Non-Compressive Myelopathy

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Non-Compressive Myelopathy

  1. 1. NON-COMPRESSIVE MYELOPATHY G.Madhu Shankar Prof.S.TITO’S UNIT
  2. 2. <ul><li>An estimate states that there are 30 common diseases of Spinal cord. </li></ul><ul><li>15 are common in clinics. </li></ul><ul><li>Most of them follow syndromic pattern. </li></ul>
  3. 3. <ul><li>Causes </li></ul><ul><li>Presentation </li></ul><ul><li>Prognosis </li></ul><ul><li>Management </li></ul><ul><li>30 slides </li></ul>
  4. 4. CLASSIFICATION… <ul><li>Myelitis(Inflammatory disease) </li></ul><ul><li>Infective </li></ul><ul><li>Non-infective </li></ul><ul><li>Vascular </li></ul><ul><li>Metabolic </li></ul><ul><li>Drugs and toxins </li></ul><ul><li>Physical agents </li></ul><ul><li>Degenerative </li></ul>
  5. 5. MYELITIS… <ul><li>Poliomyelitis </li></ul><ul><li>Leukomyelitis </li></ul><ul><li>Transverse Myelitis </li></ul><ul><li>Disseminated </li></ul><ul><li>Meningomyelitis </li></ul><ul><ul><ul><li>Pachy </li></ul></ul></ul><ul><ul><ul><li>Lepto </li></ul></ul></ul><ul><li>Encephalomyelitis </li></ul><ul><li>Meningaradiculitis </li></ul><ul><li>Abscess/Granuloma </li></ul>
  6. 6. <ul><li>Acute within days </li></ul><ul><li>Subacute 2 – 6 weeks </li></ul><ul><li>Chronic >6 weeks </li></ul>
  7. 7. Infective myelitis.. <ul><li>Viral </li></ul><ul><ul><ul><li>Herpes group viruses(HSV,EBV,CMV,HZV) </li></ul></ul></ul><ul><ul><ul><li>Enterovirus(Cox,Polio) </li></ul></ul></ul><ul><ul><ul><li>HIV/HTLV 1 asso myelopathy(HAM) </li></ul></ul></ul><ul><ul><ul><li>Rabies,Arbovirus </li></ul></ul></ul><ul><li>Non-viral(Bacterial,Fungal,Parasitc,Granulomatous) </li></ul><ul><ul><ul><li>Mycoplasma </li></ul></ul></ul><ul><ul><ul><li>Pyogenic </li></ul></ul></ul><ul><ul><ul><li>Tuberculous </li></ul></ul></ul><ul><ul><ul><li>Syphilitic </li></ul></ul></ul>
  8. 8. <ul><li>VIRAL MYELITIS: </li></ul><ul><li>Acute onset </li></ul><ul><li>Febrile meningomyelitis </li></ul><ul><li>Systemic manifestations </li></ul><ul><li>Cutaneous manifestations </li></ul><ul><li>Enterovirus  Anterior horn cells </li></ul><ul><li>Herpetic  Dorsal root ganglion </li></ul><ul><li>HSV 2  lumbosacral radiculitis </li></ul><ul><li>CSF </li></ul><ul><li>Isolation of Viral nucleic acid by PCR </li></ul>
  9. 9. <ul><li>Myelitis that presents as dysfunction of motor and sensory tracts is rarely viral but rather due to Non infectious inflammatory pathology BUT EXCEPTION BEING </li></ul><ul><li>Zoster myelitis </li></ul><ul><li>HIV associated Vacuolar myelopathy </li></ul><ul><li>HAM(Tropical spastic paraparesis) </li></ul><ul><li>Dumb rabies </li></ul>
  10. 10. NON INFECTIOUS INFLAMMATORY... <ul><li>Postinfecious and postvaccinal </li></ul><ul><li>MS </li></ul><ul><li>Acute and subacute necrotizing myelitis and Devic’s disease </li></ul><ul><li>Asso with connective tissue disorders </li></ul><ul><li>Paraneoplastic </li></ul><ul><li>Leukomyelitis </li></ul><ul><li>Disordered immune response </li></ul><ul><li>Affect CNS,optic nerves </li></ul>
  11. 11. POSTINFECIOUS AND POSTVACCINAL.. <ul><li>Most common cause of ATM </li></ul><ul><li>Temporal relationship to infection or vaccination </li></ul><ul><li>Development in days </li></ul><ul><li>Monophasic temporal course </li></ul><ul><li>Varying degrees of weakness,sensory symptoms,sphinter disturbances </li></ul><ul><li>Back pain </li></ul><ul><li>40% give a positive H/O </li></ul><ul><li>Mean age 3 – 4 th decade </li></ul><ul><li>CSF cells 10 – 100/mm3,Normal glucose,Raised protein </li></ul><ul><li>Pauci inflammatory also. Absent oligoclonal bands </li></ul><ul><li>MRI enhancement </li></ul>
  12. 12. Variants… <ul><li>Pure conus syndrome </li></ul><ul><li>Partial Brown sequard syndrome </li></ul><ul><li>Variable sensory loss over LL </li></ul><ul><li>Pure Posterior column dysfunction </li></ul><ul><li>Mimicking ASA thrombosis(Motor & spinothalamic inv alone wit preserved deep sensation) </li></ul><ul><li>Pathologically Demyelination with inflammatory infiltrates </li></ul><ul><li>Treatment: </li></ul><ul><ul><ul><li>Steroids in high doses </li></ul></ul></ul><ul><ul><ul><li>IV Ig or plasma exchange </li></ul></ul></ul><ul><li>Prognosis: </li></ul><ul><ul><ul><li>Better..Improvement occurs. </li></ul></ul></ul><ul><ul><ul><li>Acute onset/Mid thoracic Pain portend poor prognosis </li></ul></ul></ul>
  13. 13. MS(DEMYELINATIVE) <ul><li>No temporal relation </li></ul><ul><li>Slow evolution </li></ul><ul><li>Remitting and relapsing course </li></ul><ul><li>Disseminated in Time and Space </li></ul><ul><li>Optic neuritis </li></ul><ul><li>Age 20 – 40 yrs </li></ul><ul><li>MRI(MTR/MRSI) </li></ul><ul><li>CSF for Oligoclonal bands and CSF IgG index(Intrathecal IgG) </li></ul><ul><li>CSF Cells >75/uL,PMN,Protein >100 mg/dL cast a doubt. </li></ul>
  14. 14. <ul><li>MANAGEMENT </li></ul><ul><li>Acute attack </li></ul><ul><ul><ul><li>IV Methyl Prednisolone </li></ul></ul></ul><ul><ul><ul><li>Plasma Exchange </li></ul></ul></ul><ul><li>Disease modifying drugs </li></ul><ul><ul><ul><li>IFN-B-1a,1b </li></ul></ul></ul><ul><ul><ul><li>Glatiramer acetate </li></ul></ul></ul><ul><ul><ul><li>Natalizumab </li></ul></ul></ul><ul><ul><ul><li>Immunosuppresants </li></ul></ul></ul><ul><ul><ul><li>Prognosis </li></ul></ul></ul><ul><ul><ul><ul><ul><li>Individualised </li></ul></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>Grave disability </li></ul></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>Direct mortality rare. </li></ul></ul></ul></ul></ul>
  15. 15. ACUTE AND SUBACUTE NECROTIZING MYELITIS AND DEVIC’S DISEASE… <ul><li>Persistent and Profound flaccidity of limbs,Areflexia,atonic bladder(mistaken for spinal shock or GBS) </li></ul><ul><li>Saltatory progression </li></ul><ul><li>Necrosis of gray and white matter over variable extent  Infarctive/Demyelinative </li></ul><ul><li>CSF  increased Protein and cells. No bands. </li></ul><ul><li>MRI  initial edema,later atrophy over varying extent </li></ul><ul><li>EMG </li></ul><ul><li>Steroids,Plasma exchange,Cyclophosphamide </li></ul>
  16. 16. PARANEOPLASTIC MYELOPATHY… <ul><li>Acute necrotizing myelopathy </li></ul><ul><li>Subacute motor neuronopathy </li></ul><ul><li>MND </li></ul><ul><li>Stiff-man syndrome </li></ul><ul><li>Cerebellar degeneration </li></ul><ul><li>Lesions are necrotic involving both grey and white matter </li></ul><ul><li>SCLC,Ovarian ca,Lymphoma </li></ul><ul><li>Anti YO,Anti Tr,Anti Hu antibodies,Anti GAD and anti Amphiphysian. </li></ul>
  17. 17. SUBACUTE SPINAL NEURONITIS.. <ul><li>Tonic rigidity </li></ul><ul><li>Myoclonus </li></ul><ul><li>Sensory evoked painful spasms </li></ul><ul><li>Progressive brainstem involvement </li></ul><ul><li>Preserved mentation </li></ul><ul><li>Loss of internuncial neurons with preserved AHC </li></ul><ul><li>Seen following Spinal artery angiography </li></ul><ul><li>Anticonvulsants and antispastics,botulinum toxin. </li></ul>
  18. 18. VASCULAR.. <ul><li>Spinal arteries not susceptible to atherosclerosis & rarely emboli lodge. </li></ul><ul><li>Secondary to collateral circulation or aortic disease – advanced atherosclerosis,dissecting aneurysm,occlusion of aorta thoracic aortic surgery. </li></ul><ul><li>PAN,Systemic cholesterol embolisation,Hypotension & shock,Fibrocartilagenous embolism,dissection of extracranial vertebral artery. </li></ul><ul><li>AV Malformation,Dural fistula </li></ul><ul><li>Midthoracic cordbetween D3 – D8 is most vulnerable. </li></ul>
  19. 20. SPINAL INFARCTION(MYELOMALACIA).. <ul><li>Usually Anterior spinal artery territory. </li></ul><ul><li>Anterior 2/3 rd of the cord to a variable vertical extent. </li></ul><ul><li>Onset can be rapid or more commonly over few hrs. </li></ul><ul><li>Pain in the neck or back followed by varying degrees of motor,sensory & sphinter involvement. </li></ul><ul><li>Radicular pain can occur..Usually bilateral,rarely complete. </li></ul><ul><li>Dissociated sensory loss except in high cord lesion. </li></ul><ul><li>Gradual improvement is seen. </li></ul><ul><li>Remember a VARIANT OF POSTINFECTIOUS MYELITIS. </li></ul><ul><li>MRI may show edema and later myelomalacia..REMEMBER INITIAL FEW HRS TO DAYS MRI CAN BE NORMAL ALSO. </li></ul>
  20. 21. <ul><li>Dissection of aorta: </li></ul><ul><li>Paralysis of sphinters & Both LL with sensory loss below D6 </li></ul><ul><li>Infarction confined to grey matter alone </li></ul><ul><li>Involvement of Common carotid artery leading to Hemiplegia </li></ul><ul><li>Obstruction of Brachial artery </li></ul><ul><li>Aortography of spinal artery can cause rigidity,myoclonus & spasm mentioned in subacute spinal neuronitis. </li></ul>
  21. 22. VASCULAR MALFORMATION… <ul><li>Pure dural fistula </li></ul><ul><li>AVM predominantly intramedullary </li></ul><ul><li>AVM perimedullary and involving subpial cord </li></ul><ul><li>Size of the communicating vessel and size and location of feeding artery and draining vessel </li></ul>
  22. 23. <ul><li>Dural fistula: </li></ul><ul><li>Most common type </li></ul><ul><li>Region of low thoracic or conus </li></ul><ul><li>Gait imbalance,sensory symptoms  weakness and bladder involvement </li></ul><ul><li>Valsalva & exercise amplify symptoms </li></ul><ul><li>Rarely bleed. </li></ul><ul><li>Intramedullary AVM: </li></ul><ul><li>Men Past middle age </li></ul><ul><li>Dorsal surface of lower half of the cord </li></ul><ul><li>Dermatomal nevus </li></ul><ul><li>Acute cramp-like.lancinating pain along sciatic distribution </li></ul><ul><li>Disabled in 6 months,chairbound in 3 yrs,survival 6 yrs. </li></ul><ul><li>Rarely bleed. </li></ul>
  23. 24. <ul><li>Perimedullary and subpial AVM: </li></ul><ul><li>Younger age,equal sex ratio </li></ul><ul><li>Lower thoracolumbar or anterior cervical cord </li></ul><ul><li>Gradual or acute presentation </li></ul><ul><li>Bleed into Cord or subarachnoid space </li></ul><ul><li>Contrast CT/MRI </li></ul><ul><li>Selective angiography </li></ul><ul><li>Occluding the feeding vessel of AVM by open surgical or endovascular techniques </li></ul><ul><li>Dural fistulas endovascular techniques preferred </li></ul>
  24. 25. <ul><li>Klippel-Trenaunay-Weber syndrome : </li></ul><ul><li>Cutaneous nevi,malformation in lower cord,enlargement of fingers,hands,arm(haemangiectatic/neurofibromatous) </li></ul><ul><li>Fibrocartilagenous embolism: </li></ul><ul><li>Follows trauma </li></ul><ul><li>Abrupt pain followed by transverse cord lesion </li></ul><ul><li>Rarely mimick ASA thrombosis </li></ul><ul><li>Thrombosis of numerous spinal arteries & veins due to embolism of Nucleus pulposus </li></ul>
  25. 26. METABOLIC… <ul><li>B12 deficiency </li></ul><ul><li>Copper deficiency </li></ul><ul><li>Diabetes </li></ul><ul><li>Hyperthyroidism </li></ul>
  26. 27. SACD.. <ul><li>B 12 deficiency </li></ul><ul><li>Hypocupremic Myelopathy </li></ul><ul><li>Hereditary spastic paraplegia(HAM) </li></ul><ul><li>HIV associated </li></ul><ul><li>N2O inhalation </li></ul><ul><li>Cervical spondylosis </li></ul><ul><li>Familial spastic paraplegia </li></ul><ul><li>Lathyrism </li></ul><ul><li>Adhesive arachnoiditis </li></ul><ul><li>Following Chr Hepatitis </li></ul>
  27. 28. <ul><li>Vit B12 Deficiency : Areflexia(peri neuropathy),Optic atrophy,Mentation changes. </li></ul><ul><li>Macrocytosis,Low B12,Elevated homocystine and methyl melonic acid. </li></ul><ul><li>Hypocupric: Normal B12, low Copper and Ceruloplasmin. </li></ul><ul><li>HAM: Early bladder involvement,Increased DTR in UL,Preserved brainstem & mentation. </li></ul><ul><li>HTLV 1 specific antibody..Slow progression..Symptomatic management </li></ul><ul><li>HIV associated vacuolar myelopathy :Vacuolar degeneration of cord…ART no effect. </li></ul>
  28. 29. <ul><li>FAMILIAL SPASTIC PARAPLEGIA: </li></ul><ul><li>3 rd – 4 th decade..can occur in 1 st decade too </li></ul><ul><li>AD/AR/X-linked </li></ul><ul><li>Sensory involvement is minimal,Bladder is involved late in the illness. </li></ul><ul><li>Amotrophy,MR,Optic atrophy </li></ul><ul><li>Survival is long because respiration is spared </li></ul><ul><li>Only symptomatic therapy </li></ul>
  29. 30. CHRONIC ADHESIVE ARACHNOIDITIS.. <ul><li>Painful root and cord symptoms </li></ul><ul><li>Syphilis,Resistant meningitis,TB,Penicillin,Contrast,steroids </li></ul><ul><li>Thickening of Arachnoid,proliferation of connective tissue and adhesion between arachnoid & dura. </li></ul><ul><li>PERSISTANT PAIN </li></ul><ul><li>CT/MRI contrast showing total or partial loss of spinal subarachnoid space(candle guttering) </li></ul><ul><li>Degeneration of peripheral fibres of posterior and lateral column </li></ul><ul><li>Steroids,Decompressive surgery,Posterior rhizotomy,microsurical dissection,Gabapentin,transcutaneous stimulator. </li></ul>
  30. 31. <ul><li>LATHYRISM: </li></ul><ul><li>UL may show coarse tremors and involutary movements. </li></ul><ul><li>BOAA(beta N oxalylaminoalanine)L.sathyvus or grass pea. </li></ul><ul><li>Loss of myelinated fibres in postero lateral column </li></ul><ul><li>Symptoms donot progress constantly, so lifespan is not reduced. </li></ul><ul><li>KONZO: </li></ul><ul><li>African acute spastic paraplegia </li></ul><ul><li>Cassava – cyanide-like compounds </li></ul>
  31. 32. PHYSICAL AGENTS… <ul><li>Electrical injuries: </li></ul><ul><li>Amount of current,ampherage,duration of contact,resistance offered by the skin </li></ul><ul><li>Immediate or Delayed – few days to 6 weeks– Spinal atrophic paralysis </li></ul><ul><li>More injury to grey matter. </li></ul><ul><li>Heating of tissue,Vasocclusive changes,demylination,fracture . </li></ul><ul><li>Lightening injury: </li></ul><ul><li>Arborescent marks </li></ul><ul><li>Limbs may be pale and cold or cyanotic </li></ul><ul><li>Late presentation </li></ul>
  32. 33. <ul><li>Caissons disease: </li></ul><ul><li>Upper thoracic cord </li></ul><ul><li>Little or no brain inv. </li></ul><ul><li>Posterior column > lateral column </li></ul><ul><li>Decompression in hyperbaric chamber,Symptomatic treatment </li></ul><ul><li>Radiation myelopathy : </li></ul><ul><li>Early transient/Delayed progressive/Slowly evolving amotrophy </li></ul><ul><li>PAIN IS ABSENT & LESION IS EXTENSIVE </li></ul><ul><li>Coagulative necrosis ,vascular changes,secondary degeneration </li></ul><ul><li>6000 cGy over 30 – 70 days not exceeding 200cGy/day or 900cGy/week </li></ul><ul><li>Steroids,Hyperbaric oxygen and heparin split products </li></ul>

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