This document discusses the approach to evaluating and diagnosing myelopathy. It begins by defining myelopathy as spinal cord, meningeal, or perimeningeal damage or dysfunction. It then lists signs that strongly indicate or are consistent with but not diagnostic of myelopathy. Alternative diagnoses are also discussed. Common causes of acute myelopathy are then summarized, including multiple sclerosis, spinal cord infarction, and transverse myelitis. Features suggesting infectious etiology and patterns of spinal cord involvement are outlined. The document concludes by discussing compressive myelopathies and pearls for localizing spinal cord lesions.

1. APPROACH TO MYELOPATHY
The term myelopathy describes pathologic conditions
that cause spinal cord, meningeal or perimeningeal
space damage or dysfunction.
Dr Chandan kumar
VMMC& Safadrjung ,New delhi

2. Signs strongly indicating myelopathy
• Sensory level on torso
• Spinal tract crossed findings (e.g., unilateral
pyramidal signs with contralateral spinothalamic
findings)
• Spinal tract-specific sensory findings (e.g.,
selective spinothalamic findings with preserved
dorsal column findings; suspended band of
spinothalamic sensory loss)
• Urinary retention
• Neurology® Clinical Practice 2010;75(Suppl 1):S2–S8

3. Signs consistent with but not
diagnostic of myelopathy
• Glove-and-stocking sensory loss (consider
peripheral neuropathy)
• Hyporeflexia/hypotonia (consider peripheral
neuropathy)
• Unilateral or bilateral upper motor neuron
signs (consider brain or brainstem disorders)

4. Signs suggesting alternative diagnosis
• Spasms, rather than spasticity (consider stiff-
person syndrome)
• Paratonic rigidity (consider frontal lobe
disorder)
• Cognitive impairment (consider frontal lobe or
diffuse brain disorder)
• Dysarthria and dysphagia (consider brainstem
disorder, such as motor neuron disease)

5. * Conditions mimicing myelopathy
1.Myopathy or disorders of the Neuromuscular Junction, but the
absence of a sensory deficit rules them out.
2.Bilateral mesial frontal lobe lesions
(e.g., bilateral anterior cerebral artery distribution infarcts) could
mimic a myelopathy, abulia or other signs of frontal lobe
dysfunction typically coexist.
3.Autoimmune or paraneoplastic muscle stiffness syndromes,
such as stiff-person syndrome associated with glutamic acid
decarboxylase or amphiphysin autoantibodies,
may be confused with spasticity.
4.Even ascending sensory symptoms of AIDP can confuse the
diagnosis
(till spinal shock)

6. LOOK FOR DURATION
Spinal cord pathologies may be classified as
acute, subacute/ intermittent or chronic,
depending on the time course.
Acute onset that worsens within hours or days
points to a spinal cord infarct or hemorrhage.

7. PATTERN OF SPINAL CORD DISEASE
• Complete spinal cord: involvement of all the tracts
(trauma, compression or acute transverse myelitis).
• Brown Séquard or hemi-spinal cord syndrome:
ipsilateral cortico-spinal tract, posterior columns
and contralateral spinothalamic tract (multiple
sclerosis and compression).
• Anterior spinal cord syndrome: anterior horns,
corticospinal, spinothalamic and autonomic tracts
(anterior spinal artery infarct and multiple
sclerosis).

8. • Posterior spinal cord syndrome: posterior
columns (vitamin B12 or copper deficiency).
• Central syndrome: spino-thalamic crossing,
cortico-spinal and autonomic tracts .Intact posterior
column (syringomyelia, neuromyelitis optica).
• Tractopathies: selective disorders (vitamin B12
deficiency, paraneoplastic myelopathy and multiple
sclerosis).
Foramen Magnum Syndrome

10. Incidence of etiology
In 2001, De Seze et al.
43% of acute myelopathies 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 (9).
De Seze J, Stojkovic T, Breteau G, et al. Acute myelopathies: Clinical, laboratory and outcome profiles in 79
cases. Brain. 2001;124:1509-21

11. ACUTE MYELOPATHY, WHAT
CAUSES SHOULD BE CONSIDERED
Recent onset symptoms, particularly ones
that evolve rapidly, exclude surgical emergency
such as epidural metastasis or abscess.
Immediate imaging -ideally with MRI of the
entire spine definitive management (i.e.,
surgery) should be pursued without delay.

12. ACUTE MYELOPATHY, WHAT
CAUSES SHOULD BE CONSIDERED
The most commonly identified causes (initially labelled as
idiopathic )-
1.Demyelinating disorders (MS and neuromyelitis optica),
2.Spinal cord infarction,
3.Parainfectious myelitis, and
4.Systemic inflammatory disorders (e.g., Sjo¨gren
syndrome and lupus).
*Transverse myelitis is the default diagnosis for an
unexplained myelopathy evolving over the course of days
to 3 weeks with subsequent stabilization or improvement.

14. One can confidently link the two only when
myelitis occurs concurrently or within days of an
infection known to be associated with myelitis
(e.g., zoster) or when investigation such as CSF
PCR demonstrates unequivocal evidence of CNS
infection.

15. HIV and Spinal cord
• Vacuolar myelopathy- most common cause of
spinal cord dysfunction in 25% to 55% of AIDS
autopsy series (Di Rocco and Simpson, 1998; McArthur et al., 2005).
• CMV myeloradiculopathy
• VZV myelitis
• Spinal epidural or intradural lymphoma
(metastatic)
• Human T-cell lymphocytotropic virus 1 (HTLV-1)-
associated myelopathy

16. Radiation and paraneoplastic
myelopathy
• The most common form of radiation
myelopathy, chronic progressive radiation
myelopathy, generally develops 1 to 2 years
after radiotherapy. (>60 Gy)
• .Paraneoplastic disorders are a rare cause of
myelopathy. The most common clinical
syndrome is a painless myelopathy
accompanied by encephalitis or sensory
neuropathy

17. Features suggesting an infectious
etiology
1. Fever, rash (zoster, enterovirus, Lyme disease),
2.Meningismus, a history of recent travel to
endemic regions(tuberculosis, parasitic infections
such as schistosomiasis),
3.Rabies exposure,
4. Immunosuppression (herpezoster,
cytomegalovirus).
5.Consider treatable infections such as syphilis, HIV,
tuberculosis, Lyme disease, and herpesviruses.

18. Myelopathy in Nutrition Deficiency
• Vitamin B12 (T1-weighted cervical and upper thoracic
magnetic resonance image showing marked
enhancement of posterior cord)
• copper (Common symptoms - lower-limb paresthesias
and gait difficulty. characterized by sensory loss and
sensory ataxia
Finding low serum copper level, low serum
ceruloplasmin and low urinary copper excretion, establish
the diagnosis of copper deficiency)
• vitamin E, folate,

20. Radiological Investigation
For noncompressive myelopathy, the results of
MRI can be broadly subdivided into 3 categories:
1. Short T2 hyperintensity (3 vertebral segments
in length)- Focal, discrete lesions that do not
occupy the entire cord in axial cross-section are
highly suggestive of MS.

21. Radiological Investigation
2. Longitudinally extensive T2 hyperintensity (>3
vertebral segments in length).
a.Idiopathic transverse myelitis,
b.NMO,
c. Acute disseminated encephalomyelitis,
d.Cord infarction, and
e.Myelitis associated with systemic diseases
such as systemic lupus erythematosus.

22. Radiological Investigation
Normal MRI.
should undergo careful review of the images for
subtle findings of cord signal change, atrophy, or
extrinsic compression by uncommon causes
(e.g., epidural lipomatosis)
Consider and test for degenerative, infectious,
and metabolic causes of myelopathy

25. COMPRESSIVE MYELOPATHY

26. CERVICAL MYELOPATHY
In cervical myelopathy first there is weakness of
ipsilateral arm,then ipsilstersl leg then
contralateral leg and lastly contra lateral arm
occurs. This U” shaped involvement of the limbs
is called Elsberg phenomenon.
(Foramen Magnum Syndrome).

27. SYRINGOMYELIA

28. PEARLS
• Persistent Areflexic paralysis with sensory level-
Necrosis of spinal segments
• Thoracic spinal level is most commonly involved
in spinal metastasis except for prostate cancer.
• C5C6 segment lesion:
1. INVERTED SUPINATOR REFLEX
2. wasting of muscles supplied byC5C6 namely
deltoid,biceps, brachioradialis,infra &
suprasinators&rhomboids

29. • C8T1 Level:
1. WASTING OF SMALL MUSCLES OF THE HAND.
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.

30. • 9th &10th thoracic segments:
. BEEVOR’S SIGN
*L3 L4 segmental lesion:
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

31. • S1S2 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.

32. S3S4 segments:
• Large bowel & bladder are paralysed.
• There is RETENSION OF URINE & FEACES due to
unopposed action of internal sphincters.
• The external sphincters are paralyzed.
• ANAL & BULBO CAVERNOUS REFLEXES ARE LOST.
• SADDLE SHAPED ANESTHESIA occurs.
• but no paraplegia

33. PEARLS A defined line-like level is not expected. The sensory mapping is
not as precise as that shown on dermatome charts.
The sensory loss is seldom complete, which makes precise localization even
more difficult.
The sensory level may not be at the same level on the two sides of the body—
a discrepancy of up to several levels can be seen.
Look for dissociated sensory loss due to crossed projections of
pain/temperature versus uncrossed touch/ proprioception projections.
Discrepancy in sensory level between posterior column and spinothalamic
levels can occur because of intersegmental projections of the axons of the
posterolateral (Lissauer) tract.
The sensory level may be much higher than might be expected from motor
examination or pain. This is because the lesion may be much higher than
indicated by the levels of clinical findings, reinforcing the basic precept that
the examiner must start from the level of the symptoms and look up!