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Nontraumatic Spinal Cord Injury (NT/SCI)

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Presentation on theme: "Nontraumatic Spinal Cord Injury (NT/SCI)"— 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 Importance / Incidence Literature / Prior Studies
Etiologies / Clinical presentation Prognosis / Rehabilitation Outcome

3 Importance of NT/SCI Morbidity Loss of Function Cost of care
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, , 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: Outcomes:
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* % % Orthostasis* % % DVT* % % Pneumonia* % % Aut. Dysreflexia* % % Wound infection % % 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 Rx: Nonsurgical vs Surgical (no prospective controlled clinical trials)
Patients without major neurological deficits (rest, traction, collar, PT, pain rx, bl/bwl) 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) 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 Epidural Extramedullary

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/ 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
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
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 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)
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

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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