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Nontraumatic Spinal Cord Injury (NT/SCI). William McKinley MD Dept PM&R Medical College of Virginia / Virginia Commonwealth University. Objectives: NT/SCI . Importance / Incidence Literature / Prior Studies Etiologies / Clinical presentation Prognosis / Rehabilitation Outcome.

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nontraumatic spinal cord injury nt sci

Nontraumatic Spinal Cord Injury (NT/SCI)

William McKinley MD

Dept PM&R

Medical College of Virginia / Virginia Commonwealth University

objectives nt sci
Objectives: NT/SCI
  • Importance / Incidence
  • Literature / Prior Studies
  • Etiologies / Clinical presentation
  • Prognosis / Rehabilitation Outcome
importance of nt sci
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
importance nt sci cont
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)
incidence nt sci
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%
nontraumatic vs traumatic sci
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%)
nt sci vs t sci cont
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,
conclusions nt sci vs t sci
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
nt sci medical complications
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.
etiologies nontraumatic sci
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,
spinal stenosis
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)
spondylosis cont
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
rx nonsurgical vs surgical no prospective controlled clinical trials
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)
rehab outcome of myelopathy from spinal stenosis
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
neoplastic spinal cord compression scc
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)
metastatic epidural scc mescc
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
mescc cont
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%
mescc cont20
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
rehabilitation of neoplastic scc
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)
intradural extramedullary sci tumors
Intradural/Extramedullary SCI Tumors
  • Path: compression & ischemia
    • Meningioma
    • Neurofibromatosis- (cutaneous lesions, diagnosed by biopsy, early adulthood)
  • Clinical : similar to MESCC
    • often benign
intramedullary sci tumors
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
cancer related sci
Cancer-Related SCI
  • Paraneoplastic Syndrome
  • Radiation Myelitis
paraneoplastic myelitis
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
radiation myelopathy
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
vascular ischemic myelopathy
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)
vasculature of the spinal cord
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
aortic aneurysm related sci
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)
embolic ischemia
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.
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%

Spinal Hemorrhage
rheumatoid arthritis sci
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)
motor system disorders
Motor system disorders
  • Amyotropic Lat. Sclerosis (ALS)
    • Pseudobulbar Palsy - (medulla)
  • Spinal Muscular Atrophy (SMA)
  • Progressive Lateral Sclerosis (PLS) - (corticospinal pathways)
amyotropic lateral sclerosis als
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
syringomyelia
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)
inflammatory myelitis
Inflammatory Myelitis
  • Infectious:
    • Viral (HIV, HSV, Polio)
    • Bacterial
    • Fungal (TB)
    • Parasitic (Syphilis)
  • Non-infectious
    • Transverse Myelitis
    • MS
hiv aids related myelopathy
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
herpes related sci
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
poliomyelitis
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
postpolio syndrome pps
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
spinal epidural abscess
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...
spinal abscess cont
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
chronic abscess
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
syphilis sci
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
acute transverse myelitis
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
spinal multiple sclerosis ms
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
vitamin b 12 deficiency
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
friedreich s ataxia
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
conclusions nt sci
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!
conclusions nt sci cont
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