Approach to myelopathy

NOSOLOGY
 Myelopathy : A broad generic term referring to
a lesion affecting spinal cord.
 Myelitis : An inflammatory disease of the
spinal cord.

Introduction
 Myelopathy is a broad term that refers to spinal cord involvement of
multiple etiologies.
 Spinal cord diseases often have devastating consequences, ranging
from quadriplegia and paraplegia to severe sensory deficits due to its
confinement in a very small area.
 Many of these diseases are potentially reversible if they are recognized
on time.
 The importance is – weather treatable or not treatable
 Prognosis depends on an early and accurate diagnosis.

 Spinal cord pathologies may be classified as
 Acute – within days
 Subacute – 2 to 6 weeks
 Intermittent or chronic - > 6weeks
 Depending on the time course, the
extent of the involvement, the clinical
picture or syndrome, or the etiology

 Acute onset that worsens within hours or days
points to a spinal cord infarct or hemorrhage.
 When symptoms are recent, it is of paramount
importance to rule out a surgical emergency.
 This requires immediate imaging work-up, ideally
total spine magnetic resonance (MR).

 If there is evidence of spinal cord compression due to an acute lesion
(epidural metastasis or abscess), definitive management is required in
order to avoid damage or to adequately manage all other potential
diagnoses.
 If the symptoms progress for more than three weeks, transverse
myelitis is improbable.
 Other conditions must be considered such as a
 spinal tumor,
 chronic compressive disease,
 dural arterio-venous fistula,
 metabolic disorder,
 sarcoidosis, or a degenerative process.

Diffrecnce between cord,nerve and root lesion
CORD NERVE ROOT
Adjoining area involvement
can affect the ascending
and descending tracts
Formed by many
roots.supplies definite area.
Hence sensory/motor loss
will be confined to that
area.
Forms apart of nerve or
nerves.
Area supplied by one nerve
root ther eis considerable
overlapping.hence loss is
minimal.
Cord lesion is partial or
complete.Depend upon the
particular area,long tract
involvement below the
lesion UMN paralysis
occurs.Below the lseions
long sensory tracts-
spinothalamic/postolumn
deficit may be seen.
Can be motor, sensory or
mixed.
In motor nerve there may
be wasting after 3 weeks.
In sensory loss of
superficial sensation.
Mixed nerve both.
Anterior root – pure motor
with marked weakness and
wastiing.
Posterior root – pure
sensory,areflexia normal
motor power.

SPINAL SHOCK SYNDROME
Areflexia , atonia
May confuse with GBS
May persist for days to week
Mean duration of 4 to 6 weeks
Overtime spsticity,hyper reflexia
evident
CLINICAL PRESENTATION

LEGEND
First-order neuron
Second-order neuron
Third-order neuron
Pain stimulus
Mechanism of injury
Lesion
Sensory impairment
Function intact
Function lost
Light touch stimulus
Main Menu Content Menu Exit

Lesion of the right dorsal column at L1
produces what impairment?
Click for answer
Damage to the right dorsal column at L1 causes the absence of light
touch, vibration, and position sensation in the right leg. Only
fasciculus gracilis exists below T6.
Click for explanation
Main Menu Content Menu Legend Exit
R L

Right Dorsal Column Lesion
Dorsal column lesion
Ipsilateral loss of light touch,
vibration, and position sense
generalized below the lesion level
Below T6 only the fasciculus gracilis
is present.
R L
DRG
L1
Common causes
include MS,
penetrating injuries,
and compression
from tumors.
Click to animate

Lesion of the right fasciculus cuneatus
at C3 produces what impairment?
Click for answer
Damage to the right fasciculus cuneatus at C3 causes the absence of
light touch, vibration, and position sensation in the right arm and upper
trunk.
R L

Right Fasciculus Cuneatus Lesion
Fasciculus cuneatus lesion
In the right arm and upper trunk
R L
DRG
C3
Common causes
include MS,
and compression
from tumors.
Click to animate

Lesion of the right lateral corticospinal
tract at L1 produces what impairment?
Click for answer
Damage to the right lateral corticospinal tract at L1 causes upper motor
neurons signs (weakness or paralysis, hyperreflexia, and hypertonia) in the
right leg.
R L

R L
UMN
Lateral corticospinal tract lesion
Ipsilateral upper motor neurons signs
generalized below the lesion level
UMN signs
Weakness (Spastic paralysis)
Hyperreflexia (+ Babinski, clonus)
Hypertonia
Right Lateral Corticospinal Tract Lesion
L1
Common causes
include penetrating
injuries, lateral
compression from
tumors, and MS.
Click to animate

Click for answer
Damage to the right lateral spinothalamic tract at L1 causes the
absence of pain and temperature sensation in the left leg.
Lesion of the right lateral spinothalamic
tract at L1 produces what impairment?
R L

R L
DRG
Lateral spinothalamic tract lesion
Contralateral loss of pain
and temperature sense
Right Lateral Spinothalamic Tract Lesion
L1
Common causes
include MS,
and compression
from tumors.
Click to animate

Click for answer
Damage to the anterior gray and white commissures at C5-C6 causes
the absence of pain and temperature sensation in the C5 and C6
dermatomes in both upper extremities.
Lesion of the anterior gray and white
commissures (central cord syndrome) at
C5-C6 produces what impairment?
R L

C5-C6
Central Cord Syndrome
Lateral
Spinothalamic
Tract
Impaired pain and temperature
sensation, C5-C6 dermatomes,
bilaterally
DRG DRGR L
Common causes
include posttraumatic
contusion and
syringomyelia, and
intrinsic spinal cord
tumors.
Click to animate

Click for answer
Damage to the right dorsal columns at L1 causes the absence of light
touch, vibration, and position sense in the right leg. Damage to the
lateral corticospinal tract causes upper motor neuron signs in the right
leg (Monoplegia), and damage to the lateral spinothalamic tract causes
the absence of pain and temperature sensation in the left leg.
Complete transection of the right half the spinal
cord (Hemicord or Brown-Sequard syndrome)
at L1 produces what impairments?
R L

R L
Hemicord Lesion (Brown-Sequard Syndrome)
Hemicord lesion
Build the lesion
L1
Common causes
include penetrating
injuries, lateral
compression from
tumors, and MS.
Click to animate

UMN
Hemicord lesion
R L
DRG
DRG
L1
Click to animate

Click for answer
Damage to the dorsal columns, bilaterally, causes the absence of light
touch, vibration, and position sense in the both legs. Damage to the lateral
corticospinal tracts, bilaterally, cause upper motor neuron signs in the
both legs (Paraplegia), and damage to the lateral spinothalamic tracts,
bilaterally, cause the absence of pain and temperature sensation in the
both legs.
Complete transection of the spinal cord
(Transverse cord lesion) at L1 would
produce what impairments?
R L

R L
Bilateral loss of light touch,
Bilateral upper motor neurons signs
Bilateral loss of pain and
temperature sense
Transverse Cord Lesion
Transverse cord lesion
Build the lesion
Common causes
include trauma,
tumors, transverse
myelitis, and MS.
Click to animate

R L
Transverse Cord Lesion UMNUMN
DRG
DRG
Click to animate

Click for answer
Damage to the dorsal columns (fasciculus gracilis and cuneatus),
bilaterally, causes the absence of light touch, vibration, and position
sense, bilaterally, from the neck down (below the lesion level).
Complete transection of the dorsal
columns, bilaterally, (posterior cord
syndrome) in the cervical region would
produce what impairments?
R L

R L
Posterior Cord Syndrome
DRG
DRG
Dorsal column lesion (bilateral)
Bilateral loss of light touch,
vibration, and position sense,
generalized below lesion level
Common causes
include trauma,
compression from
posteriorly located
tumors, and MS.
Click to animate

Click for answer
Damage to the lateral corticospinal tracts cause upper motor
neuron signs, bilaterally, below the lesion level. Damage to
lower motor neurons in the ventral horns cause lower motor
neuron signs, bilaterally, at the lesion level. Damage to the
lateral spinothalamic tracts cause absence of pain and
temperature sensation, bilaterally, below the lesion level.
Sparing of the dorsal columns leaves light touch, vibration,
and position sense intact throughout.
Complete transection of the lateral corticospinal and
lateral spinothalamic tracts with sparing of the dorsal
columns, bilaterally, (anterior cord syndrome) in the
cervical region would produce what impairments?
R L

UMN
DRG
UMN
DRG
R L
Anterior cord lesion
Anterior Cord Syndrome
Common causes
include anterior
spinal artery
infarct, trauma,
and MS.
Click to animate

R L
Left Dorsal Column Lesion
DRG
Click to animate
T10
Main Menu Case Menu Exit

R L
Transverse Cord Lesion UMNUMN
DRG
DRG
Contralateral loss of pain and
temperature sense
Click to animate
C3

Contralateral loss of pain and
temperature sense
UMN
Hemicord lesion
R L
DRG
DRG
T12
Click to animate

Epiconus syndrome, caused by a
spinal cord lesion at the L4 to S2
level, is relatively rare. Unlike
conus syndrome, it is associated
with spastic or flaccid paresis of
the lower limbs, depending on
the precise level of the lesion.
There is weakness or total
paralysis of hip external rotation
and extension (L4–L5), and
possibly also of knee flexion (L4–
S2) and flexion and extension of
the ankles and toes (L4–S2). The
Achilles reflex is absent, while the
knee-jerk reflex is preserved. The
sensory deficit extends from L4 to
S5. The bladder and bowel empty
only reflexively; sexual potency is
lost, and male patients often have
priapism. There is transient
vasomotor paralysis, as well as a
transient loss of sweating.

Epiconus
 Epiconus syndrome, caused by a spinal cord lesion at the L4 to
S2 level, is relatively rare.
 Unlike conus syndrome, it is associated with spastic or flaccid
paresis of the lower limbs, depending on the precise level of the
lesion.
 There is weakness or total paralysis of hip external rotation and
extension (L4–L5), and possibly also of knee flexion (L4–S2)
and flexion and extension of the ankles and toes (L4–S2).

Epiconus
 The Achilles reflex is absent, while the knee-jerk
reflex is preserved.
 The sensory deficit extends from L4 to S5.
 The bladder and bowel empty only reflexively;
sexual potency is lost, and male patients often
have priapism.
 There is transient vasomotor paralysis, as well as
a transient loss of sweating.

The nerves in the CE region include lower
lumbar and all of the sacral nerve roots.
The pelvic splanchnic nerves carry
preganglionic parasympathetic fibers
from S2-S4 to innervate the detrusor
muscle of the urinary bladder.
Conversely, somatic lower motor neurons
from S2-S4 innervate the voluntary
muscles of the external anal sphincter
and the urethral sphincter via the
inferior rectal and the perineal branches
of the pudendal nerve, respectively.

Clinical signs localizing value in
myelopathy patients
 Beevor sign
 Superficial abdominal reflex
 Cremasteric reflex
 Bulbocavernous reflex
 Anal reflex

Cutaneous (Superficial)
Reflexes
• Upper (T8-T10)
• Lower (T10-T12)
• Pt. supine, knees flexed
• Use handle of reflex hammer
• Normal response →
Ipsilateral (same side)
contraction of the abdominal
muscle with a deviation of the
umbilicus toward the stroke
Motor System in Infants

Extra dural lesions
 Disc lesions: spondylosis,disc prolapse
 Vertebral lesions:
 Congenital :spina bifida
 Trauma
 Infective : potts disease
 Neoplastic-primary myeloma
 Secondary-brest,lung,prostate
 Flurosis
 Paravertebral :abscess,hematoma,aortic aneurysm

Intra dural
 Neurofibroma
 Meningioma
 Arachnoiditis

Direct involvement of roots and cords causing
dysfunction.

Lesions can interfere
with longitudinal and
radicular spinal arteries
causing ischemia of the
segment, which they
supply.
This vascular disturbance
causes local edema of
the cord which results in
degeneration of the
white matter- areas of
softening occur.( called
compressive myelitis.)

Compression can cause
pressure effect upon ascending
longitudinal spinal vein, which
leads to
edema of the cord below the
site of compression
e.g. If there is compression at
high cervical level edema can
occur at C8T1 level resulting in
small muscle wasting.
Order of compression of the
tracts:
1st Pyramidal tract,
then Posterior column,
lastly Spinothalamic tract.
But exception can occur to this
rule.

Early pyramidal ?
 Pyramidal tract is supplied by the terminal branches of spinal
arteries and hence most susceptible to compressive ischemia.
 Another explanation offered:Pyramidal tract is lying closest to
denticulate ligament
 This ligament is subject to traction in spinal cord compression.
 So pyramidal, tract is most involved.
 Obstruction of subarachnoid space, below the level of the
tumor causes loculation of CSF-causing the characteristic
changes in its composition.

Cause of compression of cord
 Intra medullary
 Extra medullary
 Extra dural
 Intra dural

Arise from the dura and compress the cord
and roots-typical
e.g.meningioma

i. Arising from vertebra invading
spinal canal and
compressing intra spinal structures
typical e.g.2ndries of
spine from primaries breast, lung,
prostate
Disc lesions;disc prolapse
/spondylosis act similarly
ii. Through intervertebral
foramina,mass can invade spinal
canal e.g Schwanoma,neurofibroma
iii. Paravertebral masses of diverse
pathologies- can
compress roots,their arteries
autonomic ,dorsal root
ganglia

Guidelines to diffrentiate intramedullary
and extramedullary tumours
Signs and
symptoms
Intramedullary
tumours
Extramedullary
tumours
Radicular pain Unusual Common, may occur
early
Vertebral pain Unusual Common
Funicular pain Common Less common
UMN signs Yes, late Yes, early
LMN signs Prominent and diffuse Unusual, if present
segmental distribution

Cont....
Paresthesias
progession
Descending
progression
Ascending
progression
Sphincter
abnormalities
Early with caudal
lesions
Late
Trophic changes Common Unusual

Upper cervical region&foramen magnum:
 1. Severe pain in the occiput & neck.
 2. In hands loss of posterior column sensation is early symptom
&severe tingling &numbness.
 Pain & weakness in the limbs & wasting may occur in the upper limb.
 3. Movements of diaphragm reduced because of compression of
phrenic nerve.
 4. Lower cranial nerve involvement &medullary involvement
can occur.
 5. Descending tract of trigeminal can be involved.

Ellsberg phenomenon
 Seen in high cervical myelopathy
 Round the clock motor weakness
 Hemi, tri, tetra paresis

C5,C6 segment lesion
 1. INVERTED SUPINATOR REFLEX
 2. wasting of muscles supplied byC5C6
namely deltoid,biceps,brachioradialis,infra &
suprasinators&rhomboids
 3.Quadriplegia

C8T1 segments
 1. Wasting of small muscles of hands.
 2. Wasting of flexors of wrist & fingers.
 3. Horner’s syndrome.
 4. DTR of upper limbs preserved.
 5. Spastic paralysis of trunk & lower limbs.
 Cervical spondylosis never involves C8& so small muscle
wasting rules out cervical spondylosis.

Mid thoracic segments
 Upper limb normal.
 2. Wasting of intercostals muscles (those
supplied by involved segments)
 3. .Movements of diaphragm normal.
 4. Spastic paralysis of abdominal muscles
&lower limbs

 9th &10th thoracic segments:
 1. BEEVOR’S SIGN
 (when patient raises the head against resistance
umbilicus is drawn upwards).

D12L1 segments
 Abdominal reflexes preserved
 Cremastric lost.
 Paraplegia
 Wasting of internal oblique & transverse
abdominal muscle.

L3,4 seggments
 1.Flexion of hip is preserved.
 2. Cremastric preserved.
 3. But Quadriceps & adductors of hip are wasted
 4. KNEE JERK IS LOST or diminished.
 5. BUT ANKLE JERK IS EXAGGERATED.
 6. Plantar-extensor.
 7. Foot drop

S1,2 segments
 1.Wasting & paralysis of intrinsic muscles of feet.
 2. Wasting & paralysis of calf muscles Plantor flexion
impaired.
 3. But dorsi flexion of foot is preserved.
 4. In the hip all muscles of hip are preserved except flexors &
adductors.
 5. In the knee flexors of knee are wasted.
 6. KNEE JERK IS PRESERVED
 7. ANKLE JERK IS LOST.
 8. Plantar reflex is lost.
 9. No foot drop.
 10. Anal & Bulbocavernous reflexes are preserved.

S345 segments
 1. Large bowel & bladder are paralysed.
 2. There is RETENSION OF URINE & FEACES due to
unopposed action of internal sphincters.
 1. The external sphincters are paralyzed.
 2. ANAL & BULBO CAVERNOUS REFLEXES ARE LOST.
 3. SADDLE SHAPED ANESTHESIA occurs.
 4. but no paraplegia

Conus syndrome
 Conus syndrome, due to a spinal cord lesion at or below S3 is also rare. It can be
caused by spinal tumors, ischemia, or a massive lumbar disk herniation.
 An isolated lesion of the conus medullaris produces the following neurological
deficits:
 Detrusor areflexia with urinary retention and overflow incontinence
 Fecal incontinence
 Impotence
 Saddle anesthesia (S3–S5)
 Loss of the anal reflex
 The lower limbs are not paretic, and the Achilles reflex is preserved (L5–S2).
 If conus syndrome is produced by a tumor, the lumbar and sacral roots
descending alongside the conus will be affected sooner or later (Fig. 3.22). In such
cases, the manifestations of conus syndrome are accompanied by deficits due to
involvement of the cauda equina: weakness of the lower limbs, and more
extensive sensory deficits than are seen in pure conus syndrome.

Common causes of compressive
Myelopathy
 I. Vertebral Disease-pain,rigidspine,Angular
deformity
 Common cause TB osteitis(young patient,raised
ESR,Evidence of Tb focus anywherelse
 II. 2ndry Carcinoma: middle age,rapid onset,severe
pain,h/o surgery for carcinoma,primary detection.
 III. Cervical Sponylosis: detected by Xray;but can be
coincidental finding

Cont....
 IV. Spinal tumor:insidious onset,slowly progressive,no
evidence of vertebral disease
 Under general exam look for neurofibroma,pigmentation
 It is difficult to guess the nature of spinal tumor
 V. Meningitis: impossible to differentiate pachy
meningitis and arachnoiditis:
 Multiple levels of lesion and patchy and streky arrest of contrast
medium indicates -arachnoiditis

Cont....
 VI. Myeloma/Pagets:Only by investigations
 VII. Other rare causes: lymphoma,leukemia,parasitic
cyst,extra dural metastasis:
 Diagnosis-on clinical grounds,evidence of disease
elsewhere and investigations

Acute myelopathy
 Trauma-fracture dislocation of vertebra
 Infection: Epidural Abscess
 Vascular:
 Thrombosis of Anterior spinal artery
 Endarteritis(tuberculous,Syphilitic)
 Hematomyelia (AV malformation,Angioma)
 Transverse Myelitis

Multiple level myelopathy
 Arachnoiditis
 Multiple secondaries
 Multiple sclerosis
 Neurofibramatosis
 Spondylosis-cervical and lumbar level

Non compressive Myelopathy
causes

Infective causes
 Bacterial:
 Acute: Staphylococcal (extramural or intradural)
 Chronic:
 Tuberculous, Syphilitic.
 Parasitic: Hydatid, cysticercosis, Schistosomiasis, falciparum malaria.
 Viral: Polio, Rabies, Herpes zoster,HIV
 Rickettsial: Typhus fever, spotted fever
 Fungal: Cryptococcus, Actinomycosis, and coccidiomycosis

Cont….
 Immuno Allergic causes:
 Post vaccinial-Rabies, tetanus, and polio
 Post exanthematous-Chicken pox, Herpes zoster
 Demyelinating:
 Multiple sclerosis, Neuromyelitis optica,
 Sub acute combined degeneration.

Cont…
 Heredo familial, Degenerative:
 Spinocerebellar degeneration.
 Familial spastic paraplegia
 Motor neuron Disease

Toxic myelopathys
 Lathyrism
 TOCP
 Arsenic
 Contrast media used in radiology
 Intra thecal penicillin
 Spinal anesthesia
 SMON-Sub acute myelo opotic neuropathy (long term enteroquinol,
large dose; Myelopotic and peripheral neuropathy. Abdominal
symptoms.

Cont ….
 Vascular Disorder:
 Arteriosclerosis-Anterior spinal artery thrombosis
 Dissecting aneurysm of aorta
 AV malformation in spinal cord
 2ndry to surgery on aorta
 Metabolic /Nutrional;
 B12 deficiency. Pellagra, Myelopathy of chronic liver disease

Cont…
 Tropical;
 Tropical spastic paraplegia
 Para Neoplastic Syndrome:
 Physical agents;
 Irradiation
 Electric shock to spinal cord
 Caisson’s disease.

Manifestations of cord /root lesion
depends upon
 1. Level of lesion
 2. Speed of onset
 3. Vascular involvement
 4. Site

Cont….
 Level of the lesion:
 AboveL1 vertebra- Damage to both cord and roots.
 Below L1 vertebra- only roots are involved.
 Speed of the lesion:
 A rapidly progressive cord lesion produces flaccid
paralysis and absent reflexes and extensor
 plantar. This is similar to spinal shock in trauma.
 After weeks or days tone becomes hypertonic.

Vascular involvement
 In cord compression damage may be due to mechanical
stretching or ischemia.
 In certain cases clinical findings indicate cord damage well
beyond the site of compression.
 This shows vessel compression at the site of lesion is
causing distant ischemic effect.

Site of lesion
 Intra medullary lesion produces only segmental
signs &symptoms.
 Extra dural lesions on the other hand produce both
signs of root lesion cord lesion.
 i.e. At the site of lesion –LMN segmental lesion with
segmental sensory loss & below that level-
 UMN Lesion & sensory loss plus root pain.

Investigations in myelopathy
 Plain Xray Spine
 Myelogram
 CT scan
 CSF analysis
 MRI

Xray
 Plain Xray Spine
 Lateral and oblique view:
 Signs of degeneration of spines
 Reduction of intervertebral space
 Narrowing of intervertebral foramina
 Osteophyte formation.
 Widening of IV foramina-Neuro fibroma
 Widening of inter peduncular distance:-long standing intramedullary,
intradural lesion.

Cont …
 Secondaries,myeloma,tuberculous infection:
 Destruction of vertebra/collapse of vertebra..
 AP view:
 Erosion of the pedicle-extra medullary tumor
 Para spinal mass; -extra medullary tumor /cold abscess.

Myelogram
 A. Extradural:
 Complete block shows with ragged edge.
 At times even with complete block contrast can be coaxed
beyond the block to determine its
 upper extent otherwise a cervical puncture may be required.
 Partial block;
 Extra dural; Dura mater is lifted away from the vertebral body.

Cont…
 B. Intra dural;
 Intra medullary; contrast is splayed around dilated cord.
 Extramedullary; cord displaced to one side.

Cont…
 CSF Analysis;Lumbar puncture can worsen neurological disease,
because of the pressure gradient it creates.
 So when a compressive lesion is suspected lumbar puncture &CSF
analysis can be done at the time of Myelography.
 CSF protein is increased often especially below the block,more
so in extra medullary.
 Cell count; Increase in Leukocytoes indicates infections like
Abcess/TB.

Cont…
 CT scan:
 Plain CT; Can diagnose narrowing of disc disease of lumbar region.
 Can identify narrowing of lumbar canal& thickening of facet joint.
 Contrast CT is required to show Cord compression.:
 Best done 6-12 hrs after myelography.
 Can show amount of compression & delineate neurofibroma likeintraspinal
lesions..
 MRI:
 Saggital views are to be taken., not axial views as in case of CT scan.
 Can differentiate Syringomyelia from intramedullary tumors.

Other investigations
 Xray chest: May show P.T,Lymphomaor malignancy.
 ➼C.S.F.-Electrophoresis to show oligoclonal bands of
multiple sclerosis.
 ➼Serological tests for Syphilis.
 ➼IgG/Albumin ratio-to diagnose multiple sclerosis.
 ➼ Routine blood test;hematological,biochemical
 ➼ Routine urine exam,urine for culture and sensitivity

Cont…
 There are cases where the etiology is never identified, and they
are classified as idiopathic myelopathy.
 In 2001, De Seze et al. found that acute myelopathys
 43% were secondary to multiple sclerosis;
 16.5% were due to a systemic disease;
 14% to a spinal cord infarct;
 6% to an infectious disease;
 4% were secondary to radiation; and
 16.5% were idiopathic.

Introduction
 TM ranges from 1.34 to 4.60 cases per million Increases to 24.6
cases per million if MS are included.
 TM can occur at any age, a bimodal peak in incidence occurs in
second and fourth decades of life.
 No familial or ethnic predisposition, and no evidence of
geographic variation in incidence.
 Two recent studies did find a higher incidence in females,
because higher incidence of MS.
 After excluding patients whose MRI scans were consistent with
MS, incidence of idiopathic myelitis was estimated to be 6.2 per
million.

Cont…
 Transverse myelitis : A heterogeneous syndrome
acute or sub acute spinal cord dysfunction, resulting in
paresis, a sensory level, and autonomic impairment
below the level of lesion.
 Transverse myelitis :
 Longitudinally extensive
 Longitudinally limited

Acute complete transverse
myelitis(ACTM)
 TM causing paresis of lower or upper extremities
 A sensory level
 Autonomic impairment below the level of lesion
 MRI- typically a single lesion spanning 1 or 2 vertebral segments
 On axial section either full thickness involvement, or the
central portion of the spinal cord is maximally affected.

Acute partial TM(APTM)
 TM causing asymmetric neurological impairment
localizable to spinal cord.
 Deficit attributable to a specific anatomic tract
 On MRI it spans 1 or 2 vertebral segments
 There is involvement of small portion of spinal cord on
axial section

Longitudinally- Extensive tm(letm)
 A spinal cord lesion that extends over 3 or more vertebral
segments on MRI
 On axial section it typically involve center of the cord over
more than two- thirds of spinal cord area

Secondary transverse myelitis:
 TM related to systemic inflammatory autoimmune
disorder(eg, lupus, Sjogren syndrome, Sarcoidosis)
It typically an ACTM

Idiopathic transverse myelitis
 Transverse myelities without any clear etiology despite
through investigation.
 With clear inclusion and exclusion criteria

Clinical presentation
 Consider the age and gender
 Older patients(>50) more likely to suffer spinal cord infarction.
 Female patients higher risk of developing TM.
 TM typically has acute to sub acute onset
 Neurological features reaching the nadir within a week.
 An apoplectic onset with deficit reaching the nadir in less than 4hours
indicates a vascular event.
 Deficit continue to worsen beyond 4 weeks is uncharacteristic of TM.

Clinical presentation cont…
 Sensory symptoms both positive and negative are common
 Circumferential band of dysesthesia attributable to the
dermatome rostral to the sensory level, around their trunk .
 This may be associated with a constricting sensation that ranges
from mild discomfort to severe spasmodic or burning pain(MS
hug)
 Also called Anaconda squeeze
 TM pain may central, deep aching or radicular in nature.
 Lhermitte phenomenon – paresthesia traveling down the
limbs and trunk with neck flexion
 Reverse Lhermitte – paresthesia with neck extension indicates
extra axial compressive lesion
 Inverse Lhermitte – paresthesia traveling upward

Paraneoplastic syndrome
 Anti-Ri(ANNA-2) antibody
 CRMP-5 IgG antibody
 Anti- amphiphysin antibody
 Anti- GAD antibody
 NMDAR antibody


Causes of TM
 Atopic myelitis
 Drugs and toxin
 TNF-alpha inhibitors
 Sulphasalazine
 Epidural anesthesia
 Chemotherapeutic agents: Cytarabine , Cisplatin
 Heroin
 Benzene
 Idiopathic TM

IDIOPATHIC TM
 TM without any clear etiology despite a through investigations.
 The reported proportion of patients with TM and ITM varies widely
from 16% to approximately 60%.
 The mean age of disease onset appears to be between 35 and 40 years,
with female preponderance.
 MRI typically demonstrate a cervicomedullary lesion , extending over
2 vertebral segments and involving more than two-thirds of cross
sectional area of the spinal cord.
 Cord swelling seen in half of cases, gadolinium enhancement seen 1/3
to ½ of cases .
 CSF shows increased protein in most cases, pleocytosis and OCB
sometime seen.
 Typically monophasic but recurrence in ¼ to 1/3 of cases.

Idiopathic TM cont…
 Risk factor for recurrence
 Male gender
 Age older than 50 years
 Severe motor weakness and sphincteric disturbance
 Negative csf OCB, normal IgG index, NMO IgG
seronegativity
 Recurrence are associated with poor outcome

Pediatric TM
 Incidence in children much lower than adult
 Bimodal distribution
 Toddlers younger than 3 years
 Children between 5 and 17 years
 Males and females are equally affected
 Antecedent infections(usually URI) or preceding
vaccination are common.
 Because of URI clustering common in winter season.

Pediatric TM
 More frequently post infectious, thoracic,
centromedullary, and longitudinally extensive
 Risk of conversion to MS is lower
 Functional recovery often better than adult population.
 Complete recovery appear to be the rule , poor outcome
only in minority
 The course in the pediatric TM 3 phases
1 .Onset 2. plateau 3 recovery

Pediatric TM cont…
 The plateau may last for 4 weeks. If recovery not started
by the end of this period chance recovery less.
 CSF frequently reveals pleocytosis and elevated protein.
OCB and IgG indices are less.
 Early treatment with iv methyl prednislone had a
significant positive outcome
 Most common long term neurological complication in
bladder dysfunction

Sjogren syndrome
 Chronic, protean, progressive, systemic autoimmune disorder
 Wide range of neurological manifestation can occur – including
AON, and TM
 The prevalence of neurologic involvement 8.5%to 70%.
 Spinal cord involvement occur in 20 to 35%.
 The lesion tends to affect cervical cord, may be longitudinally
extensive.
 Spinal cord involvement often refractory to steroids. Iv
cyclophosphamide is effective.

Systemic lupus erythematosus
 Is a systemic, chronic, autoimmune disorder
 TM accounts for 1 to 2% of the cases, but constitute most devastating complication.
 Tends to occur within first 5 years from the diagnosis.
 AON, and brainstem may accompany TM
 Mimic MS and representing a diagnostic confusion.
 SLE related myelopathy produce 2 different pattern
 Grey matter myelitis
 White matter myelitis
 Most common MRI finding in SLE related myelopathy
 Longitudinally extensive T2 hyper intense lesion
 Severe causes entire spinal cord may involve, may extend up to medulla
 A third of patients do not have MRI finding

Grey matter White matter
Presentation LMN features with urinary
retention
UMN features
Prodrome – fever, nausea,
vomiting
Frequent Infrequent
Clinical course More rapid deterioration:
More severe weakness at
nadir
LMN features persist beyond
the time expected for spinal
shock
More aggressive immuno
therapy needed
Less severe clinical
deterioration, longer time to
reach nadir, less severe
weakness at nadir
Longterm disability Greater Less
CSF Neutrophilic pleocytosis,
higher protein,
hypoglycorrachia
Mild pleocytosis, mildly
elevated protein, normal
glucose

MRI Cord swelling, frequent
LETM,less frequent
gadolinium enhancement
Infrequent cord
swelling: less frequent
LETM, more frequent
gadolinium
enhancement
Recurrence Very rare More than 70% of patients
Prior optic neuritis Absent Frequent
Coexisting NMO-IgG
seropositivity
None Frequent
Higher SLE disease
activity
Frequent infrequent
Difference between gray and white matter myelitis

Evaluation and diagnosis
 MRI of complete spinal axis mandatory to exclude
structural lesions.
 Spinal cord cephalad to the suspected level of lesion
always to be imaged to rule out misleading signs.
 The most sensitive MRI sequence for detecting spinal cord
lesions
 Short-tau-inversion recovery(STIR) fast spin –echo
 T2 weighted fast spin echo sequences

Evaluation cont…
 TM may be
 Longitudinally limited
 Longitudinally extensive
 The location and length of cord on MRI gives clues about
underlying disease.
 Longitudinally limited TM span 1 or 2 seg
 ACTM
 APTM

Evaluation cont…
 ACTM
 Complete spinal cord syndrome
 On axis section full thickness involvement or
 Central portion of the cord maximally involved
 APTM
 Asymmetric spinal cord involvement
 Neurological deficit attributable to specific tract
 On axis section involvement of portion of spinal cord
 Increased risk of recurrence and transition to MS

LETM
 Extends 3 or more vertebral segments
 On axial section it typically involves more than two-thirds of
the spinal cord thickness
 Differential diagnosis include
 NMO spectrum disorders
 ADEM
 SAID- SLE,SS, neuro sarcoidosis, neur Behcet disease
 Parainfectious TM
 Para neoplastic

Mimics of LETM
 Neoplasms : primary intra medullary spinal cord
tumors, metastic tumors and lymphomas
 Radiation myelitis
 Metabolic myelopathies:B12 deficiency, copper
deficiency, nitrous oxide toxicity
 Vascular myelopathies
 Anterior spinal artery infarction
 Spinal dural arteriovenous fistula

Clinical evaluation
 Meticulous history and detailed clinical examination are
indispensible.
 An antecedent infection or prior vaccination may indicate ADEM or
parainfectious TM.
 Travel abroad indicate exotic infectious causes of TM, schistosomiasis.
 Concomitant malignancy indicate a Para neoplastic pathology.
 Women in the reproductive age are at higher risk of acquired
demyelinating disease and SAID.
 A history of relapsing and remitting attack of neurologic deficit such
as AON, INO suggest MS.

Evaluation cont…
 Serum B12, Thyroid function test, syphilis, HIV
serologys always obtained to evaluate treatable causes
of myelopathy.
 Vitamin E, serum copper and ceruloplasmin levels
checked.
 Serum aquaporin-4 specific auto antibodies should be
checked in all patients with TM because of its high
specificity for NMO or NMO spectrum of disorder.

Investigation into suspected TM
 Must be obtained for all cases
 MRI of the spine
 Brain MRI
 CSF: cells,diffrential, protein, glucose, VDRL, IgG index,
oligoglonal bands, cytologic analysis
 Serum B12, methylmelonic acid, HIV antibodies, syphilis
serologies, TSH, 25-hydroxy vitamin

Investigations cont…
 Must be obtained for all patients with LETM
 Serum NMO-IgG
 ESR,C-reactive protein, ANA, antibodies to extractable
nuclear antigen, RA factor, APL antibody,and ANCA
 VEP

Investigations cont…
 May need to be obtained
 Neuro- ophthalmological examination
 Paraneoplastic panel
 Infectious serologies and csf studies(culture,pcr study)
 Serum copper and ceruloplasmin
 Serum vitamin E level
 CT of chest
 Nerve conduction study and EMG
 Minor salivary gland biopsy

TM with normal MRI
 Clinical picture consistent with ATM but MRI is normal.
 The quality of imaging study must be evaluated
 Motion degradation and studies performed on low field
strength or open MRI – suboptimal in identifying spinal cord
lesions
 If the image quality is questionable – repeat imaging with
sedation, possibly with general anesthesia to reduce motion
artifact or using superior scanner can reveals missed lesion

Cont…
 Not all pathologic processes can be visualized by MRI.Sometime lesion can be
identified using non conventional technique – magnetization transfer
ratio(MTR)
 On occasion Ascending inflammatory poly radiculopathy or AIDP can mimic
myelitis
 NCS and somatosensory evoked potential helps to differentiate central and
peripheral etiologies.
 MRI may be normal: FA, ALS, B12 deficiency, copper deficiency myelopathy,
HSP, HTLV 1 associated myelopathy, adrenomyeloneuropathy

Spinal cord biopsy
 Restricted investigation
 Myelitis/myelopathy associated with enlarging lesion
 Treatment unresponsive lesion
 For detection of infection, granulomatous disease or neoplasm

Initial treatment
 Determined by
 Presenting clinical symptom
 The appearance on MRI
 The finding on CSF
 Once an inflammatory etiology identified by CSF analysis, the
clinician must decide whether or not infection is a etiology
 Any systemic symptoms suggestive of infection(fever, chills, rash etc.)
a thorough infectious workup essential.

Cont…
 If CSF results suggestive of infective cause appropriate antibiotics, or
antiviral agents should be started as soon as possible.
 Empirical steroids may be beneficial in tuberculoses meningitis and
some bacterial infections.
 If clinical symptoms, CSF profile, and appearance on MRI are
indicative of an autoimmune or inflammatory disease – serological
workup for SAID should be done.
 Iv steroids with or without immunosuppressive therapy may be
beneficial.
 In severely affected patients plasma exchange, iv immunoglobulin
indicated

Conclusion
 The term ATM initially applied to idiopathic causes.
 Now recognized as clinical syndrome associated with
multiple etiologies
 ITM remains the default diagnosis for unexplained non
compressive myelopathy with radiographic or imaging
evidence of inflammation

cont….
 In a recent large case series of 170 patients with acute non
compressive myelopathy
 40.6% (69/170) patients had an identifiable cause on initial evaluatioN
 On follow up etiology was secured in 71.2% (121/170) of cases.
 The most commonly identified cases were
 Demyelinating disease (MS 27%, NMO 6%)
 Infarction 15%
 Para infectious myelitis 12%
 SAID 8 % ( SS, SLE)

Cont…
 Effective interpretation of
 clinical symptoms and signs
 High quality neuroimaging
 Biomarkers such as CSF IL6 levels
 NMO IgG
 Help to identify the cause and guide in treatment
 In the absence of definitive diagnosis emprical treatment in iv corticosteroid,
plasma exchange and possibly immunosuppressant may be warranted
 Validated diagnostic criteria , biomarkers and improved imaging will enhance
study of acute ATM .
 With improved understanding treatment tailored to the underlying disease
can be developed.

Approach to myelopathy

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