7. NON COMPRESSIVE MYELOPATHY
INFLAMMATORY MYELOPATHIES –
MYELITIS
1. INFECTIOUS- VIRAL, BACTERIAL ,FUNGAL
PARASTIC
2. AUTOIMMUNESLE.SJOGREN,SARCOIDOSIS,BECHET
S,MCTD
3. DEMYELINATING- MS,NMO, ADEM, POST
VIRAL POST VACCINIAL
4.PARANEOPLASTIC
8. NON INFLAMMTORY MYELOPATHIES
INHERITED-
HSP,INHERITED METABOLIC
DISORDERS
METABOLIC-
VIT B12,COPPER,FOLATE ,AIDS
ASSOCIATED,VIT E DEFICIENCY
TOXIC – CASSAVA
,LATHYRISM,FLUOROSIS,SMON,NITROUS OXIDE
VASCULAR – ANT SPINAL ARTERY
THROMBOSIS, AVM, DURAL AV FISTULA
9. EVALUATION OF MYELOPATHY
HISTORY AND EXAMINATION S/O MYELOPATHY
MRI WITH GADOLINIUM
ENHANCEMENT
NON COMPRESSIVE
COMPRESSIVE
CSF
ANALYSIS
NEUROSURGICAL
INFLAMMATORY
NON INFLAMMATORY
INFECTIOUS
POST
INFECTIOUS
AUTOIMMUNE
SPINAL CORD
INFARCTION
AVM
11. EPIDEMIOLOGY
Incidence - up to 3 per 100,000 patient years
(0.003%)
no familial or ethnic predisposition
More common in females
12. CLINICAL FEATURES
symptoms typically develop over hours to
days and then worsen over days to weeks
pyramidal, sensory, and autonomic
dysfunction
to varying degrees
“band like” horizontal area of altered
sensation on the neck or torso
13. NEUROIMAGING
Excludes compressive etiology
Brain MRI should be performed to determine if
other demyelinating lesions within (CNS) are
present ,MS AND NMO SPECIFIC LESIONS
NORMAL MRI with myelopathy –
- not myelopathy
- Friedreich‟s ataxia, motor neuron
disease, vitamin B12 or copper deficiency
myelopathy, hereditary spastic
paraparesis, HIV,HTLV -1
14. CSF EXAMINATION
A low CSF glucose concentration
- infection (fungal, bacterial, or
mycobacterial),
isolated low in neurosarcoidosis
carcinomatosis, ,SLE
Elevated protein concentration 50% of
patients with transverse myelitis
CSF WBC count defines inflammatory
myelitis.
15. 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%.
16. PROGNOSIS
1/3rd of patients recover with little to no
sequelae,
1/3rd a moderate degree of residual disability
and
1/3rd remain severely disabled
Poorer prognosis in acute transverse myelitis
rapid progression of symptoms,
back pain,
spinal shock,
17. MANAGEMENT
ACUTE MANAGEMENT –
HIGH DOSE IV STEROIDS –
methylprednisolone 1 gm for 3- 7 days
If poor response to steroids –
plasmapheresis should be offered within 15
days
Has shown favourble outcome in
demyelinating and autoimmune diseases
18. SUPPORTIVE MANAGEMENT - SPASTICITY
BLADDER DYSFUNCTION
GI DYSFUNCTION
COMPLICATIONS DUE TO IMMOBILITY –
PRESSURE SORE,DVT
MALNUTRTITION,RESPIRATORY
THERAPY
20. SPINAL CORD INFECTIONS
WHEN TO SUSPECT SPINAL CORD
INFECTION ?
1. DEMOGRAPHIC FACTORS – residence in
endemic areas,h/o exposures,blood
transfusion ,chemotherapy ,transplant
recipent(CMV,HHV7)
2. CLINICAL CLUES – other systems –retina
(cmv) pharynx(ebv), lung
(cryptococcus,TB),vesciles (hsv), erthema
migrans(lyme .s) and neurologicmeningoencephalitis,encephalopathy
21. HIV INFECTION
MYELOPATHY can be due to
HIV- itself, Herpes
Zoster,Tuberculosis,HTLV-1
HIV MYELOPATHYDIAGNOSIS OF EXCLUSION
SYMMETRIC PAINLESS SPASTIC parapareis
with impaired joint position vibration
Concomitant neuropathy
22. Pathologically –vacoular myelopathy
..spongy degeneration .,demyelination
(axons relatively preserved )
ViT b12 deficiency may contribute
No effective treatment ..ART may reduce the
incidence
23. SYPHILIS
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
Ataxic phase- sensory ataxia ,slapping gait
Paralytic phase –
CSF VDRL
12- 14 MU OF AQUEOUS PENICILLIN G DAILY
-10-14 DAYS
25. DEMYLEINATING DISORDERS
MULTIPLE SCLEROSIS - lesions are
usually small (<2 vertebral segments in
length) and peripheral,
cause asymmetric symptoms and signs
Lhermitte sign
isolated proprioceptive loss of an upper
extremity („„sensory useless hand
syndrome‟‟),
Brown-Se´quard syndrome, or, more
commonly, incomplete involvement
26.
CSF oligoclonal bands (OCBs) are present in more
than 90% of patients,
other conditions that cause inflammation in the CNS,
including NMO, paraneoplastic disorders, SLE,
neurosarcoidosis, Behçet‟s disease
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
MRI brain FINDINGS predicts relapse
27. NEUROMYELITIS OPTICA
Lesions are centrally located and necrotic
leading to more symmetric symptoms and
signs,greater disability
NMO is relatively more common in Asian and
African individuals,
autoimmune conditions including
SLE, SjoGren‟ssyndrome, and thyroid
autoimmune disorders may coexist
28. NMO-IgG -recently identified serum
antibody highly specific (>90%) and sensitive
(>70%) for NMO.
Risk of developing recurrent myelitis or new
onset optic neuritis in patients with an LETM
was more than 50% in NMO-IgG seropositive
31. ACUTE DISSEMINATED ENCEPHALOMYELTIS
ADEM-monophasic disorder that affects the
brain and occasionally the spinal cord
history of preceding viral or other infectious
illness.
show demyelinating lesions that are generally of
the same age
POSTVACCINE MYELITIS-occurring in the 3
weeks
following a vaccination, such as smallpox
,hepatitis B, typhoid, influenza, rubella, and
32. ACUTE IDIOPATHIC TRANSVERSE MYELITIS
idiopathic nature is a diagnosis of exclusion
preceding nonspecific fever, nausea possibly
indicating a prior viral infection is common
16-60% of all Transverse myelitis
Female preponderance
Typically monophasic (1/4th recur)
may have an NMO spectrum disorder.
The lesion length varies from less than one
segment to the entire cord.
34. IMMUNE DISEASES
Sarcoidosis- CNS involvement -5% of cases
18% presented with myelopathy
Subacute or chronic ,relapsing
,polyradiculopathy ,myelopathy
plain films of the chest ,ACE levels specificity
at 80 to 95 the sensitivity is 60%.
35. Systemic Lupus Erythematosus -1 to 2%
of patients with SLE
ANA , APLA antibody that is positive in 43 to
73% with myelitis
patients with NMO ANA was positive in
52.6%
Sjögren’s Syndrome –
that 35%- spinal cord involvement
36. PARANEOPLASTIC DISORDERS AND
MYELOPATHY
subacute myelopathies
MAY OCCUR BEFORE DETECTION OF CANCER
search for antibodies and an underlying malignancy
warranted if other etiologies for the myelopathy are not
apparent
often produce tract-specific involvement/TM/recurrent
Ab to CRMP5 may lead to myelopathy and optic
neuropathy that may mimic NMO
Amphiphysin- specific antibodies raise the possibility of
breast cancer.
Detection and t/t of underlying cancer ,immunosupression
41. SPINAL CORD INFARCTION
1% of all strokes, 5% of acute myelopathies
6th to 7th decade
Atherosclerosis , surgery of aorta,systemic
hypotension, Iatrogenic causes- vertebral
angiography,spinal trauma
Relative hypovascularity of thoracic
cord(>60% of SCI occur)
42. CLINICAL FEATURES
Pain(often radicular) and sensory symptoms
first
Clinical Nadir within 12 hrs
Anterior spinal atery syndrome –symmetric
weakness with B/l Spinothalamic with bladder
Post spinal artery ischemia –rare
Frequent overlap of signs .
Can be devastating and life threatening
mortality rate of 20-25%
43. MANAGEMENT
High risk procedures serial neurologic
assesment
In Aortic manipulation –vasopressors
manitain MAP>90
MRI spine –r/o compression (epidural
hematoma).DWI more sensitive
CSF drainage maintain CSF pressure at 10
mm Hg
Antiplalelet used (trials not available)
45. SUBACUTE COMBINED DEGENERATION
-
-
Despite widespread screening vit b12
deficency is – 15-25% of older individuals
ETIOLOGY malabsorptive disorders, such as
poor nutrition, atrophic gastritis, H2
antagonists and metformin, fish tapeworm
46. CLINICAL FEATURES
Fatigue, generalised weakness
Slowly progresive myelopathy
Paraparesis with hyperreflxia and spasticity
Mild sensory symptoms with loss of vibration
,proprioception
Bladder bowel also can occur
Associated peripheral neuropathy
Psychological symptoms ,cognitive decline
Optic neuropathy
47. DIAGNOSIS
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
48. Tests for cause of deficiency-serum
gastrin, anti intrinsic factor antibody (elevated
gastrin in 30% elderly )
MRI SPINE –hyperintense T2 WEIGHTED
signal in posterior and lateral colums
50. TREATMENT
Oral or parenteral vitb12
NEUROLOGIC symptoms- high dose im
1000ug im for 2 weeks daily f/b 1000ug
monthly for 6 months
51. NITROUS OXIDE EXPOSURE
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
53. COPPER DEFICIENCY
Prosthetic group for various enzymes
Causes- gastric surgery,zinc toxicity
,TPN,malabsorption
Clinical -Myelopathy,myeloneuropathy
Diagnosis-serum ceruloplasmin,serum or
urinary copper
Supplementation with oral copper
54. TOXIC MYELOPATHIES
LATHYRISM – Lathyrus sativus ,toxic amino acid(B
oxalyl amino alanine) from grass pea
Spastic paraperesis with degenerative changes in
spinal cord
Preventable- avoid pure grass consumption,mix with
cereals
KONZO- poorly processed cassava
SEEN IN AFRICA
Spastic paraperesis
55. -
-
SMON- clioquinol was used as antiparasitic drug in
Japan
Subacute paraparesis with optic atrophy
ORGANOPHOSPHORUS- CAN CAUSE MYELOPATHY AND
MYELONEUROPATHY
Most imp content –TOCP
Acute intoxication f/b latent phase of several weeks
Progressive leg weakness –sensory motor
neuropathy with spacticity paraparesis
RBC cholinesterase
57. RADIATION ASSOCIATED MYELOPATHY
Radiation-induced myelopathies are usually
slowly progressive but may occur up to 15
years after the end of radiation treatment,
cord swelling or enhancement may be seen
initially but later atrophy
simultaneous involvement of the adjacent
vertebrae in the same field
58.
59. REFERNCES
Seminars in neurology –vol 28, Feb 2008
Seminars in neurology –vol 32, April 2012
Neurology clinics – (spinal cord diseases) feb
2013 vol 31
metabolic and toxic myelopathies –American
academy of neurology 2008
infectious and inflammatory myelopathies American academy of neurology
