NON-COMPRESSIVE MYELOPATHY

G.Madhu ShankarProf.S.TITO’S UNIT

• An estimate states that there are 30 common diseases of Spinal cord.

• 15 are common in clinics.• Most of them follow syndromic pattern.

• Causes• Presentation• Prognosis• Management• 30 slides

CLASSIFICATION…• Myelitis(Inflammatory disease)

InfectiveNon-infective

• Vascular• Metabolic• Drugs and toxins• Physical agents• Degenerative

MYELITIS…• Poliomyelitis• Leukomyelitis• Transverse Myelitis• Disseminated• Meningomyelitis

• Pachy• Lepto

• Encephalomyelitis• Meningaradiculitis• Abscess/Granuloma

• Acute within days• Subacute 2 – 6 weeks• Chronic >6 weeks

Infective myelitis..• Viral

• Herpes group viruses(HSV,EBV,CMV,HZV)• Enterovirus(Cox,Polio)• HIV/HTLV 1 asso myelopathy(HAM)• Rabies,Arbovirus

• Non-viral(Bacterial,Fungal,Parasitc,Granulomatous)

• Mycoplasma• Pyogenic• Tuberculous• Syphilitic

• VIRAL MYELITIS:• Acute onset• Febrile meningomyelitis• Systemic manifestations• Cutaneous manifestations

• Enterovirus Anterior horn cells• Herpetic Dorsal root ganglion• HSV 2 lumbosacral radiculitis

• CSF• Isolation of Viral nucleic acid by PCR

• 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

NON INFECTIOUS INFLAMMATORY...• Postinfecious and postvaccinal• MS• Acute and subacute necrotizing myelitis and

Devic’s disease• Asso with connective tissue disorders• Paraneoplastic

• Leukomyelitis• Disordered immune response• Affect CNS,optic nerves

POSTINFECIOUS AND POSTVACCINAL..• Most common cause of ATM• Temporal relationship to infection or vaccination• Development in days• Monophasic temporal course

• Varying degrees of weakness,sensory symptoms,sphinter disturbances

• Back pain• 40% give a positive H/O• Mean age 3 – 4 th decade

• CSF cells 10 – 100/mm3,Normal glucose,Raised protein• Pauci inflammatory also. Absent oligoclonal bands• MRI enhancement

Variants…• Pure conus syndrome• Partial Brown sequard syndrome• Variable sensory loss over LL• Pure Posterior column dysfunction• Mimicking ASA thrombosis(Motor & spinothalamic inv alone

wit preserved deep sensation)Pathologically Demyelination with inflammatory infiltrates

• Treatment:• Steroids in high doses• IV Ig or plasma exchange

• Prognosis:• Better..Improvement occurs.• Acute onset/Mid thoracic Pain portend poor prognosis

MS(DEMYELINATIVE)• No temporal relation• Slow evolution• Remitting and relapsing course• Disseminated in Time and Space• Optic neuritis• Age 20 – 40 yrs• MRI(MTR/MRSI)• CSF for Oligoclonal bands and CSF IgG

index(Intrathecal IgG)• CSF Cells >75/uL,PMN,Protein >100 mg/dL cast a

doubt.

• MANAGEMENT• Acute attack

• IV Methyl Prednisolone• Plasma Exchange

• Disease modifying drugs• IFN-B-1a,1b• Glatiramer acetate• Natalizumab• Immunosuppresants

• Prognosis» Individualised»Grave disability»Direct mortality rare.

ACUTE AND SUBACUTE NECROTIZING MYELITIS AND DEVIC’S DISEASE…

• Persistent and Profound flaccidity of limbs,Areflexia,atonic bladder(mistaken for spinal shock or GBS)

• Saltatory progression• Necrosis of gray and white matter over variable

extent Infarctive/Demyelinative• CSF increased Protein and cells. No bands.• MRI initial edema,later atrophy over varying

extent• EMG• Steroids,Plasma exchange,Cyclophosphamide

PARANEOPLASTIC MYELOPATHY…• Acute necrotizing myelopathy• Subacute motor neuronopathy• MND• Stiff-man syndrome• Cerebellar degeneration

• Lesions are necrotic involving both grey and white matter

• SCLC,Ovarian ca,Lymphoma

• Anti YO,Anti Tr,Anti Hu antibodies,Anti GAD and anti Amphiphysian.

SUBACUTE SPINAL NEURONITIS..• Tonic rigidity• Myoclonus• Sensory evoked painful spasms• Progressive brainstem involvement• Preserved mentation• Loss of internuncial neurons with preserved

AHC• Seen following Spinal artery angiography• Anticonvulsants and antispastics,botulinum

toxin.

VASCULAR..• Spinal arteries not susceptible to atherosclerosis &

rarely emboli lodge.• Secondary to collateral circulation or aortic disease –

advanced atherosclerosis,dissecting aneurysm,occlusion of aorta thoracic aortic surgery.

• PAN,Systemic cholesterol embolisation,Hypotension & shock,Fibrocartilagenous embolism,dissection of extracranial vertebral artery.

• AV Malformation,Dural fistula• Midthoracic cordbetween D3 – D8 is most

vulnerable.

SPINAL INFARCTION(MYELOMALACIA)..• Usually Anterior spinal artery territory.• Anterior 2/3rd of the cord to a variable vertical extent.• Onset can be rapid or more commonly over few hrs.• Pain in the neck or back followed by varying degrees of

motor,sensory & sphinter involvement.• Radicular pain can occur..Usually bilateral,rarely complete.• Dissociated sensory loss except in high cord lesion.• Gradual improvement is seen.• Remember a VARIANT OF POSTINFECTIOUS MYELITIS.

• MRI may show edema and later myelomalacia..REMEMBER INITIAL FEW HRS TO DAYS MRI CAN BE NORMAL ALSO.

• Dissection of aorta:• Paralysis of sphinters & Both LL with sensory

loss below D6• Infarction confined to grey matter alone• Involvement of Common carotid artery leading

to Hemiplegia• Obstruction of Brachial artery

• Aortography of spinal artery can cause rigidity,myoclonus & spasm mentioned in subacute spinal neuronitis.

VASCULAR MALFORMATION…• Pure dural fistula• AVM predominantly intramedullary• AVM perimedullary and involving subpial cord

• Size of the communicating vessel and size and location of feeding artery and draining vessel

• Dural fistula:• Most common type• Region of low thoracic or conus• Gait imbalance,sensory symptomsweakness and bladder

involvement• Valsalva & exercise amplify symptoms• Rarely bleed.• Intramedullary AVM:• Men Past middle age• Dorsal surface of lower half of the cord• Dermatomal nevus• Acute cramp-like.lancinating pain along sciatic distribution• Disabled in 6 months,chairbound in 3 yrs,survival 6 yrs.• Rarely bleed.

• Perimedullary and subpial AVM:• Younger age,equal sex ratio• Lower thoracolumbar or anterior cervical cord• Gradual or acute presentation• Bleed into Cord or subarachnoid space

• Contrast CT/MRI• Selective angiography

• Occluding the feeding vessel of AVM by open surgical or endovascular techniques

• Dural fistulas endovascular techniques preferred

• Klippel-Trenaunay-Weber syndrome:• Cutaneous nevi,malformation in lower

cord,enlargement of fingers,hands,arm(haemangiectatic/neurofibromatous)

• Fibrocartilagenous embolism:• Follows trauma• Abrupt pain followed by transverse cord lesion• Rarely mimick ASA thrombosis• Thrombosis of numerous spinal arteries & veins due

to embolism of Nucleus pulposus

METABOLIC…

• B12 deficiency• Copper deficiency• Diabetes• Hyperthyroidism

SACD..• B 12 deficiency• Hypocupremic Myelopathy• Hereditary spastic paraplegia(HAM)• HIV associated• N2O inhalation• Cervical spondylosis• Familial spastic paraplegia• Lathyrism• Adhesive arachnoiditis• Following Chr Hepatitis

• Vit B12 Deficiency: Areflexia(peri neuropathy),Optic atrophy,Mentation changes.

• Macrocytosis,Low B12,Elevated homocystine and methyl melonic acid.

• Hypocupric:Normal B12, low Copper and Ceruloplasmin.

• HAM: Early bladder involvement,Increased DTR in UL,Preserved brainstem & mentation.

• HTLV 1 specific antibody..Slow progression..Symptomatic management

• HIV associated vacuolar myelopathy:Vacuolar degeneration of cord…ART no effect.

• 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.• Amotrophy,MR,Optic atrophy

• Survival is long because respiration is spared

• Only symptomatic therapy

CHRONIC ADHESIVE ARACHNOIDITIS..• 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• CT/MRI contrast showing total or partial loss of

spinal subarachnoid space(candle guttering)• Degeneration of peripheral fibres of posterior and

lateral column• Steroids,Decompressive surgery,Posterior

rhizotomy,microsurical dissection,Gabapentin,transcutaneous stimulator.

• LATHYRISM:• UL may show coarse tremors and involutary

movements.• BOAA(beta N oxalylaminoalanine)L.sathyvus or

grass pea.• Loss of myelinated fibres in postero lateral

column• Symptoms donot progress constantly, so lifespan

is not reduced.• KONZO:• African acute spastic paraplegia• Cassava – cyanide-like compounds

PHYSICAL AGENTS…• Electrical injuries:• Amount of current,ampherage,duration of contact,resistance

offered by the skin• Immediate or Delayed – few days to 6 weeks– Spinal atrophic

paralysis• More injury to grey matter.

• Heating of tissue,Vasocclusive changes,demylination,fracture.• Lightening injury:• Arborescent marks• Limbs may be pale and cold or cyanotic• Late presentation

• Caissons disease:• Upper thoracic cord• Little or no brain inv.• Posterior column > lateral column• Decompression in hyperbaric chamber,Symptomatic

treatment• Radiation myelopathy:• Early transient/Delayed progressive/Slowly evolving

amotrophy• PAIN IS ABSENT & LESION IS EXTENSIVE• Coagulative necrosis,vascular changes,secondary

degeneration• 6000 cGy over 30 – 70 days not exceeding 200cGy/day or

900cGy/week• Steroids,Hyperbaric oxygen and heparin split products