Non compressive myelopathy

6,688 views

Published on

management of non compressive myelopathy

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

Published in: Education, Health & Medicine
2 Comments
31 Likes
Statistics
Notes
No Downloads
Views
Total views
6,688
On SlideShare
0
From Embeds
0
Number of Embeds
8
Actions
Shares
0
Downloads
435
Comments
2
Likes
31
Embeds 0
No embeds

No notes for slide

Non compressive myelopathy

  1. 1. EVALUATION AND DIAGNOSIS OF NON COMPRESSIVE MYELOPATHY SANKALP MOHAN SENIOR RESIDENT NEUROLOGY
  2. 2. SPINAL CORD CROSS SECTION
  3. 3. MYELOPATHIES CLASSIFICATION
  4. 4. SPINAL CORD SYNDROMES • COMPLETE CORD SYNDROME • (TRAUMA ,TRANSVERSE MYELITIS) • BROWN SEQUARD SYNDROME (HEMICORD) • (TRAUMA COMPRESSION,MS) • ANTERIOR CORD SYNDROME • (SPINAL ARTERY INFARCTION)
  5. 5. • POSTERIOR CORD SYNDROME • (VIT B12 DEFICIENCY, COPPER DEFICIENCY) • CENTRAL CORD SYNDROME • (SYNRINGOMYELIA,INTRAMEDULLARY TUMORS) • CONUS AND CAUDA EQUINA
  6. 6. COMPRESSI VE EXTRAMEDULLARY INTRAMEDULLARY NON COMPRESSI VE INFLAMMATORY NON INFLAMMATORY
  7. 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. 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. 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. 10. ACUTE TRANSVERSE MYELITIS
  11. 11. EPIDEMIOLOGY Incidence - up to 3 per 100,000 patient years (0.003%)  no familial or ethnic predisposition  More common in females 
  12. 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. 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. 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. 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. 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. 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. 18. SUPPORTIVE MANAGEMENT - SPASTICITY  BLADDER DYSFUNCTION  GI DYSFUNCTION  COMPLICATIONS DUE TO IMMOBILITY – PRESSURE SORE,DVT MALNUTRTITION,RESPIRATORY THERAPY 
  19. 19. INFECTIOUS CAUSES
  20. 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. 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. 22. Pathologically –vacoular myelopathy ..spongy degeneration .,demyelination (axons relatively preserved )  ViT b12 deficiency may contribute  No effective treatment ..ART may reduce the incidence 
  23. 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. 24. OTHER CSF STUDIES
  25. 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. 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. 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. 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 
  29. 29. MULTIPLE SCLEROSIS NEUROMYELITIS OPTICA
  30. 30. 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 
  31. 31. 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. 
  32. 32. DIAGNOSTIC CRITERIA
  33. 33. 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%. 
  34. 34. 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 
  35. 35. 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
  36. 36. VASCULAR  BLOOD SUPPLY OF SPINAL CORD
  37. 37. 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) 
  38. 38. 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% 
  39. 39. 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) 
  40. 40. METABOLIC MYELOPATHIES
  41. 41. 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
  42. 42. 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 
  43. 43. 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 
  44. 44. 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 
  45. 45. AXIAL T2 WEIGHTED SAGGITAL T2 WEIGHTED
  46. 46. TREATMENT Oral or parenteral vitb12  NEUROLOGIC symptoms- high dose im  1000ug im for 2 weeks daily f/b 1000ug monthly for 6 months 
  47. 47. 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
  48. 48. 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 
  49. 49. 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 
  50. 50. 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
  51. 51. - - 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
  52. 52. OTHER TOXIC MYELOPATHIES Chemotherapy induced  Hepatic Myelopathy  Heroin myelopathy  Fluorosis 
  53. 53. 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 
  54. 54. 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 

×