paraparesis—an intrinsic (neuro)degenerative
cause of myelopathy. In the absence of lower motor
neuron signs, the diagnosis may be supported by
detecting subclinical evidence of denervation on
electromyography. Primary lateral sclerosis is a
virtually pure symmetrical spastic paraparesis,
progressing to tetraparesis then pseudobulbar
palsy, with sphincter involvement and emotional
lability only occurring late in the course of the
disease. Primary lateral sclerosis has a much bet-
ter prognosis than amyotrophic lateral sclerosis.
The diagnosis rests on a minimum disease dura-
tion of 3 years and the absence of pointers to other
causes (eg, no family history to suggest hereditary
spastic paraplegia, no oligoclonal bands in CSF to
suggest primary progressive MS).
PRINCIPLES OF MANAGEMENT
Investigation
The key investigation for a patient presenting
with a myelopathy or lumbar polyradiculopa-
thy is spinal MRI to identify or exclude cord or
cauda equina compression. The decision to image
a patient with a cervical or lumbar monoradicu-
lopathy depends on the severity and duration of
symptoms. If cord or cauda equina compression
has been excluded, further investigation will be
determined by the patient’s clinical presentation
and spinal MRI fi ndings (table 4).
Surgery
The need for urgent surgery is not controversial
for patients with cord or cauda equina compres-
sion from:
▶ benign extradural or extramedullary–intra-
dural tumour
▶ epidural abscess
▶ epidural haematoma
▶ acute central disc prolapse.
Indeed, the last three diagnoses are neurosurgi-
cal emergencies. Malignant extradural tumours
respond as well to radiotherapy with dexametha-
sone as they do to surgery.
Surgery for severe trauma should be restricted
to spinal stabilisation where there is any risk of
further injury, and to aid early rehabilitation.
There is no justification for prolonged courses
of corticosteroids but methylprednisolone
within 8 h of injury may have some modest
benefit.
266
Less clearcut is the surgical management of
chronic spondylotic compression, where there is
a serious lack of high quality evidence:
▶ Patients with cervical spondylotic myelopathy
are often very elderly, with significant comor-
bidities. The course of the neurological impair-
ment is not necessarily progressive. However,
with advancing myelopathy and deteriorating
disability, in a patient fit for surgery, there is
a case for decompression, mainly to prevent
further deterioration (any improvement being
a bonus). The posterior operation of decom-
pressive laminectomy may be preferred if
multiple levels are affected. Anterior surgery
(eg, Cloward’s procedure) is used for predomi-
nantly discogenic disease.
▶ Many patients presenting with radicular pain
from a lateral disc prolapse in the cervical or
lumbar region are satisfactorily managed with-
out surgery (see below). Operative intervention
should be contemplated only after a failed trial
of conservative therapy of at least 6 weeks,
but sometimes sooner if there are significant
symptoms and signs of root compression.
Medical treatment
Some medical causes of myelopathy demand
treatment as urgently as those amenable to sur-
gery, to minimise irreversible cord damage, for
example, vitamin B12 replacement for subacute
combined degeneration or plasma exchange for a
relapse of NMO.
Most patients with acute radicular pain from a
lateral cervical or lumbar disc prolapse are man-
aged successfully with analgesia, maintenance of
mobility and, if necessary, the involvement of a
multidisciplinary pain management team. Epidural
and root directed injections of corticosteroids are
of uncertain benefit but are sometimes used with
the aim of delaying or avoiding surgery.
Rehabilitation
Specialist spinal units have revolutionised the
prognosis for survival of patients with severe cord
injury. Their environment promotes the atten-
tion to detail required to prevent potentially life
threatening complications, including venous
thromboembolism, pressure sores and urinary
tract damage. Other important areas of manage-
ment for paraplegic and tetraplegic patients are:
▶ prevention of contractures
▶ control of spasticity
▶ bladder, bowel and sexual function
▶ maintenance of nutrition
▶ psychological stress.
Educating and hence empowering patients in the
management of their own condition is vital.
Autonomic dysreflexia is a particular complication
of cervical and high thoracic spinal cord injuries.
It is a reflex response to a noxious stimulus, such
as bladder distension, urinary tract infection or
lower body trauma. The response manifests as
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 Neurology in Practice

Table 4 Investigation of myelopathy CONCLUSIONS

Investigation Purpose/diagnosis ▶ The most important investigation for a patient
presenting with a myelopathy or lumbar
Commonly performed
polyradiculopathy is spinal MRI, to detect
Spinal MRI Detect compression, identify intrinsic lesions, abnormal
vessels or eliminate spinal cord or cauda equina
Brain MRI Evidence of demyelination, leukodystrophy* compression.
Chest x-ray Occult neoplasm, sarcoid ▶ The most common cause of a spastic parapare-
EMG/nerve conduction Evidence of lower motor neuron involvement to support sis in young adults in temperate zones is MS,
a diagnosis of amyotrophic lateral sclerosis, evidence whereas cervical spondylotic myelopathy pre-
of a coexistent neuropathy
dominates in the elderly.
CSF (cells, protein, oligoclonal bands, Evidence of inflammation, infection
culture, PCR) ▶ A spinal epidural abscess presents with
Visual evoked potentials Coexistent optic nerve involvement the triad of pain (local, with tender-
Routine haematology and biochemistry Subacute combined degeneration, other metabolic ness and/or radicular), fever and signs of
including vitamin B12 causes myeloradiculopathy.
Serology including autoimmune panel, Autoimmune connective tissue disorders, specific ▶ In myelopathy, a sensory level implies a lesion
evidence of specific infections infections (HIV, syphilis)
Less commonly used, selected patients only
at or above that level; a motor or reflex level
HTLV-1 serology Tropical spastic paraparesis
generally has greater localising value.
Aquaporin-4 antibodies Neuromyelitis optica ▶ Spinal shock may last 1–2 weeks after the onset
Antineuronal antibodies Paraneoplastic myelopathy of an acute severe transverse myelopathy and
Very long chain fatty acids Adrenomyeloneuropathy mimic a lower motor neuron syndrome.
Serum copper/caeruloplasmin Copper deficiency myeloneuropathy ▶ The blood supply to the spinal cord is vulnera-
Blood cultures Causative organism of epidural abscess, suspicion of ble to hypotension; the artery of Adamkiewicz,
infective endocarditis
may be responsible for most of the supply to
Mutation analysis For example, for hereditary spastic paraplegia genetics
the lower cord.
Angiography Dural arteriovenous fistula, arteriovenous malformation
▶ A Brown–Séquard syndrome, albeit often par-
*Brain imaging may serve additional purposes, such as revealing focal atrophy of the precentral gyrus,
supporting a diagnosis of primary lateral sclerosis or occasionally leading to the discovery of a parasagittal tial, may be caused by any asymmetrical cord
meningioma, masquerading as a myelopathy by causing a spastic paraparesis, albeit asymmetrical. lesion, such as a plaque of demyelination.
▶ The Lhermitte phenomenon occurs com-
monly in MS, but also with other cervical cord
Further reading lesions, such as spondylotic compression and
subacute combined degeneration.
▶ Longitudinally extensive transverse myelitis,
Anatomical source texts defi ned as extending for at least 3 vertebral
▶ Brodal A. Neurological anatomy in relation to clinical medicine, 3rd Edn.
segments, is typical NMO, but also of post-
Oxford: Oxford University Press, 1981. infective transverse myelitis.
▶ Standring S, ed. Gray’s anatomy: the anatomical basis of clinical practice.
▶ Subacute combined degeneration is typically
39th Edn. Amsterdam: Elsevier Churchill Livingstone, 2005 (chapters 18
due to vitamin B12 deficiency but a similar
and 46).
clinical picture may be seen with copper
Commoner causes and complications of myelopathy; some of the diseases
deficiency and nitrous oxide inhalation.
mentioned in this article are described in more detail in other contributions to
the ‘Bare essentials’ series ▶ Acute cauda equina syndrome from a lumbar
▶ Coles A. Multiple sclerosis. Pract Neurol 2009;9:118–26.
central disc prolapse is a neurosurgical emer-
▶ Davies N, Thwaites G. Infections of the nervous system. Pract Neurol
gency; look for ‘red fl ags’: bilateral leg pain and
2011;11:121–31. lower limb motor features, sphincter distur-
▶ Panicker JN, Fowler CJ. Uro-neurology. Pract Neurol 2010;10:178–85.
bance and saddle anaesthesia.
▶ Talbot K. Motor neuron disease. Pract Neurol 2009;9:303–9. ▶ Cord or cauda equina compression from a
Rarer causes malignant extradural tumour is a neuro-
▶ Wong SH, Boggild M, Enevoldson TP, et al. Myelopathy but normal MRI: oncological emergency; treatment with radio-
where next? Pract Neurol 2008;8:90–102. therapy and steroids is as good as surgical
Management and rehabilitation intervention.
▶ Lin VW, ed. Spinal cord medicine: principles and practice. New York: ▶ Many patients presenting with radicular pain
Demos, 2003. from a cervical or lumbar lateral disc prolapse
are successfully managed conservatively.
▶ Early transfer of patients with severe spinal
sympathetic overactivity with severe headache cord injury to specialist rehabilitation units
and hypertension, the latter occasionally fatal. improves outcome.
Prevention may involve the judicious use of
α-adrenergic blocking antihypertensive drugs, as Acknowledgements To Jonathan Rohrer, London who com-
well as avoidance of potential precipitants. Acute mented on this article, and to Richard Davenport, Edinburgh, who
treatment of autonomic dysreflexia includes sit- reviewed it.
ting the patient upright, removing the precipitant Competing interests None.
and control of hypertension, for example, with Provenance and peer review Commissioned;
sublingual nifedipine. externally peer reviewed.

2011;11:259–267. doi:10.1136/practneurol-2011-000069 267