Nontraumatic Spinal Cord Injury (NT/SCI)

1. Nontraumatic Spinal Cord Injury (NT/SCI) William McKinley MD Dept PM&R Medical College of Virginia / Virginia Commonwealth University

2. Objectives: NT/SCI • Importance / Incidence • Literature / Prior Studies • Etiologies / Clinical presentation • Prognosis / Rehabilitation Outcome

3. Importance of NT/SCI • Morbidity • weakness, neurogenic bladder / bowel, spasticity, infections, venous thrombosis, depression • Loss of Function • mobility, self-care, vocational, social • Cost of care • hospitalization, home, lost wages

4. Importance: NT/SCI (cont.) • NT/SCI represents a significant % of SCI and of those undergoing SCI rehab • Common causes: Spinal Stenosis (SS), tumor, ischemia • Prior Literature: lacking for NT/SCI (demographics, clinical, outcomes) as opposed to Traumatic SCI (MVA, violence, falls)

5. Incidence: NT/SCI • Kurtzke (1975): Incidence = 8/100,000 • Gibson (1991): Stenosis 16%, Cancer 14% (SCI admissions) • Murray (1994): NT/SCI 31% (less than 40)(87% greater than age 40) • McKinley (1999): NT/SCI 39%, Stenosis 26%, Tumor 10%

6. SCI Etiology: MCV/VCU

7. Nontraumatic vs Traumatic SCI • McKinley W, Seel R, Hardman J. Arch PM&R 80, 619-23, 1999 • (Five year perspective study (N=220)) • 39% NT/SCI (26% SS, 10% tumor) • (Comparison NT/SCI vs T/SCI) • Demographics: • Significantly (P < .01) older (61 yr vs 39yr), married (57% vs 38%), female (50% vs 16%), retired (76% vs 33%)

8. NT/SCI vs T/SCI (cont..) • Injury characteristics: • Significantly (P < .01) paraplegia (73% vs 55%)& incomplete SCI (91& vs 58%) • Outcomes: • Significant FIM gains (admit-disch.) • Similar FIM efficiency • Similar discharge to home rates • “Matching study”: shorter rehab LOS (rehab charges), similar FIM efficiency,

9. Conclusions: NT/SCI vs T/SCI • Significant % of SCI • Represent different demographic make-up (older, married, not working) • Less severe neurological presentation (paraplegia. Incomplete) • Similar outcomes vs traumatic SCI • Further Studies: morbidity, long-term functional outcomes, cost, RTW, community reintegration

10. NT/SCI: Medical Complications • NT/SCI (37) vs T/SCI (77) • Spasticity* 22% 42% • Orthostasis* 8% 38% • DVT* 8% 22% • Pneumonia* 5% 27% • Aut. Dysreflexia* 0% 24% • Wound infection 16% 3% • Similar incidences: Pain, UTI, depression, pressure ulcers, GI bleeds, Het. Oss.

11. Etiologies: Nontraumatic SCI • Spinal Stenosis, Tumor, Vascular • Inflammatory • infectious (viral, bacterial, fungal, parasitic) • Non-infectious (transverse myelitis, MS, polio) • “Other” • RA, Radiation, Syringomyelia, MND, vitamin B-12, Friedreich ataxia,

12. Spinal Stenosis • Spondylosis w/o neurological compression reported in 55% (>50 yr) and 75% (> 65yr) • Anterior compression - vert. body osteophytes • Posterior comp. - Lig. Flavum (extension) • Lateral compression - intervertebral. Foramen • Commonly lower cervical (C4-7), lumbar (L2-4)-cord is mobile & widest • A-P canal (nl)17-18mm (Sx: < 12 mm)

13. Spondylosis (cont.) • Clinical: neck/back/radicular pain, asymmetric weakness, hyperreflexia, aggravated by standing/walking (extension) • Onset: gradual (months-years), stepwise deterioration with periods of stability • Dx: MRI*, X-ray (narrow disc space/sp. canal, foramina) (poor correlation with clinical finding), CT, myelogram, EMG

14. Surgery Severe/prog. neuro, failed conservative Decompression CCS/good outcome consider complications (mortality 2%, worsening 2%, cost) Epstein (N=114) 36% improved, 38% stable, 26% worse Rx: Nonsurgical vs Surgical (no prospective controlled clinical trials) • Nonsurgical: • Patients without major neurological deficits (rest, traction, collar, PT, pain rx, bl/bwl)

15. Rehab Outcome of Myelopathy from Spinal Stenosis • (McKinley, Tellis, Cifu. J. Spinal Cord Med. 21(2) 131-6, 1998) • N=46, 59% lumbar & 41% cervical, 100% incomplete SCI • Results: older (68 vs 39 yr), female (46% vs 18%) married (57% vs 36%), unempl/retired (89% vs 43%) • Significant FIM gains • Similar outcomes vs T/SCI: FIM, LOS, D/C

16. Neoplastic Spinal Cord Compression (SCC) • Classification: • Extramedullary (95%) • Epidural – (55%)…*metastatic • (breast, lung, prostate, lymphoma) • Intradural (40%) • (neurofibromas, meningioma) • Intramedullary (5%) • (gliomas) • Spinal cord : brain tumor (1:4)

17. EpiduralExtramedullary

18. Metastatic Epidural SCC (MESCC) • Vertebral mets - seen 15-40% (100,000 yr) • Hemodynamic mets – bone marrow • Batsons epidural venous plexus – pelvic, abdominal and thoracic (valsalva, coughing) • Location: 85% vertebra, 10% paravert, 5% epidural • Primary site: breast, lung, prostate 50% (others: lymphoma, renal, MM) • 9% of cases have unknown primary tumor • Children: neuroblastoma, sarcoma, lymphoma

19. MESCC (cont.) • Epidural SCC occurs in 5% • Path: cord compression/ischemia • edema, demyelination, hemorrhage, cystic necrosis • Clinical: Onset: days to weeks, localized pain (95%), worse w/ supine, paresis is rare initial clinical finding but 75% have at dx (50% unable to ambulate, 15% paraplegic) • Thoracic 70%, Cervical 10%, Lumbar 20%

20. MESCC (cont.) • Dx: MRI (contrast), CT, myelo, bone scan • Rx: CSF chemo, steroids, XRT • radiosensitive: breast, prostate, lymphoma • Surgical considerations: tumor removal, diagnosis, deterioration after XRT, spinal instability or bony compression • Prognosis: neurological function at XRT • Ambulation: 80% if ambulating at initiation, 50% if weak, <10% if paraplegic • 30-50% improvement in radiosensitive tumors

21. Rehabilitation of Neoplastic SCC • Important considerations: • Pain Rx, Psychological Rx • Outcome comparison w/ Traumatic SCI • (McKinley, Wyneken, CIfu: Archive PM&R 1996 • (McKinley, Huang, Brunsvold. Archives PM&R 1999) • older age (58yr vs 36yr), female (58% vs 18%), paraplegia (88% vs 52%), incomplete (88% vs 57%) • Functional improvements (FIM) at DC & 3 mo f/u • Similar FIM Efficiency as Traumatic SCI • LOS shorter (25 vs 57 days)

22. Intradural/Extramedullary SCI Tumors • Path: compression & ischemia • Meningioma • Neurofibromatosis- (cutaneous lesions, diagnosed by biopsy, early adulthood) • Clinical : similar to MESCC • often benign

23. Intramedullary SCI Tumors • Clinical: Males 56%, thoracic 50%, • Path: Gliomas most common (ependymomas 60%, astrocytomas 25% (most common in children)) • Ependymomas – benign (ependymal cells line CNS) • Astrocytomas – graded 1-4 (1 and 2 = 76%) • Prognosis: 5 year survival 80% with grades 1 and 2, < one year survival with grades 3 and 4 • Rx: biopsy, XRT, surgical removal

24. Cancer-Related SCI • Paraneoplastic Syndrome • Radiation Myelitis

25. Paraneoplastic myelitis • Definition: “remote effects of tumor, not due to direct invasion/compression • Path: Subacute necrosis of gray/white matter, no evidence of infection , inflammation or ischemia • Associated with lung cancer, Lymphoma • Clinical: progressive weakness

26. Radiation Myelopathy • Incidence 2-3% • Path: delayed vascular necrosis of white/gray matter • Onset occurs 6-48 months (usually 12-15) after XRT • seen w/ 2500-6500 total rads • importance: spacing & size of radiation field • Rec: total dose < 6000 rads (given over 30-60 days, daily fraction < 200, weekly < 900 rads) • Clinical: sensory changes, paresthesias, weakness with decreased reflexes • Rx: trial of steroids

27. Vascular “Ischemic” Myelopathy • Incidence: 1% of all strokes • Etiology: aortic dissection, embolism, vascular surgery, AVM, hypotension, hemorrhage • Path: tissue vulnerability (inadequate anastamosis) • No permanent damage < 30 minutes of interrupted blood supply (aortic clamping). gray matter more vulnerable (metabolic rate 3.5X white) • Brain more vulnerable than Sp cord (brain TBF = 50ml/min, sp cord = 20 ml/min)

28. Vasculature of the Spinal Cord • segmental Radicular arteries • aorta, subclavian (vertebral) • Spinal arteries • (1) anterior & (2) posterior • “Watershed” areas: thoracic region • GSA (Adamkiewicz) T9-12 • Venous drainage – Batson’s posterior spinal plexus

29. Spinal Cord Vasculature

30. Aortic Aneurysm-related SCI • Most common cause of vascular cord injury • Path: ischemia during surgery (1-2%) or dissection (2%)-occludes sp art • Clinical: anterior spinal artery (ASA) syndrome, sudden onset pain & paralysis, thoracic region primarily (lack of collateral circulation)

31. Embolic Ischemia • Rare • Path: atheromatous emboli • “Caisson disease” (decompression sickness) • Emobilization of anterior spinal artery • Path: embolic occlusion. of venous plexus by nitrogen bubbles during decompression.

32. Etiology: anti-coagulation (25-35%), AVM (SAH), coagulopathy Clinical: sudden pain & neuro symptoms Site: Intramed., subarach, subdural, epidural >50% cervical, males 2:1, mean age 55 Rx: prompt surgical evacuation of clot Prognosis: Decompression < 24 hrs yields full recovery in 50% Spinal Hemorrhage

33. Rheumatoid - Arthritis & SCI • Rare • Atlantoaxial subluxation in pts w/ RA (5-10%) • Path: Loosening of transverse ligament & destruction of odontoid leads to displacement of atlas and SCC or ischemia • Clinical: N/V, vertigo, neck pain, spasms, weakness • Rx: surgical stabilization (40% improved post surgery)

34. Motor system disorders • Amyotropic Lat. Sclerosis (ALS) • Pseudobulbar Palsy - (medulla) • Spinal Muscular Atrophy (SMA) • Progressive Lateral Sclerosis (PLS) - (corticospinal pathways)

35. Amyotropic Lateral Sclerosis (ALS) • Most frequent motor neuron disease • Path: Progressive degeneration of motor neurons in spinal cord, brain stem • Clinical: paralysis w/o sensory loss, spasticity • bulbar muscles (pharynx, tongue), impaired speech, swallowing, respiration • spares bladder/bowel • Dx: EMG/widespread denervation, biopsy- group atrophy, slightly increased CPK • Rx: maintenance, ? feeding tube, ventilator Rx • Prognosis: Life expectancy 4-7 years

36. Syringomyelia • Etiology: post-traumatic, idiopathic • path: Longitudinal canal cavitation (Greek: syrinx = “tube”) • Clinical: pain, impaired pain/temp, weakness, associated w/scoliosis • anterior commissure involvement • cervical cord, brain stem (syringobulbia) • Dx: MRI • Rx: surgical decompression, shunting (one-way valves emptying into subarachnoid space or peritoneal cavity)

37. Syringomyelia (“Syrinx”)

38. Inflammatory Myelitis • Infectious: • Viral (HIV, HSV, Polio) • Bacterial • Fungal (TB) • Parasitic (Syphilis) • Non-infectious • Transverse Myelitis • MS

39. HIV (AIDS) – related myelopathy • Acquired Immunodeficiency Syndrome (AIDS) • Human Immunodeficiency Virus (HIV) • Spinal Cord Involvement: • Compression by asso neoplasms (Karposi or lymphoma) • Direct infection by opportunistic organisms (HSV, CMV) • vacuolar degeneration - white matter, 10-30% (autopsy) • Clinical: slowly progressive spastic weakness, sensory loss, ataxia, incontinence, burning pain • Lateral and post-column involvement, thoracic • Dx: CSF with increased mononuclear cells, increased protein gamma globulin • Rx: trial of steroids

40. Herpes-related SCI • Rare • Path: Acute viral infection involving dorsal root ganglion (DRG) , +/- posterior cord • simplex (HSV), varicella-zoster (VSV) • Clinical: May follow cutaneous vesicular eruption (shingles) immediately or w/i weeks, asso with pain • Dx: CSF pleocytosis, protein • Rx: VZ immune globulin, acyclovir, capsacin, elavil

41. Poliomyelitis • Rare, anterior horn cell involvement • Path: Poliovirus (enterovirus) is usually non-paralytic. • Clinical: Paralytic illness develops early, mortality 5-25%, peaks in days • Fever, HA, neck & back pain, asymmetric weakness w/sensory sparing, areflexia, may involve bulbar m’s (respiratory impairment) • Dx: clinical, CSF w/inc cells, protein • Rx: maintenance, salk vaccine prevention

42. Postpolio Syndrome (PPS) • Def: clinical sx, 30+ yrs post-polio • Clinical: fatigue, weakness, pain • cold intol., sleep dist., swall. dysf. • Path: unknown, ? late muscle denervation • ? Late functional loss in “Nonparalytic Polio” • can see normal motor strength in 50% loss of anterior horn cells

43. Spinal (Epidural) Abscess • rare (increasing with immunosuppressed patients) • Path: extension of adjacent vertebral osteomyelitis (most common 25-50%) or hematogenous spread from distant infection (perinephric, pharyngeal, paraspinal) via arterial supply and Batsons Plexus • Clinical: onset-days-weeks, fever, pain, percussive tenderness, radicular pain, weakness • Staph Aureus (75% of acute), strep, e-coli • Location: posterior epidural space, thoracic...

44. Spinal Abscess (cont.) • Dx: early Dx essential, fluid cx, CSF (increased protein and cells-contra level of infection), MRI (T1-hypointense), blood cx, ESR, X-ray (osteo or paravert mass), myelogram • Rx: surgical drainage and IV antibiotics (6-8 weeks). Antibiotics alone controversial (19% worsening) • Prognosis: depends on neurological involvement. • Good prognosis if treatment begun before weakness or < 36 hours • Paraplegia >48 hours = poor prognosis for recovery

45. Chronic Abscess • *Micobacterium Tuberculosis (TB) • Other (breucellosis, actinomycosis) • Path: hematogenous seeding, paravertebral epidural abscess w/vert. Body destruction with anterior SCC (mechanical vs ischemic) • “Pott’s disease” (5-20% w/ neurological compromise) • Clinical: fever, back pain, paresis • Dx: xray, myelogram • Rx: isoniazid/rifampin, ethambutol

46. Spinal TB (Potts Disease)

47. Syphilis & SCI • rare, < 2% of those w/primary infection • Path: SC invasion by treponema parasite (tertiary stage), chronic inflammatory process • syphiliticmeningitis, Sx: BS or ACS • tabes dorsalis – DRG (“Shingles”) + post columns, males, 10-20 years post primary inf • Clinical: pain, ataxia, incontinence • Dx: VDRL in serum, FTA-ABS, CSF serology • Rx: Penicillin

48. Acute Transverse Myelitis • Path: Acute inflammatory lesions (auto immune response), not due to viral invasion of CNS • perivascular demyelination and cord necrosis, edema • Clinical: ascending flaccid paralysis, days to weeks • viral sx (malaise-N/V-fever), LBP, urine retention, T8-12 • Dx: clinical presentation and exclusion of other dx • ddx: vascular, infection, MS • post-viral/vaccinal (33%) • CSF nl, MRI (T-2 images hyperintense- inflammation) • Rx: trial of steroids • Prognosis: poor with MRI changes, “severe” weakness and EMG denervation

49. Spinal Multiple Sclerosis (MS) • Path: multifocal SC lesions (demyelination), +/_ asso w/ cerebral, optic or cerebellar MS • Clinical: asym spastic paresis, incont. “Lhermitte” • Dx: MRI, evoked pots., CSF (oligoclonal IgG) • Rx: steroids, immuno (cytoxan), ACT 4

50. Vitamin B-12 deficiency • Subacute Combined Degeneration (SCD) of sp cord • Path: inability to transfer B-12 across intestinal mucosa. posterior and lateral column changes • Vitamin B-12 (cobalamin) deficiency can effect spinal cord, brain, peripheral nerves • Clinical: weakness, ataxia, spasticity, dec. position sense, mental status changes (irritability, somnolence, confusion) • Dx: serum cobalamin, shilling test, asso w/ macrocytic anemia • Rx: B-12 injections