spinal cord disease

1. Paraplegia and Spinal Cord
Syndromes

2. A spinal cord syndrome may develop from extramedullary and intramedullary pathological processes.
Initial symptoms may be gradual in onset and progressive, including pain, dysesthesia, or subtle
upper or lower
extremity weakness. In other cases, such as an inflammatory myelitis, acute onset of severe motor,
sensory, and autonomic deficits may develop without premonitory symptoms.
both the rostrocaudal segmental level of disease involvement or
trauma and completeness of the lesion in the transverse plane
anticipate the person’s impairments and disability.

5. Structural information about the integrity of the spine may be obtained
from radiographic plain films and computed tomography (CT) for bone
pathology. Myelography is indicated when extrinsic cord compression is
suspected, especially when magnetic resonance imaging (MRI) is
contraindicated. MRI with contrast best reveals intrinsic and extrinsic
cord pathology. Spinal angiography identifies vascular pathology.
Patients presenting with an acute spinal cord syndrome after trauma
show both early (days to 3 months) and late (up to 2 years) changes in
their motor and sensory deficits.

6. Both neurological improvements and clinical worsening may occur.
When some sparing of sensation and movement is present in the first
72 hours after trauma, the prognosis for walking is rather good. Indeed,
up to 90% of patients with a cervical central cord injury who have any
spared sensation and movement below the level of injury by 4 weeks
after trauma will become functional ambulators.

7. Differential Diagnosis of Diseases Affecting
the Spinal Cord

8. Spinal Shock
Spinal shock refers to the period of depressed spinal reflexes caudal to
an acute spinal cord injury; it is followed by the emergence of
pathological reflexes and return of cutaneous and muscle stretch
reflexes .The bulbocavernosus and cremasteric reflexes commonly
return before the ankle jerk, Babinski sign, and knee jerk.

9. Unilateral Transverse Lesion
A hemisection lesion of the spinal cord causes a Brown-Séquard
syndrome. A pure hemisection is unusual, but patients may show
features of a unilateral lesion or hemisection. A Brown-Séquard lesion
is characterized by ipsilateral weakness and loss of both vibration and
position sense below the level of the injury. In addition, there is a loss
of temperature and pain sensation below the level of the lesion on the
contralateral side. commonly encountered after traumatic injuries,
including bullet and stab wounds.

11. Central Cord Syndrome
triad of (1) motor impairment disproportionately more severe in the upper
than the lower extremities, (2) bladder dysfunction usually including urinary
retention, and (3) sensory dysfunction of varying degrees.
dissociated sensory loss for pain and temperature, whereas vibration and
position sense remain preserved.
a cape-like sensory deficit may be encountered in patients with a cervical
level injury, but sensation within more caudal dermatomes would generally
be spared.
A traumatic central cord syndrome is mostly encountered in elderly patients
who have suffered a relatively minor trauma in the form of a cervical
hyperextension injury, commonly in the setting of an underlying cervical
spondylosis. Falls and motor vehicle injuries are common etiologies.
Syringomyelia or tumors may also produce a central cord syndrome.

12. hyperextension injury
seen in older patients
with CCS secondary to
underlying cervical
spondylosis.
Cervical spondylosis with cervical stenosis in a 73-year-old
man who presented with a burning sensation and weakness
in the upper extremities 2 years after a fall. Sagittal T2-
weighted MR image shows severe cervical spondylotic
changes (arrow), with a disk-osteophyte complex and
buckled ligamentum flavum (white arrowheads). Note the
mild increased signal intensity (black arrowhead) in the cord
secondary to myelomalacia.

14. Ependymoma in a 45-year-old man with
numbness, tingling, and weakness in both upper
extremities, as well as mild ataxia. (a) Sagittal T2-
weighted MR image shows an expansile lesion
(thick straight white arrows) with a heterogeneous
mild hyperintense signal at the C7-T1 spinal level,
a focal hypointense signal (black arrow) due to
hemorrhage, polar cysts (thin straight white
arrows) at the C4-C6 spinal level, and cord edema
(curved arrows). Note the “tumor cap” sign—that
is, the hypointense signal (white arrowhead) along
the cranial margin of the lesion. There is a fluid-
fluid level (black arrowheads) in the cyst at the C5-
C6 level. (b) Sagittal contrast-enhanced MR
image shows an intensely enhancing expansile
mass (large white arrow), with a focal hypointense
signal (arrowhead) secondary to hemorrhage and
polar cysts (small white arrows). (c) Axial contrast-
enhanced MR image shows an intensely
enhancing lesion with focal hemorrhage
(arrowhead). The lesion nearly replaces the entire
cord.

15. Findings in a 10-year-old boy with a grade III astrocytoma who presented with neck and
bilateral shoulder pain, difficulty gripping a pen, and a change in handwriting. Sagittal T2-
weighted (a) and contrast-enhanced (b) MR images show an expansile lesion (arrows)
with a homogeneous hyperintense signal and mild diffuse enhancement.

16. Hemangioblastoma in a 16-year-old girl with a history of neck
pain. (a) Sagittal T2-weighted MR image shows an expansile lesion
with a heterogeneous hyperintense signal (long straight arrows) from
the C4 through C6 level, diffuse edema (short straight arrows), cystic
changes (curved arrows), and foci of hypointense signal (arrowhead)
due to hemorrhage or flow voids. (b) Sagittal contrast-enhanced MR
image shows an intensely enhancing expansile lesion (arrows) and foci
of hypointense signal (arrowhead) due to hemorrhage or flow voids.

17. Chiari I malformation with syringohydromyelia in
a 7-year-old girl with recurrent headaches,
frequent cuts to both hands, bilateral upper-
extremity weakness, and mild ataxia. (a) Sagittal
T2-weighted MR image shows pointed cerebellar
tonsils (double-headed arrow) below the plane of
the foramen magnum (white line) and interrupted
syringohydromyelia, which is greater in the
cervical cord (arrow) than in the thoracic cord
(arrowhead). (b) Axial T2-weighted MR image of
the cervical cord shows a large syrinx (arrow).

18. Anterior Spinal Artery Syndrome(ACS)
involves the anterior two-thirds of the spinal cord, sparing the posterior columns. The
corticospinal and spinothalamic tracts are both affected. The syndrome is clinically
characterized by paralysis and sensory impairments below the level of the lesion, with
impaired sensation of pain and temperature; vibration sense and proprioception are
preserved. Fiber tracts for autonomic control are also typically compromised, resulting in
bladder, bowel, and sexual dysfunction.
caused by trauma -central disc compression or a bone fragment - myelitis.
Vascular occlusive causes are perhaps the most common etiology a spinal cord stroke from
atherothrombotic or embolic occlusion of the anterior spinal cord artery.
Invasive vascular and thoracoabdominal surgical procedures due to obstruction or
hypoperfusion of the artery of Adamkiewicz near the T6 level.
surgery at the distal aorta and proximal iliac arteries
retroperitoneal hematomas or abscesses.
survivors of cardiac arrest
significant hypotensive episodes
midthoracic anterior cord ischemic syndrome, as the vascular supply near the T6 segment is
particularly susceptible to distal field ischemia

19. (a) Drawing shows a
patchy shaded area,
representing the
involved in ACS, with
of the dorsal
columns (DC). The
affects the CST, STT,
horn motor neurons
autonomic center
(★). (b) Drawing
areas affected by the
features of ACS. These
features include motor
involving the CST and
pain and temperature
sensations involving

20. Acute spinal cord ischemia in a 66-year-old man who presented with acute paraplegia 2 days after
undergoing coronary artery bypass graft placement. (a, b) Axial diffusion-weighted MR image (a) and
corresponding apparent diffusion coefficient map (b) show restricted diffusion (arrow). (c) Sagittal T2-
weighted MR image shows a linear pencil-like hyperintense signal (arrows) in the lower thoracic cord.

21. Spinal cord infarction in a 53-year-old woman with a history of sepsis
and severe hypotension, who presented with paraparesis and urinary
incontinence after recovering from shock. Axial T2-weighted MR image
shows a hyperintense signal (arrows) resembling snake eyes in the
anterior spinal cord.

22. Anterior Horn and Pyramidal Tract Syndromes
Paralysis may be encountered in the setting of motor impairments in
combination with relative sparing of sensory and autonomic functions,
as seen in motor neuron disease including amyotrophic lateral sclerosis
(ALS).
Lower motor neuron weakness with atrophy and loss of reflexes is
typically seen in combination with upper motor neuron weakness, signs
of spasticity, and hyperreflexia. Different limbs may be affected to
various degrees, but symptoms are progressive over the course of the
disease. However, innervation of the external anal and urethral
sphincters is normally preserved in ALS, with sparing of bladder and
bowel function.

25. Combined Posterior and Lateral Column
Disease
• A clinical syndrome characterized by the development of a spastic
ataxic gait pattern may be caused by lesions affecting the posterior
and lateral white matter tracts. Friedreich ataxia represents a genetic
etiology, and vitamin B12 deficiency may result in subacute combined
degeneration with spastic paretic gait and sensory ataxia. Dorsal horn
and column injury alone may result from tabes dorsalis.

27. Foramen Magnum and Upper Cervical Spine
nvolvement of the lower portion of the brainstem is suggested by speech impairments,
including dysarthria and dysphonia, as well as by dysphagia.
facial numbness and nystagmus may be detected.
spastic hemiparesis or quadriparesis.
upper extremity weakness, muscular atrophy, and decreased muscle stretch reflexes.
Lesions affecting the uppermost portion of the cervical spine may be challenging to
diagnose owing to a nonlocalizing symptom complex upon initial presentation. Pain is a
common early symptom and may be localized to the neck or occipital region.
Sometimes the pain may be aggravated by neck movement.
A lower motoneuron injury presentation with upper extremity muscular weakness and
atrophy may also be part of the clinical presentation.
disease process affecting the upper cervical spinal cord(above C3) may also
compromise breathing.
Cause: Arnold-Chiari malformations; traumatic injuries; rheumatoid arthritis;
syringomyelia; vascular lesions such as vertebral artery thrombosis, dissection, or an
arteriovenous malformation; and a variety of tumors including meningiomas and
Multiple sclerosis.

28. Lower Cervical and Upper Thoracic Spine
localization of the underlying lesion is most precise for the extramedullary
pathological processes (e.g., tumors, herniated discs) that compress individual
segmental nerve roots or spinal nerves. Intramedullary lesions may also present
with pain, but the segmental localization is commonly less precise.
Extramedullary lesions typically first irritate segmental nerve roots and the spinal
nerve, with radicular pain and sensory deficits typically following the corresponding
dermatomal distribution. Similarly, motor deficits involve each myotome affected
by the lesion. Muscle stretch reflexes may also provide helpful information with
regard to the primary level of injury, as the affected segmental reflex is typically
depressed or absent and caudal reflexes are hyperactive.
Lower and upper motoneuron signs may also be present in adjacent segments. If
segmental nerve roots and the spinal cord are compressed by a herniated disc or
space-occupying lesion at the C5–C6 level, for example, a decreased brachioradialis
reflex may reflect a C6 radiculopathy, whereas a brisk and hyperactive finger flexor
reflex reflects an upper motoneuron syndrome.

33. Thoracic Levels
Traumatic spinal cord injury at the thoracic level usually produces a
complete lesion. The segmental level of injury is best determined by a
careful sensory examination of dermatomes. Useful clinical landmarks
are the nipple line for the T4 dermatome and the umbilicus for the T10
dermatome. Pain may follow a radicular pattern around the chest or
abdomen corresponding to the segmental levels of injury.
combination of at-level pain, sensory deficits, and muscular weakness,
autonomic dysfunction.

34. Thoracic spinal haemangioblastoma with severe cord compression
(consistent with VHL).

35. Extensive increased T2 signal and expansion of the cord is seen
extending between C7 and T12. The T2 signal abnormality involves
central grey matter and dorsal columns. Linear sagittally orientated
enhancement is seen posteriorly within the cord in the mid and lower
thoracic cord.
Transverse myelitis - Devic disease

36. Spinal cavernoma

37. Spinal dural arteriovenous fistula in a 45-
year-old man with progressive weakness,
tingling and numbness in the bilateral lower
extremities, and urinary
incontinence. (a) Sagittal T2-weighted MR
image shows a central hyperintense signal in
the conus medullaris and lower thoracic cord
(straight arrows), a peripheral hypointense
signal (arrowheads), and flow voids (curved
arrows) along the dorsal surface of the
cord. (b) Sagittal contrast-enhanced MR
image shows tortuous enhancing vessels
(arrows) along the dorsal surface and
variable contrast enhancement (arrowheads)
of the cord.

38. Conus Medullaris and Cauda Equina
The conus medullaris of the spinal cord terminates approximately at the level
of the L1 vertebra.
conus medullaris usually result in weakness or paralysis of the lower
extremities, absence of lower extremity reflexes, and saddle anesthesia .
some patients with conus medullaris injuries exhibit a mixed upper and
lower motoneuron syndrome.
Cauda equina injuries present with lower extremity weakness, areflexia,
decreased muscle tone, and variable sensory deficits. At least one-third of
these patients suffer considerable central pain. Affected limb and pelvic floor
muscles develop flaccid weakness, and electromyography shows denervation
after either a conus medullaris or cauda equina injury, especially following
anatomically complete lesions.
trauma to the thoracolumbar spine
A lumbar spinal stenosis due to a congenitally small-diameter spinal canal or
central disc and spondylotic narrowing one or more levels below L1 may
present with a subtle course.

39. Lumbar spondylosis with canal stenosis and CES in a 72-year-old man with increasing bilateral lower-extremity
weakness and right lower-extremity paresthesia. (a) Sagittal T2-weighted MR image shows a multilevel disk bulge or
herniation (arrowheads) and ligamentum flavum buckling (thin arrows). The combination of these findings can lead to an
hourglass appearance of the spinal canal. Crowded redundant cauda equina nerve roots (thick arrows) have a
serpiginous appearance. (b) Axial T2-weighted MR image shows a “trefoil” configuration (arrows) of the central canal and
buckling

40. Type II arachnoiditis in a 40-year-old woman who had a history of prior spinal surgery and presented with lower-
extremity weakness (greater on the left than on the right) and altered sensation. (a) Sagittal T2-weighted MR
image shows an empty thecal sac (arrow). (b) Axial T2-weighted MR image shows an empty thecal sac (arrow),
with peripheral clumping of the nerve roots (arrowheads).

41. Type I arachnoiditis in a 32-year-old man who had a history of
meningitis and presented with back pain, bilateral lower-extremity
weakness, and urinary and fecal incontinence. Axial T2-weighted MR
image shows central conglomeration (arrow) of the nerve roots.

42. Juvenile pilocytic astrocytoma in a 5-year-old girl with difficulty walking, bilateral leg pain, and
urinary and fecal incontinence. Sagittal T2-weighted MR image shows an expansile lesion
with solid (large arrow) and cystic (small arrows) components in the conus medullaris and
edema (arrowhead) in the adjacent cord. This solid component exhibited intense
enhancement (not shown).

43. PAIN AND AUTONOMIC DYSFUNCTION
pain and dysfunction in the autonomic nervous system can aid in the
localization of spinal cord syndromes. Pain is frequently associated with
spinal cord injuries, along with autonomic impairments that may affect
blood pressure and heart rate, bladder, bowel, sexual, and
cardiorespiratory function.

44. Pain Syndromes
result of compression, inflammation, or injury to the vertebral column,
ligaments, dura mater, nerve roots, dorsal horn, and ascending spinal
cord sensory tracts. Neuropathic pain may take the form of paresthesia
(abnormal but not unpleasant sensation that is either spontaneous or
evoked), dysesthesia (an abnormal unpleasant sensation that is
spontaneous or evoked), allodynia (pain evoked by ordinary stimuli
such as touch or rubbing), and hyperalgesia (an augmented response to
a stimulus that is usually painful).

45. Local Pain
Localized neck or back pain may result from irritation or injury to
innervated spine structures including ligaments, periosteum, and dura.
The pain is typically deep and aching, may vary with a change in
position, and often becomes worse from increased load or weight
bearing on affected structures. Percussion or palpation over the spine
may in some patients worsen the local pain. When the injured or
diseased spinal structures are irritated, secondary symptoms may
develop, including muscle spasm and a more diffusely located pain.

46. Projected Pain
pathological process involving the facet joints may be experienced as
focal or radiating pain in an upper or lower extremity. When a nerve
root is irritated or injured, the projected pain is radicular.
Radicular pain commonly has a sharp, stabbing quality or causes
dysesthesia. It may be exacerbated by activities that stretch the
affected nerve root (e.g., straight leg raising or flexion of the neck).
Straining or coughing may also increase the intensity and severity of
radicular pain. Nerve root irritation may also result in sensory and
motor deficits following the same dermatome and myotome
distribution as the affected nerve root.

47. Central Neurogenic Pain
Paresthesia, dysesthesia, allodynia, and hyperalgesia accompany injury to
the spinal cord in at least half of patients and also occur after
thalamocortical stroke. Regardless of segmental level or completeness of
injury, most patients with a traumatic spinal cord injury develop a clinically
significant pain syndrome at some time after sustaining the lesion.
Pain developing after a spinal cord injury is commonly described as burning,
pricking, or aching in quality. It can be experienced as deep or superficial.
Some patients develop a severe and excruciating pain syndrome after cord or
cauda trauma that is at-level and below-level.
The medication most recently approved by the US Food and Drug
Administration for spinal pain is pregabalin. The mechanisms for such painful
phantom phenomena are not well understood but include structural and
molecular dorsal horn, thalamic, and cortical adaptations to ordinary and
noxious inputs.

48. Autonomic Dysreflexia
Injuries to the spinal cord that result in paraplegia from a lesion above T6
may also impair autonomic control and result in episodes of severe
hypertension or hypotension. Autonomic dysreflexia represents an acute
syndrome characterized by excessive and uncontrolled sympathetic output
from the spinal cord. As a result, the blood pressure is suddenly and
markedly elevated. Associated symptoms include headache; malaise;
blurring of vision; flushed, sweaty skin above the level of injury; and pale,
cool skin below it. An episode of autonomic dysreflexia can be triggered by
any noxious stimulus below the segmental level of injury. Common triggers
include bladder distention, constipation, rectal fissures, joint injury, and
urinary tract infection. Autonomic dysreflexia may present soon after the
initial injury but more commonly becomes symptomatic several months after
the spinal cord injury. Prevention is the best approach. Treatment of acute
symptoms targets removal of noxious stimuli and cautious lowering of the
blood pressure

49. Bowel and Bladder Dysfunction
bowel control depend on segmental reflexes involving both autonomic and
somatic motor neurons as well as descending and ascending tracts of the
spinal cord.
Traumatic spinal cord injuries with paraplegia taking place above the T12
vertebra will interrupt spinal cord long-tract connections between
supraspinal micturition centers in the brainstem and cerebral cortex and the
sacral spinal cord. An upper motoneuron syndrome follows.
injury to the T12 vertebra and below results in a direct lesion to the sacral
spinal cord and associated nerve roots. A direct lesion to preganglionic
parasympathetic neurons and somatic motoneurons of the Onuf nucleus
located within the S2–S4 spinal cord segments results in the denervation of
pelvic targets. Injuries to both the conus medullaris and cauda equina
present as a lower motoneuron syndrome characterized by weak or flaccid
detrusor function.