Presentation on theme: "Darrell Laudate 12/4/09 AM Report"— Presentation transcript:
1 Darrell Laudate 12/4/09 AM Report Acute MyelopathiesDarrell Laudate12/4/09 AM Report
2 Overview of Acute Myelopathy Spinal cord dysfunction or myelopathy, can occur due to a lesion arising within the spinal cord or due to compression of the spinal cord originating outside of itFrequently devastating, often producing quadriplegia, paraplegia, and sensory deficitsMany spinal cord diseases are reversible if recognized and treated at an early stage
3 Transverse Myelopathy vs. Myelitis Transverse Myelopathy refers to clinical presentation of severe motor, sensory, and autonomic dysfunction (bowel, bladder, and sexual abnormalities ) below a spinal cord lesion due to any acute/ subacute process affecting the spinal cord, compressive or notOften associated with back painTransverse Myelitis refers to an inflammatory process of the grey and white matter of the spinal cord
4 Transverse Myelopathies (cont.) Localization of the lesion depends upon the level of the spinal cord involved and the extent of the involvement of the various long tracts. In some cases, there is almost total paralysis and sensory loss below the level of the lesion, others only partial lossIf cervical area is involved, all four limbs may be involved and there is risk of respiratory paralysis (segments C3,4,5 to diaphragm)Lhermitte's sign: an electric shock-like sensation down the neck, back, or extremities that occurs with bending of the neckLesions of the lower cervical (C2-T1) region will cause a combination of upper and lower motor neuron signs in the upper limbs, and exclusively upper motor neuron signs in the lower limbs.A lesion of the thoracic spinal cord (T1-12) will produce a spastic paraplegia.A lesion of the lower part of the spinal cord (L1-S5) often produces a combination of upper and lower motor neuron signs in the lower limbs
5 Upper vs Lower Motor Neuron Lesions Lower motor neuron lesion is a lesion which affects nerve fibers traveling from the anterior horn of the spinal cord to the relevant muscle(s) Associated with areflexialeads to flaccid paralysis (paralysis accompanied by muscle loss)Upper motor neuron lesion is a lesion of the neural pathway above the anterior horn cell or motor nuclei of the cranial nerves and are marked by:Spasticity, increase in tone in the extensor muscles (lower limbs) or flexor muscles (upper limbs)Clasp-knife response where initial resistance to movement is followed by relaxationWeakness in the flexors (lower limbs) or extensors (upper limbs), but no muscle wastingBrisk tendon jerk reflexesBabinski or Hoffman sign is presentincrease deep tendon reflexPronator drift(Spinal Shock - loss of sensation accompanied by motor paralysis with initial loss but gradual recovery of reflexesOccurs following a spinal cord injury, thus what may have looked like a lower motor neuron lesion can later reveal itself to be an upper motor lesion)Pronator drift: indicates spasticity; person is asked to flex his arms 90 degrees at the shoulders, supinate his forearms, close his eyes and hold the position; If a forearm pronates then the person is said to have pronator drift on that side.Babinski positive if upgoingHoffman - tapping the nail or flicking the terminal phalanx of the third or fourth finger. A positive response is seen with flexion of the terminal phalanx of the thumb.
6 Important Dermatomal Landmarks C2 - posterior half of the skull capC3 - area correlating to a high turtle neck shirtC4 - area correlating to a low-collar shirtC6 - (radial nerve) 1st digit (thumb)C7 - (median nerve) 2nd and 3rd digitC8 - (ulnar nerve) 4th and 5th digit, also the funny boneT4 - nipples.T5 - Inframammary fold.T6/T7 - xiphoid process.T10 - umbilicus (important for early appendicitis pain)T12 - pubic bone area.L1 - inguinal ligamentL4 - includes the knee caps
7 Evaluation of suspected myelopathy Imaging is indicated in all patient with suspicion for myelopathyMRI is generally the most appropriate study as it images the spine, paraspinal region, and spinal cord; also may reveal evidence of intrinsic lesionsmost patients with suspected cord compression should have total spinal cord imagingsole imaging of the lumber spine is often ordered with cord compression suspicion, but recall the spinal cord ends at L1, thus visualization of the spinal cord is not possible with a lumbar MRI alonelumbar MRI is useful however to exclude cauda equina compression (lower extremity weakness and sensory and bladder disturbances without upper motor neuron signs)Exceptions may include pts with upper and lower extremity symptoms as cervical localization is likely -> Cervical MRI
8 Other Studies Lumbar Puncture Serologic Studies as clinically indicatedCT myelogram may be beneficial in patients with suspected inflammatory or demyelinative lesions of the spinal cord
10 Compressive Myelopathies Cervical spondylosisEpidural, intradural, or intramedullary neoplasmEpidural abscessEpidural hemorrhage/hematomaHerniated discPosttraumatic compression by fractured or displaced vertebra or hemorrhageClinical criteria alone cannot distinguish spinal cord compression and intrinsic cord lesions
11 Acute Viral Myelitis Two Forms Enteroviruses (poliovirus, coxsackie virus, and enterovirus 71), Flaviviruses (West Nile virus and Japanese encephalitis virus) have been known to target the gray matter (Anterior horn cells) of the spinal cord, producing acute lower motor neuron disease.1usually accompanied with fever, headache, and meningismusproduces asymmetrical flaccid weakness with reduced or absent reflexes and few sensory symptoms or signsMRI often shows hyperintensities in the anterior horns of the spinal cord on T2-weighted imaging Cerebrospinal fluid (CSF) analysis demonstrates a moderate pleocytosisThese features help to distinguish this form of viral myelitis from Guillain-Barré syndrome, which usually produces symmetric deficits, with no MRI abnormalities, and is associated with elevated CSF protein levels without pleocytosis.
12 Acute Viral MyelitisCMV, VZV, HSV I &II, HCV, and EBV are associated with a second form of viral myelitis has clinical and diagnostic test features that are similar to transverse myelitisAssociation between the myelitis and the virus is not always clear, some may represent post-infectious transverse myelitis, others, a positive polymerase chain reaction (PCR) test in the CSF suggests that the myelitis is directly related to the viral infectionTreated with Herpes zoster, HSV, and EBV myelitis are treated with intravenous acyclovir (10 mg/kg q8h) or oral valacyclovir (2 gm tid) for 10–14 days; CMV with ganciclovir (5 mg/kg IV bid) plus foscarnet (60 mg/kg IV tid), or cidofovir (5 mg/kg per week for 2 weeks).2
14 Other Infectious Myelopathies HIVMore of a chronic myelopathy, Often found mostly in late stages of AIDS and associated with AIDS related dementia in halfslowly progressive spastic paraparesis is accompanied by loss of vibration and position sense and urinary frequency, urgency, and incontinenceCSF may show nonspecific protein elevationART may reverse the symptoms3BacterialMycoplasma (acute and post infectious), Listeria monocytogenesTBvia secondary cord compression from verterbral osteomyelitis, aka Pott’s diseaseAlso via compressive tuberculomasLyme diseaseCases have been described in which clinical and MRI features resembling acute transverse myelitis have been attributed to Lyme disease.4CSF in these cases typically demonstrates a lymphocytic pleocytosis and elevated proteinSchistosomiasis (in endemic areas)
15 Tabes Dorsalis (Locomotor Ataxia) Form of tertiary neurosyphilis in which the nerves of the dorsal (or posterior) columns degenerateLoss of sense of position (proprioception), vibration, and discriminative touchLatency period of 3-20 yearsCardinal signs of tabes are loss of reflexes in the legs; impaired position and vibratory sense; Romberg's signalso:bilateral Argyll Robertson pupilsfleeting and repetitive lancinating pains, primarily in the legsParesthesias/ formincationvisceral crisis (Bladder disturbances, and acute abdominal pain with vomitingpersonality changes, dementia, deafness, visualskeletal musculature is hypotonic due to destruction of the sensory limb of the spindle reflexAtaxia of the legs and (tabetic) gait due to loss of position sense occurs in half of patients
16 Tabes dorsalis (cont.)VDRL & RPR (nontreponemal tests) may be nonreactive in late neurosyphilisIf suspcion suspicion for neurosyphilis, serum FTA-ABS or TPPA (treponemal tests) are preferredCSF may be completely normal in tabes dorsalis, or may show mild lymphocytic pleocytosis with 10 to 50 cells/microL and protein concentrations of 45 to 75 mg/dL.Syphilitic meningovascular myelitis can represent an earlier form of syphilis infectionfocal inflammation of the meninges can secondarily affect the adjacent anterior spinal artery thus result in a CVA or spinal cord infarctionTreatment - Penicillin G 3 to 4 million units IV every four hours or 24 million units continuous IV infusion for 10 to 14 days
17 Connective tissue Associated Myelopathies SLEMay be the initial feature but onset is usually present with other active lupus signs.thought to be due to an arteritis, with resultant ischemic necrosis of the spinal cordANA, ds-DNA, anti-Sm, Anti-neuronal (may correlate with active CNS lupus)Has been associated with antiphospholipid antibodies in some studies but not all.5Treatment: Prednisone (1.5 mg/kg per day), plasmapheresis, and cyclophosphamide.6Antiphospholipid antibodies a may also benefit with warfarin as well as steroids and immunosuppressive treatment.7Mixed connective tissue diseaseSjogren's syndrome (antibodies to the Ro/SSA or La/SSB)Scleroderma (ANA, anti-Scl-70, anti-centromere (ACA), anti-RNA polymerase III, and anti-beta2-glycoprotein I antibodies)Ankylosing spondylitisAcute myelopathy will typically occur in the setting of fracture of ankylosed spine or atlantoaxial- axial subluxationcauda equina sydrome rare but associated with long standing diseaseRheumatoid arthritisatlantoaxial subluxation, atlantoaxial impaction, and/or subaxial subluxationRarely associated with CNS vasculitis and more rarely with myelopathy from vasculitis
18 Sarcoid Neurosarcoidosis Typically occur perivascularly, but they can be extramedullary or intramedullary, and can involve the cauda equina.Occurs 5% of Sarcoid patientsMRI signal abnormalities are not specificneurosarcoid lesions can appear similar to transverse myelitis or can resemble a tumorCSF profile consists of variable lymphocytic pleocytosis; oligoclonal bands are present in one-third of caseGenerally treated with corticosteroids and other immunomodulatory agents and can improve
19 B12 Deficiency (subacute combined degeneration of spinal cord) Damage to peripheral nerves caused by demyelination and irreversible nerve cell death.Symptoms includeparesthesias in the hands and feetloss of vibration and position sensationprogressive spastic and ataxic weaknessLoss of reflexes due to an associated peripheral neuropathy in a patient who also has Babinski signs, is an important diagnostic clueOptic atrophy and irritability or other mental changes may be prominent in advanced casesThis myelopathy tends to be diffuse rather than focal; signs are generally symmetric and reflect predominant involvement of the posterior and lateral tracts, including Romberg's signRomberg:Ask the subject to stand erect with feet together and eyes closed. Stand close by as a precaution in order to stop the person from falling over and hurting himself. Watch the movement of the body in relation to a perpendicular object behind the subject (corner of the room, door, window etc). A positive sign is noted when a swaying, sometimes irregular swaying and even toppling over occurs. The essential feature is that the patient becomes more unsteady with eyes closed.The essential features of the test are as follows:the subject stands with feet together, eyes open and hands by the sides.the subject closes the eyes while the examiner observes for a full minute.Because the examiner is trying to elicit whether the patient falls when the eyes are closed, it is advisable to stand ready to catch the falling patient. For large subjects, a strong assistant is recommended.Romberg's test is positive if the patient sways or falls while the patient's eyes are closed
20 B12 deficiency (cont.)Usually established by the presence of decreased Vit B12 levelin the cases of low-normal B12, the presence of elevated MMA and homocysteine levels may be usefulTreatment: 1mg Cbl IM once daily for 1 week, followed by 1 mg IM every week for four weeksif the underlying disorder persists, 1 mg every month for the remainder of the patient's life.
21 Hypocuric Myelopathy (Copper Deficiency) Very similar to subacute combined degenerationProgressive spasticity, severe gait abnormalities including ataxia, and a neuropathy.Also associated with anemia and neutropenia in certain patientsMore common after gastric bypass, also with zinc supplementationDiagnosis usually confirmed with low levels of serum copper are found and often there is also a low level of serum ceruloplasminSymptoms are potentially reversible with copper supplementation and reversal of underlying cause
22 Multiple SclerosisMost common autoimmune inflammatory demyelinating disease of the CNSWomen of Northern European descent who are of child-bearing age.Histological examination of active plaques reveals perivascular infiltration of lymphocytes (predominantly T cells) and macrophages with occasional plasma cells. Perivascular and interstitial edema may be prominent.
23 Multiple Sclerosis (cont.) Multiple sclerosis (MS) is a clinical diagnosis. There are no clinical findings that are unique to this disorder, but some are highly characteristicOlder criteria considered clinical characteristics and a number of laboratory studiesthese findings were then used to place patients in categories ranging from clinically definite to laboratory supported definite to clinically probable to laboratory supported probable MSMcDonald criteria focus on a demonstration with clinical, laboratory and radiologic data of the dissemination of MS lesions in time and space, also incorporated specific MRI findings into the diagnostic schemeA diagnosis cannot be made until other possible conditions have been ruled out and there is evidence of demyelinating events separated anatomically and in time
24 Multiple Sclerosis (cont.) CSFoligoclonal bands using isoelectric focusing is the most important diagnostic CSF study when determining a diagnosis of MS2/3 will have normal leukocyte count>50cells/microliter occurs only rarely and should raise suspicion of alternative etiologyWill also see elevation of the CSF immunoglobulin level relative to other protein components, suggesting intrathecal synthesis, generally IgG MRICerebral or spinal plaques that are ovoid and hyperintense on proton density and T2-weighted studies, and they are hypointense (if visible at all) on T1-weighted images.Conventional T2-weighted MRI techniques may underestimate MS plaque size and burden, Diffusion tensor imaging and MR spectroscopy may correct thisEvoked potentials (EPs): CNS electrical events generated by peripheral stimulation of a sensory organ)Can detect abnormal CNS function that may be clinically undetectable or help define the anatomical site of the lesion in tracts not easily visualized by imaging (e.g., optic nerves, dorsal columns).
25 Neuromyelitis optica (Devic’s Disease) Autoimmune, inflammatory disorder in which the optic nerves and spinal cord are targeted but may also affect the brain,Resembles multiple sclerosis (MS) as it has varying degrees of weakness or paralysis in the legs or arms, loss of sensation (including blindness), and/or bladder and bowel dysfunction.Lesions are different from those observed in MS in that the attacks appear to be not mediated by the immune system's T cells but rather by antibodies called NMO-IgG that target aquaporin 4 of astrocytes.6Acts as a channel for the transport of water across the cell membraneFound in the processes of the astrocytes that surround the blood-brain barrier,This blood-brain barrier is weakened in Devic's disease, but it is unclear how NMO-IgG immune response leads to demyelinationCriteria: Optic neuritis, Myelitis, and one of the following:MRI evidence of a contiguous spinal cord lesion three or more segments in length, orSeropositivity for NMO-IgGTreatment: Steroids, Cytoxan, PLEX, +/- Rituximab
26 Cervical SpondylosisChronic degenerative and hypertraphic changes that involve intervertebral disks, vertebral bodies, facet joints, and ligamentsif severe, can result in narrowing of cervical spinal canal and cause spinal cord compressionIn many case series, cervical spondylotic myelopathy is the most common cause of myelopathy, particularly in older adultsCervical Spondylotic Myelopathyclinical syndrome associated with spondylosis but with spinal cord dysfunctioncommonly in pts > 55y, perhaps most common form of myelopathyinsidious onset of numbness, parathesias in upper extremities, spastic or stiff-legged gait that is often not associated with painsurgical decompression is generally used to treat if symptomatic, although there is no evidence from RCT proving the efficacy of this therapy7
27 Metastatic Disease to Spinal Cord Oncologic emergencyusually requring treatment with corticosteroids (usually dexamethasone), and emergent radiation therapy or surgeryindications for decompressive surgery for metastatic epidural spinal cord compression includes: when tissue diagnosis is needed, presence of spinal instability, or tumor is known to be radioresistantOne study showed patients who underwent anterior decompressive surgical resection of metastatic epidural spinal cord compression due to cancer had better ambulatory outcomes than with radiation therapy alone.8
28 Epidural AbscessRare, occurring in only 1 patient per 10,000 admitted to the hospital.9Most common pathogen is Staphylococcus aureus, which accounts for about two-thirds of cases 9Typically originate via contiguous spread from infections of skin and soft tissues or as a complication of spinal surgery and other invasive procedures, including indwelling epidural catheters.Expected back and/or radicular pain usually but not always accompanied systemic signs of infectionMRI preferred testRequires emergent surgical decompression and antibiotic therapy are indicated to treat epidural abscess
29 Spinal Cord Infarction Rare compared with CVAMost frequently caused by surgical procedures and pathologies affecting the aortaMay also occur in the setting of vascular risk factors or aortic diseasePresents with sudden spinal cord dysfunction that typically corresponds to the territory of the anterior spinal arteryWeakness and pinprick loss below the level of the infarction but sparing vibration and position senseNo treatment available and prognosis is variable and dependent upon severity of presenting deficit
30 Dural AVM of Spinal Cord Rare cause of ischemic spinal cord dysfunctionObstructs venous outflow of the spinal cordMay progress over months to yearsSurgical obliteration of the fistula can potentially reverse this condition
31 ReferencesSolomon et al. Infectious causes of acute flaccid paralysis. Curr Opin Infect Dis Oct;16(5):375-81Hauser et al. "Chapter 372. Diseases of the Spinal Cord" (Chapter): Harrison's Principles of Internal Medicine, 17e:Meurs et al. Acute transverse myelitis as a main manifestation of early stage II neuroborreliosis in two patients. Eur Neurol 2004; 52:186.Staudinger et al. Remission of HIV myelopathy after highly active antiretroviral therapy. Neurology 2000; 54:267.Lennon et al. IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel. J. Exp. Med (4): 473–7.Transverse myelitis as the first manifestation of systemic lupus erythematosus or lupus-like disease: good functional outcome and relevance of antiphospholipid antibodies. AU D'Cruz DP; Mellor-Pita S; Joven B; Sanna G; Allanson J; Taylor J; Khamashta MA; Hughes GR SO J Rheumatol 2004 Feb;31(2):280-5.Transverse myelopathy in systemic lupus erythematosus: an analysis of 14 cases and review of the literature. AU Kovacs B; Lafferty TL; Brent LH; DeHoratius RJ SO Ann Rheum Dis 2000 Feb;59(2):120-4.Acute transverse myelopathy in systemic lupus erythematosus: clinical presentation, treatment, and outcome. AU Mok CC; Lau CS; Chan EY; Wong RW SO J Rheumatol 1998 Mar;25(3):467-73Porter et al. Endocrine and reproductive manifestations of sarcoidosis. QJM 2003; 96:553.McCormick et al. Cervical Spndylotic myelopathy: make the difficult diagnosis, then refer to surgery. Cleve Clin J Med. 2003;70:Patchell et al. Direct decompressive surgical resection in the treatment of spinal cord compression caused by metastatic cancer: a randomised trial. Lancet ;366:643.Darouiche, RO. Spinal epidural abscess. N Engl J Med 2006; 355:2012.