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