brief review of diiferent causes of non compressive myelopathy and their characterstics

1. PRESENTED BY:
DR. HIRDESH CHAWLA
JUNIOR RESIDENT III

2. COMPRESSIVE
EXTRAMEDULLARY INTRAMEDULLARY
NON
COMPRESSIVE
INFLAMMATORY NON INFLAMMATORY

3. Features Compressive Non compressive
Bone deformity + -
Bony tenderness + -
Girdle like sensation + -
Upper level of Sensory
loss
+ -
Root pain + -
Onset and progress Gradual May be acute
Bladder and bowel
involvement
Early Usually late(early in
ATM)
Symmetry Asymmetrical(U
shaped)
Symmetrical

4.  TIME COURSE:
 Acute- within days(usually vascular, radiation, infection)
 Sub acute- 2-6 weeks
 Chronic- more than 6 weeks(degenerative, demyelinating , sarcoidosis , dural AV
fistula , metabolic , tabes dorsalis , HIV related)
 PHASE:
 Single event(most common)
 Multiphasic (rare)-Demyelinating, Vascular, Systemic disease
 EXTENT OF INVOLVEMENT:
 Monofocal- ATM
 Multifocal-ADEM,NMO,MS

5.  DISTRIBUTION:
 Gray matter-Poliomyelitis
 White matter-Leucomyelitis
 Whole cross sectional area-Transverse
myelitis
 Meninges and SC-Meningomyelitis
 Meninges and root-Meningoradiculitis

6.  Complete spinal cord: involvement of all the tracts (trauma,
compression or acute transverse myelitis).
 Brown Séquard syndrome: multiple sclerosis and compression.
 Anterior spinal cord syndrome: anterior spinal artery infarct and
multiple sclerosis
 Posterior spinal cord syndrome: vitamin B12 or copper
deficiency.
 Central syndrome: spino-thalamic crossing, cortico-spinal and
autonomic tracts (syringomyelia, neuromyelitis optica).
 Medullary cone: sacral emerging fibres (post-viral myelitis).
 Cauda equina: acute cytomegalovirus infection, polyradiculitis
 Tractopathies: vitamin B12 deficiency, paraneoplastic
myelopathy and multiple sclerosis.

7.  SACD
 HIV associated myelopathy
 Freidrich’s ataxia
 Tabes dorsalis

8. 1. INFECTIOUS- Viral, Bacterial, Fungal and
Parasitic
2. AUTOIMMUNE- SLE, Sjogren’s, Sarcoidosis,
Behcet Syndrome ,MCTD, Poly Arteritis Nodosa, p
ANCA positive vasculitis.
3. DEMYELINATING- MS,NMO, ADEM, Post viral
post vaccinial
4.PARANEOPLASTIC
5. ENCEPHALOMYELITIS

9. INHERITED- HSP, Inherited metabolic disorders
METABOLIC- Vit. B12,Copper,folate and Vit. E
deficiency. AIDS Associated.
TOXIC –Cassava, Lathyrism, Fluorosis, SMON, Nitrous
Oxide
VASCULAR – Ant. Spinal artery thrombosis, AVM,
Dural AV fistula

10.  Excludes compressive etiology.
 There needs to be gadolinium enhancement
of the spinal cord for inflammatory pathology
 Brain MRI should be performed to determine
if other demyelinating lesions within (CNS)
are present ,MS and NMO specific lesions.

12.  NOT a myelopathy (Guillain Barre’s, Inflammatory
radiculopathy)
 MRI performed during convalescence period.
 Friedreich’s ataxia,
 Motor neuron disease,
 Vitamin B12 or copper deficiency myelopathy,
 Hereditary spastic paraparesis,
 HIV,HTLV-1

13. IMAGING FEATURES POTENTIAL DIAGNOSIS
Blood within the spinal cord(bright
and dark T1 and T2 signal)
Vascular malformation(cavernous
angioma or dural AV fistula)
Flow void within SC Dural AV fistula or AVM
Central T2 signal abnormality Venous hypertension
Ring enhancing lesion Infection or tumour(consider
steroids to rule out inflammation
process before biopsy)
Fusiform lesion over >3 spinal
segments
NMO
T2 bright lesion in white matter
,<2 SC segments,<50% of cord
diameter, Rostral caudal extent
MS

14.  Represent extensive involvement of the spinal cord, with abnormal T2 signal
traversing at least three vertebral body segments in length.
 Differential diagnosis
 NMO(typical)
 Multiple sclerosis- confluent short segment lesions mimicking LESC
 Immunological- Neurosarcoidosis,Sjogrens,SLE
 Behcet’s disease
 Paraneoplastic myelitis
 Post infectious
 Vascular- Infarction,Dural AV fistula
 Metabolic and radiation myelopathy


15.  A low CSF glucose concentration:-
 infection (fungal, bacterial,or mycobacterial),
 Isolated low in neurosarcoidosis, carcinomatosis, SLE
 CSF WBC count defines inflammatory myelitis.
 High immunoglobulin G index in the CSF-Inflammatory
 If none of these findings are present at the time of onset of
symptoms, MRI and lumbar taps must be repeated two to seven
days later.

16.  Pleocytosis-Infections, Inflammations, Demyelinating
disorders.
 Mononuclear cells in AV malformations, paraneoplastic
 Erythrocytosis- AVM,AVF,SLE,Behcet’s
 Elevated protein- Tumours, paraneoplastic, vascular,
radiation.
 CSF normal-Delayed progressive radiation myelopathy
 Bizzare giant cells- vacuaolar myelopathy of HIV.
 Pronounced pleocytosis, normal glucose,eosinophilia-
schistosomiasis

17. HISTORY AND EXAMINATION S/O MYELOPATHY
NON COMPRESSIVE
INFLAMMATORY NON INFLAMMATORY
COMPRESSIVE
NEUROSURGICAL
MRI WITH GADOLINIUM
ENHANCEMENT
CSF
ANALYSIS

20.  Incidence - up to 3 per 100,000 patient years (0.003%)
 Female preponderance
 Typically monophasic (1/4th recur)
 First peak between 10-19 yrs of age and other between 30-39 yrs. Of age
 Symptoms typically develop over hours to days and then worsen over days to
weeks(acute to subacute)
 Pyramidal(flaccid paraplegia), sensory(definite sensory level), and autonomic
dysfunction to varying degrees .
 Backache and progressive paraparesis are presenting complaints.
 Early bladder and bowel involvement.
 “band like” horizontal area of altered sensation on the TORSO and rarely on
neck.(but unlike compressive-no root pain, spinal tenderness).
 Plantars are extensor (maybe absent in spinal shock stage)

22.  CSF IL-6 has been described as a biomarker
to help predict disability in acute transverse
myelitis
 The sensitivity of NMOIgG is 70% whereas the
specificity approaches 100%.

23.  MRI findings include focal and central high
signal areas in T2 sequences, occupying more
than two thirds of the spinal cord axially, and
extending over three to four segments,
generally in the thoracic spine.
 Spinal expansion may or may not be found
and, in general, there is contrast medium
enhancement, usually patch-like or diffuse.
 MRI findings are usually normal in 40% of
cases

28. WHEN TO SUSPECT SPINAL CORD INFECTION ?
 DEMOGRAPHIC FACTORS –
 Residence in endemic areas,
 H/o exposures
 blood transfusion
 chemotherapy
 transplant recipent (CMV,HHV7)
 CLINICAL CLUES – other systems –
 Lymphadenopathy
 Retina (CMV),
 Pharynx(EBV),
 Lung (cryptococcus, TB),
 Vesicles (HSV, Entero virus 71),
 Erythema migrans(Lyme’s) and
 Neurologic- meningoencephalitis, encephalopathy
 Recurrent genital infections-Behcets

29.  Enterovirus(Coxsackie and polio),West Nile Anterior horn
cells, motor nucleus of brainstem
 Herpetic Dorsal root ganglion,extensive inflammatory necrosis
of SC
 Schistosomiasis causes intensely inflammatory and
granulomatous myelitis.Diagnosed by elevated titres of Ab
against schistosome.
 HSV-2 produces a distinctive syndrome of recurrent sacral cauda
equina neuritis(lumbosacral radiculitis) with urinary retention in
association with outbreaks of genital herpes (Elsberg’s
syndrome)
 *Hence disturbance of function are either in sensory or motor
NEURONS rather than tracts

30.  Myelitis that presents as dysfunction of motor
and sensory tracts is rarely viral but rather due to
Non infectious inflammatory pathology BUT
EXCEPTION BEING
 Zoster myelitis
 HIV associated Vacuolar myelopathy
 HAM(Tropical spastic paraparesis)
 Dumb rabies

31.  MYELOPATHY can be due to
 HIV- itself,
 Herpes Zoster,
 Tuberculosis,
 HTLV-1
HIV MYELOPATHY-
 DIAGNOSIS OF EXCLUSION
 Pathologically – vacuolar myelopathy , spongy degeneration , demyelination
(axons relatively preserved)
 Most severe in thoracic segments with posterior and lateral columns affected
diffusely
 SYMMETRIC PAINLESS SPASTIC paraparesis
 Lesions resemble SACD but B12 levels are normal
 HAART has no effect on myelopathy

32.  Slowly progressive with UMN signs
 Early bladder involvement with disorder of sphincter
control.
 Upper extremities are spared(except for lively tendon
reflexes)
 Preserved brainstem and mentation.
 Posterior column and corticospinal tract in thoracic cord is
most commonly involved.
 CSF shows –normal protein and glucose and increased
HTLV-1 antibodies

33.  Tabes dorsalis ,meningomyelitis, pachymenigitis, spinal vascular
syphilis
 TABES DORSALIS- less than 5% of neurosyphilis
 Post. columns and spinal roots
 Preataxic - Lightening pains of the legs, ARP, urinary
incontinence
 Ataxic phase- sensory ataxia ,slapping gait
 Paralytic phase
 CSF shows-pleocytosis, increased protein,increase in IgG with
OGB, serological tests

35. • Most common cause of ATM
• Temporal relationship to infection or vaccination
• Development in days to 2 weeks when patient is resolving from
febrile epsiode
• Monophasic temporal course
• Varying degrees of weakness, ascending sensory symptoms,
sphincter disturbances.
• 40% give a positive H/O infection.(EBV,CMV and Mycoplasma most
common. NOT Campylobacter jejuni)
• Slight asymmetric of symptoms and signs, sensory level on trunk
and Babinski positive distinguishes it from GBS

36. • CSF- WBCs 10 – 100/mm3,Normal glucose,
Raised protein ,Pauci inflammatory also. Absent
oligoclonal bands
• MRI –Minimal gadolinium enhancement with
slight T2 signal abnormalities over 2-3 segments.
 POSTVACCINE MYELITIS-occurring in the 3 weeks
following a vaccination, such as smallpox
,hepatitis B, typhoid, influenza, rubella, and
tetanus

37.  ADEM-Monophasic disorder that affects the brain and occasionally the spinal cord.
 History of preceding viral or other infectious illness.(not definite criteria for
diagnosis)
 Show diffuse demyelinating lesions that are generally of the same age.
 Usually include encephalopathy but may also include focal or multifocal
demyelinating inflammatory syndromes of the CNS such as optic neuritis and
myelitis.
 ADEM is a differential diagnosis for isolated demyelinating syndrome, which is a
more common precursor of MS in adults .
 ADEM symptoms include rapidly progressing encephalopathy associated with
seizures or multiple neurologic deficits.
 The spinal cord is affected in 11% to 28% of patients, generally in the thoracic and
cervical segments

38.  There are no diagnostic criteria, but ADEM must be suspected when one
or more of the following are present :
 •Initial multifocal presentation with multiple symptoms.
 • Less than 10 years of age.
 • Signs and symptoms of meningoencephalitis.
 • Encephalopathy.
 • Bilateral optic neuritis.
 • CSF pleocytosis without oligoclonal banding.
 • MRI shows lesions in areas not affected by MS, such as the grey
matter or the cortex.
 • Lesions on MRI appear larger with poorly defined edges that enhance
with gadolinium

41.  Lesions are usually small (<2 vertebral
segments in length) and peripheral.
 Cause asymmetric symptoms and signs
 Lhermitte sign
 Acute spinal MS is relatively painless and
without fever and improves with residual
signs.

42.  Polman et al. reviewed McDonald’s diagnostic
criteria in 2010 and proposed the following :
 Space: One or more lesions with and without
gadolinium enhancement in two of the
following areas:
 periventricular,
 juxtacortical,
 infratentorial, or
 spinal cord.
 Time: One new lesion on T2 sequences or a
gadolinium enhancing lesion when compared
to the previous MR image, and concomitant
finding of asymptomatic lesions with or
without enhancement.

43.  CSF oligoclonal bands (OCBs) are present in more
than 90% of patients(may be absent after first attack)
 Immunoglobulin (Ig)G index is seen in more than
60%. following equation: IgG Index -(CSF
IgG/albumin)/(serum IgG/albumin). Ratio -0.3 and
0.6
 Subclinical optic nerve involvement on visually evoked
response testing
 Advanced neuroimaging such as diffusion tensor and
magnetization transfer imaging may help identify the
involvement of the apparently normal white matter,
which is abnormal in MS and normal in ADEM.

44.  On the sagittal plane, the plaques may be anterior,
central or posterior.
 Acute lesions enhance with gadolinium, due to
rupture of the blood-brain barrier. This enhancement
is less in cerebral lesions.
 Unlike neuromyelitis optica, viral or idiopathic
myelitis, in MS no black holes are visualized in the
spinal cord .
 NAA has been has been found to be reduced on
spectroscopy, in spinal cord areas that appear normal
on conventional MRI.

46.  Lesions are centrally located and necrotic
leading to more symmetric symptoms and
signs and greater disability
 NMO is relatively more common in Asian and
African individuals, Female preponderance
with mean age of 40 yrs.
 Autoimmune conditions including SLE,
SjoGren syndrome, and thyroid autoimmune
disorders may coexist

48.  Combination of Optic neuritis with Myelitis
also occur in:
 Multiple sclerosis
 ADEM
 Sjogren’s syndrome
 SLE
 Rarely with viral and bacterial infections.
 Paraneoplastic (Ab to CRMP 5)

49.  Radiological characteristics include a central longitudinal
and extensive cervicodorsal lesion (three or more spinal
segments) with spinal expansion, of low signal in T1
sequences and high signal in T2 sequences and patchy
enhancement.
 It has been demonstrated that 60% of patients may have
periventricular lesions (areas of high aquaporin 4
concentration).
 In this case, NMO is not associated with cerebral white
matter lesions, and the spinal lesions are confluent and
extend to multiple segments (which is infrequent in MS);
 Cranial nerve and cerebellar involvement is common in MS
and is not present in NMO.

50.  NMO-IgG -against Aquaporin 4(water
channel protein)recently identified serum
antibody highly specific (>90%) and sensitive
(>70%) for NMO.
 Typically oligoclonal bands are absent(unlike
in MS)

52. Acute
Demyelin
ating
Disease
MRI SPINE MRI BRAIN CSF
Multiple
sclerosis
<2 spinal
segments,peripherally
located,predilection for
posterior and lateral funiculi
White matter lesions,
Dawson fingers,
juxtacortical,
periventricular
OGB and raised IgG
index
NMO >3 segments, gadolinium
Enhancement and cord
swelling.T1 dark lesions and
Bright spotty lesions
In 60% of pts. Usually
periventricular. sometimes
hypothalamic or
brainstem
Prominent pleocytosis
with PMN or eosinophil
predominance, no OGB
in 80%, normal IgG
index
ADEM Variable lesion length Large, confluent white
matter lesions. lesions of
same age
Pleocytosis..OGB and
IgG index may be
abnormal transiently
Idiopathic
transverse
myelitis
Variable lesion length No brain lesion Pleocytosis..OGB and
IgG index may be
abnormal transiently

54.  CNS involvement -5% of cases, 18% with myelopathy
 Asymmetrical ascending paraparesis with bladder involvement in most
patients.
 Subacute or chronic,relapsing , slowly progressive polyradiculopathy ,
myelopathy
 Gadolinium enhancement of active intramedullary lesions.
 CSF-increased cells and protein, normal glucose, increased IgG levels
and activated histiocytes.
 Characterstic lesion is a multifocal-subpial nodular enhancement of
meninges adjacent to lesion within the cord or nerve roots.
 CXR PA ,ACE levels specificity at 80 to 95 the sensitivity is
60%.Definitive diagnosis requires biopsy.

55.  Systemic Lupus Erythematosus -1 to 2% of patients with SLE
 The most accepted hypothesis is a vascular mechanism
secondary to ischemic lesions
 ANA , APLA antibody is positive in 43 to 73% with myelitis
 patients with NMO ANA was positive in 52.6%
 CSF shows mild lymphocytic pleocytosis. Oligoclonal bands are
variable finding
 Sjögren’s Syndrome - 35% of cases have spinal cord involvement.
Includes episodes of optic neuritis but no oligoclonal bands

57.  Subacute myelopathies
 MAY OCCUR BEFORE DETECTION OF CANCER
 Amphiphysin- specific antibodies raise the
possibility of breast cancer.

58. • Lesions are necrotic involving both grey and white
matter
 The lesion often involves the thoracic spinal cord
extending one or several contiguous segments, that
shows a high-intensity signal in T2 sequences and
gadolinium enhancement.
 In contrast,nodular enhancement seen in intramedullary
metastasis or extradural mets. With cord compression
 There is increased protein concentration in the CSF with
few mononuclear cells. No tumour cells in CSF
• Anti YO, Anti Tr, Anti Hu antibodies, Anti GAD and anti
Amphiphysian.

61.  BLOOD SUPPLY OF SPINAL CORD

62.  1% of all strokes, 5% of acute myelopathies
 6th to 7th decade
 CAUSES:
 Atherosclerosis ,
 surgery of aorta,
 systemic hypotension,
 Iatrogenic causes- vertebral angiography, spinal trauma
 Relative hypovascularity of thoracic cord(>60% of SCI
occur)

63.  Pain(often radicular) and sensory symptoms first
 Clinical Nadir within 12 hrs
 Anterior spinal artery syndrome –symmetric weakness
with B/l Spinothalamic with bladder involvement.
 Post. spinal artery ischemia –rare
 Frequent overlap of signs .
 Can be devastating and life threatening

64.  Arterial thrombosis: aortic surgery, spinal angiography,
vasculitis , embolism, arterial dissection, hypotension, and
prothrombotic states.
 Anterior spinal artery lesion: anterior spinal syndrome
 Posterior spinal artery lesion: posterior column syndrome
 Subcommisural artery lesion: Brown Séquard syndrome
 Arteriovenous fistula- Weakness with Upright posture or
walking
 Venous infarct

65.  CSF is normal, although in arteriovenous
fistulas there may be higher protein
concentrations without pleocytosis .
 Spinal MRI shows single central hyperintensity

66. Type of vascular lesion MRI findings
Anterior spinal artery occlusion Elongated pencil like lesion in anterior
cord
Posterior spinal artery occlusion Triangular lesion in posterior cord
Subcommisural Artery Lateral cord lesion
Hematomyelia Appearance of blood products, Flow
voids in the cord
Fibrocartilaginous disc embolism Loss of vertical IV disc height,
microfractures in vertebral
endplates,T2 signal abnormality
AV fistulas Long spinal cord lesion extending
into conus,tortuous vessels.Spinal
angiogram needed to confirm

69. - Despite widespread screening Vit . B12
deficiency is – 15-25% of older individuals
- ETIOLOGY :-
- malabsorptive disorders
- atrophic gastritis,
- H2 antagonists and metformin,
- fish tapeworm

70.  Fatigue, generalised weakness
 Slowly progresive myelopathy
 Mild sensory symptoms with loss of vibration and proprioception
sense(POSTERIOR COLUMN)-First manifestation
 Paraparesis with hyperreflexia and spasticity(PYRAMIDAL)
 Bladder bowel also can occur
 Associated PERIPHERAL NEUROPATHY
 Psychological symptoms ,cognitive decline
 Optic neuropathy

71.  CBC- macrocytosis ,pancytopenia ,MCV (only in
40%)
 Vitamin B12 levels –lacks sensitivity and
specificity
 1/3 rd of cases with normal Vit. B12 levels have
elevated homocysteine and MMA levels
 Subclinical Vit. B12 deficiency occurs with age
 Low levels with neurologic manifestations – cause
and effect relation poor

72.  MRI SPINE –hyperintense T2 WEIGHTED signal
in posterior and anterolateral columns
without contrast enhancement. INVERTED V
SIGN

74. Laughing gas used in anasthesia
N2o interferes with metabolic pathway of methionine synthesis
Symptoms similar to vit. B12 deficiency – usually acute
Myelopathy, neuropathy, myeloneuropathy,impaired cognitive function
More prone in already vit. B12 deficient
Prophylactic use of vit. B12 before anasthesia

75.  CAN alone cause myelopathy (less common)
 CAUSES:-
 Alcoholics
 GI disease
 pregnancy
 drugs – trimethoprim
 Myelopathy, neuropathy, optic neuropathy ,cognitive
decline
 Serum folate ,red cell folate (more reliable) and serum
homocysteine levels for diagnosis

76.  Posterior and lateral columns are affected with reduced
ankle reflexes
 Causes-
 Gastric surgery
 Zinc toxicity
 TPN
 Malabsorption
 Hypocupremic anemia with ringed sideroblasts with
vacuolated myeloid precursors in marrow mimiking MDS
 Clinical -Myelopathy, myeloneuropathy
 Diagnosis-serum ceruloplasmin, serum or urinary copper

77.  LATHYRISM – Lathyrus sativus ,toxic amino acid(B oxalyl amino
alanine) from grass pea
 Spastic paraparesis with degenerative changes in spinal cord
 Preventable- avoid pure grass consumption, mix with cereals
 KONZO- poorly processed cassava
 SEEN IN AFRICA
 Spastic paraparesis

78. - SMON- clioquinol was used as antiparasitic drug in Japan.
- Subacute paraparesis with optic atrophy. Inevitable death
- ORGANOPHOSPHORUS-
CAN CAUSE MYELOPATHY AND MYELONEUROPATHY
- Most imp content –TOCP(Tri ortho cresyl phosphate)
- Acute intoxication f/b latent phase of several weeks
- Progressive leg weakness –sensory motor neuropathy with
spacticity paraparesis
- RBC cholinesterase

79.  Chemotherapy induced
 Hepatic Myelopathy
 Heroin Myelopathy
 Fluorosis

81.  FAMILIAL SPASTIC PARAPLEGIA:
 3rd – 4th decade.
 Can occur in 1st decade too
 AD/AR/X-linked
 Sensory involvement is minimal,Bladder is
involved late in the illness.
 Amyotrophy, MR, Optic atrophy, cataracts,
epilepsy,peripheral neuropathy and deafness
 Survival is long because respiration is spared
 Only symptomatic therapy

83.  Amount of current, duration of contact, resistance offered by the skin
 Immediate or Delayed – few days to 6 weeks
 The deep white matter is the most affected since it comprises the cortex and the
subcortical arcuate fibers-when high ampere current flows through body
 Heating of tissue, Vasocclusive changes, demyelination, fracture.
 Involvement of anterior spinal artery and its branches
 SPINAL ATROPHIC PARALYSIS-delayed,focal muscular atrophy. gray matter
affected the most.because of low voltage current
 Lightening injury:
 Arborescent marks
 Limbs may be pale and cold or cyanotic
 Late presentation of an atrophic limb paralysis
 Also associated with severe motor polyneuropathy

84.  Upper thoracic cord
 Little or no brain inv.
 Posterior column > lateral column
 Decompression in hyperbaric chamber,
Symptomatic treatment

85.  MC - Mediastinal irradiation for Hodgkin’s disease
 Early transient(3 – 6 months after)-Lhermitte sign. Disappear
after few months. Spongy appearance of white matter with
demyelination.
 Delayed progressive/Slowly evolving amyotrophy- Between
12-15 months, sensory followed by motor symptoms
 PAIN IS ABSENT(+nt in spinal mets.)
 LESION IS EXTENSIVE in rostro caudal fashion than in vascular
and demyelinating myelopathy
 Coagulative necrosis,vascular changes, secondary
degeneration
 Could be avoided if 6000 cGy over 30 – 70 days not
exceeding 200cGy/day or 900cGy/week

86. • Painful root and cord symptoms
• Syphilis, Resistant meningitis, TB,
• Penicillin, Contrast, steroids,
• Thickening of Arachnoid, proliferation of connective
tissue and adhesion between arachnoid & dura.
• PERSISTANT PAIN commonly in lumbofemoral regions,
but weakness and atrophy are less frequent.
• CT/MRI contrast showing total or partial loss of spinal
subarachnoid space(candle guttering).Mri shows loss of
normal ring of CSF or localised loculations of CSF
• Degeneration of peripheral fibres of posterior and
lateral column