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management of non compressive myelopathy By Dr Sankalp ,DM resident GMC kota under guidance of Dr Vijay Sardana (HOD,Neurology)
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EVALUATION AND DIAGNOSIS OF NON COMPRESSIVE MYELOPATHY
SANKALP MOHAN
SENIOR RESIDENT NEUROLOGY
SPINAL CORD CROSS SECTION
MYELOPATHIES CLASSIFICATION
SPINAL CORD SYNDROMES
• COMPLETE CORD SYNDROME• (TRAUMA ,TRANSVERSE MYELITIS)
• BROWN SEQUARD SYNDROME (HEMICORD)• (TRAUMA COMPRESSION,MS)
• ANTERIOR CORD SYNDROME
• (SPINAL ARTERY INFARCTION)
• POSTERIOR CORD SYNDROME
• (VIT B12 DEFICIENCY, COPPER DEFICIENCY)
• CENTRAL CORD SYNDROME
• (SYNRINGOMYELIA,INTRAMEDULLARY TUMORS)
• CONUS AND CAUDA EQUINA
COMPRESSIVE
EXTRAMEDULLARY
INTRAMEDULLARY
NON COMPRESSIVE
INFLAMMATORY
NON INFLAMMAT
ORY
NON COMPRESSIVE MYELOPATHY
INFLAMMATORY MYELOPATHIES –MYELITIS
1. INFECTIOUS- VIRAL, BACTERIAL ,FUNGAL PARASTIC
2. AUTOIMMUNE- SLE.SJOGREN,SARCOIDOSIS,BECHET S,MCTD
3. DEMYELINATING- MS,NMO, ADEM, POST VIRAL POST VACCINIAL
4.PARANEOPLASTIC
NON INFLAMMTORY MYELOPATHIES
INHERITED- HSP,INHERITED METABOLIC DISORDERS
METABOLIC- VIT B12,COPPER,FOLATE ,AIDS ASSOCIATED,VIT E DEFICIENCY
TOXIC – CASSAVA ,LATHYRISM,FLUOROSIS,SMON,NITROUS OXIDEVASCULAR – ANT SPINAL ARTERY THROMBOSIS, AVM, DURAL AV FISTULA
EVALUATION OF MYELOPATHY
HISTORY AND EXAMINATION S/O MYELOPATHY
NON COMPRESSIVE
INFLAMMATORY
NON INFLAMMAT
ORY
COMPRESSIVE
NEUROSURGICAL
MRI WITH GADOLINIUM ENHANCEMENT
CSF ANALYSIS
INFECTIOUSPOST INFECTIOUSAUTOIMMUNEMS RELATED
SPINAL CORD INFARCTIONAVMOTHER
ACUTE TRANSVERSE MYELITIS
EPIDEMIOLOGY
Incidence - up to 3 per 100,000 patient years (0.003%)
no familial or ethnic predisposition More common in females
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
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
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.
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%.
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,
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
SUPPORTIVE MANAGEMENT- - SPASTICITY BLADDER DYSFUNCTION GI DYSFUNCTION COMPLICATIONS DUE TO IMMOBILITY –
PRESSURE SORE,DVT MALNUTRTITION,RESPIRATORY THERAPY
INFECTIOUS CAUSES
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 neurologic- meningoencephalitis,encephalopathy
HIV INFECTION
MYELOPATHY can be due to HIV- itself, Herpes
Zoster,Tuberculosis,HTLV-1HIV MYELOPATHY- DIAGNOSIS OF EXCLUSION SYMMETRIC PAINLESS SPASTIC
parapareis with impaired joint position vibration
Concomitant neuropathy
Pathologically –vacoular myelopathy ..spongy degeneration .,demyelination (axons relatively preserved )
ViT b12 deficiency may contribute No effective treatment ..ART may
reduce the incidence
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
OTHER CSF STUDIES
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
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
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
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
MULTIPLE SCLEROSIS NEUROMYELITIS OPTICA
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 tetanus
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.
DIAGNOSTIC CRITERIA
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%.
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
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
VASCULAR
BLOOD SUPPLY OF SPINAL CORD
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)
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%
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)
METABOLIC MYELOPATHIES
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
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
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
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
SAGGITAL T2 WEIGHTED
AXIAL T2 WEIGHTED
TREATMENT
Oral or parenteral vitb12 NEUROLOGIC symptoms- high dose im 1000ug im for 2 weeks daily f/b 1000ug
monthly for 6 months
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
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
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
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
- 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
OTHER TOXIC MYELOPATHIES
Chemotherapy induced Hepatic Myelopathy Heroin myelopathy Fluorosis -
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
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