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

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Presentation on theme: "Nontraumatic Spinal Cord Injury (NT/SCI) William McKinley MD Dept PM&R Medical College of Virginia / Virginia Commonwealth University."— Presentation transcript:

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

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

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

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

5 Incidence: NT/SCI w Kurtzke (1975): Incidence = 8/100,000 w Gibson (1991): Stenosis 16%, Cancer 14% (SCI admissions) w Murray (1994): NT/SCI 31% (less than 40)(87% greater than age 40) w 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, , 1999 (Five year perspective study (N=220)) w 39% NT/SCI (26% SS, 10% tumor) (Comparison NT/SCI vs T/SCI) w 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..) w Injury characteristics: Significantly (P <.01) paraplegia (73% vs 55%)& incomplete SCI (91& vs 58%) w Outcomes: Significant FIM gains (admit-disch.) Similar FIM efficiency Similar discharge to home rates w “Matching study”: shorter rehab LOS (rehab charges), similar FIM efficiency,

9 Conclusions: NT/SCI vs T/SCI w Significant % of SCI w Represent different demographic make-up (older, married, not working) w Less severe neurological presentation (paraplegia. Incomplete) w Similar outcomes vs traumatic SCI w 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 w Spinal Stenosis, Tumor, Vascular w Inflammatory infectious (viral, bacterial, fungal, parasitic) Non-infectious (transverse myelitis, MS, polio) w “Other” RA, Radiation, Syringomyelia, MND, vitamin B-12, Friedreich ataxia,

12 Spinal Stenosis w 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 w Commonly lower cervical (C4-7), lumbar (L2-4)-cord is mobile & widest w A-P canal (nl)17-18mm (Sx: < 12 mm)

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

14 Rx: Nonsurgical vs Surgical (no prospective controlled clinical trials) w Nonsurgical: Patients without major neurological deficits (rest, traction, collar, PT, pain rx, bl/bwl) w 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

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

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

17 Epidural Extramedullary

18 Metastatic Epidural SCC (MESCC) w 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 w Primary site: breast, lung, prostate 50% (others: lymphoma, renal, MM) 9% of cases have unknown primary tumor Children: neuroblastoma, sarcoma, lymphoma

19 MESCC (cont.) w Epidural SCC occurs in 5% w Path: cord compression/ischemia  edema, demyelination, hemorrhage, cystic necrosis w 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.) w Dx: MRI (contrast), CT, myelo, bone scan w Rx: CSF chemo, steroids, XRT –radiosensitive: breast, prostate, lymphoma Surgical considerations: tumor removal, diagnosis, deterioration after XRT, spinal instability or bony compression w 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 w Important considerations: Pain Rx, Psychological Rx w 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 w 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 w Paraneoplastic Syndrome w 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/ total rads  importance: spacing & size of radiation field  Rec: total dose < 6000 rads (given over 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 w 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 Spinal Hemorrhage  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%

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 w Infectious: Viral (HIV, HSV, Polio) Bacterial Fungal (TB) Parasitic (Syphilis) w 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  syphilitic meningitis, Sx: BS or ACS  tabes dorsalis – DRG (“Shingles”) + post columns, males, 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) w Path: multifocal SC lesions (demyelination), +/_ asso w/ cerebral, optic or cerebellar MS w Clinical: asym spastic paresis, incont. “Lhermitte” w Dx: MRI, evoked pots., CSF (oligoclonal IgG) w 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

51 Friedreich’s ataxia  Spino-Cerebellar ataxia  Path: posterior column degeneration, mild cortospinal tract degeneration  genetic linkage (chromosome 9)  Clinical: ataxia, asymmetric weakness, pes cavus, kyphoscoliosis  Steadily progressive, Onset: 1st/2nd decade  Rx: trial of 5-HT to modify symptoms

52 Conclusions: NT/SCI  Clinical: Acute (vascular, inflam) vs chronic (SS, Tumor, MS)  spasticity (SS), fever (infectious)  Dx: MRI (SS, tumor, syrinx), CSF (inflam.), EMG (MND), post-viral(ATM)  Asso. Dz. (SS, tumor, aneurysm, AIDS etc.)  Rx: (SS, extramed. tumors, hematoma, SCD, syph, abscess)  determine early!

53 Conclusions NT/SCI (cont.):  Important medical morbidities, functional disabilities  significant % of rehab. Admissions  Etiologies: spinal stenosis, tumor, vascular  Successful rehabilitation outcomes  Future research necessary to study Nontraumatic spinal cord diseases & outcome

54 Thanks for your attention !!!

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