management of non compressive myelopathy By Dr Sankalp ,DM resident GMC kota under guidance of Dr Vijay Sardana (HOD,Neurology)

1. EVALUATION AND DIAGNOSIS OF
NON COMPRESSIVE
MYELOPATHY
SANKALP MOHAN
SENIOR RESIDENT
NEUROLOGY

2. SPINAL CORD CROSS SECTION

3. MYELOPATHIES CLASSIFICATION

4. SPINAL CORD SYNDROMES
• COMPLETE CORD SYNDROME
• (TRAUMA ,TRANSVERSE MYELITIS)
• BROWN SEQUARD SYNDROME
(HEMICORD)
• (TRAUMA COMPRESSION,MS)
• ANTERIOR CORD SYNDROME
• (SPINAL ARTERY INFARCTION)

5. • POSTERIOR CORD SYNDROME
• (VIT B12 DEFICIENCY, COPPER DEFICIENCY)
• CENTRAL CORD SYNDROME
• (SYNRINGOMYELIA,INTRAMEDULLARY
TUMORS)
• CONUS AND CAUDA EQUINA

6. COMPRESSI
VE
EXTRAMEDULLARY
INTRAMEDULLARY
NON
COMPRESSI
VE
INFLAMMATORY
NON INFLAMMATORY

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

10. ACUTE TRANSVERSE
MYELITIS

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


19. INFECTIOUS CAUSES

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


24. OTHER CSF STUDIES

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


30. MULTIPLE SCLEROSIS
NEUROMYELITIS
OPTICA

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.


33. DIAGNOSTIC CRITERIA

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

39. VASCULAR

BLOOD SUPPLY OF SPINAL CORD

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)


44. METABOLIC
MYELOPATHIES

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


49. AXIAL T2 WEIGHTED
SAGGITAL T2 WEIGHTED

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

52. FOLATE DEFICIENCY
CAN alone cause myelopathy (less common)
 Alcoholics, neural tube defects,GI
disease,pregnancy ,drugs – trimethoprim
 Myelopathy,neuropathy,optic neuropathy
,cognitive decline
 Serum folate ,red cell folate(more reliable)
serum homocysteine
 Oral folate 1mg tds f/b 1mg per day


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

56. OTHER TOXIC MYELOPATHIES
Chemotherapy induced
 Hepatic Myelopathy
 Heroin myelopathy
 Fluorosis 

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


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
