Differential diagnosis of LETM in adults
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Differential diagnosis of LETM in adults

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journal club on longitudinally extensive transverse myelitis

journal club on longitudinally extensive transverse myelitis

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Differential diagnosis of LETM in adults Differential diagnosis of LETM in adults Presentation Transcript

  • Differential diagnosis of LETM in adults Christopher Eckstein, Stephanie syc, Shiv saidha, John hopkins university,Baltimore European Neurological journal,Oct,2010
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    • TM-focal spinal cord inflammation
    • Acute complete vs Acute partial
    • LETM-TM that extends 3 or more vertebral segments in length
    • Most frequent cause is NMO
    • Consider various potential etiologies
    • Clinical, laboratory and Imaging features
  • Causes of LETM
    • Inflammatory
    • NMO
    • Sjogrens
    • SLE
    • Behcets
    • Primary CNS vascuitis
    • Multiple sclerosis
    • Infectious
    • HIV
    • HTLV
    • Neurosyphilis
    • CMV,HSV,VZV
    • Parasitic
    • Metabolic
    • Copper deficiency
    • Vit B12 def
    • Vascular
    • Dural AVM
    • Spinal cord infarction
    • Intramedullary spinal neoplasms
    • Ependymoma
    • Astrocytoma
    • Radiation myelopathy
  • Neuromyelitis optica
    • Relatively homogenous disorder
    • Monophasic or relapsing
    • Uncommon secondary progression
    • Significant morbidity
    • Poorer prognosis
  • Wingerchuk diagnostic criteria for definite NMO
    • 1.optic neuritis
    • AND
    • 2.Acute myelitis
    • AND
    • 3. Two of
    • a) contiguous spinal cord MRI extending 3 or more vertebral segments
    • b) Brain MRI not meeting diagnostic criteria for MS
    • c) NMO IgG seropositive satus
    • Spinal lesions-LE,confluent,centarlly located,>50% area of spinal cord
    • Brain lesions-linear vs oval,limited distribution periventicular
    • CSF-pleocytosis >50 cells,elevated protein,OCB on 10-30%.
    • Anti aquaporin antibody-sensitivity of 50-75%,90% specificity
    • Steroids, systemic immunosuppression.
  • Multiple sclerosis
    • Acute partial TM
    • OSMS japaneese 59% LETM
    • MRI-asymmetrical, dorsolateral,<50% of total cross sectional area
    • CSF OCB in 90%
    • Caution about confluence of multiple discrete lesions
  • Sjogren’s syndrome
    • Autoimmune rheumatologic
    • Sicca symptoms,CNS,PNS
    • TM,ON-may precede sicca
    • Acute,relapsing,progressive
    • Clinical and serological overlap with NMO
    • High index of suspician
    • CSF-moderate pleocytosis,elevated protein 100 mg/dl, Absent OCB
    • MRI-LETM 7-8 vertebral lengths
    • Steroids
    • Methotrexate, azathioprine, Cyclophoshamide
  • SLE
    • Neurologic manifestations-1-3%
    • Myelitis is concomitant with optic neuritis, depression, seizures,psychosis.
    • Systemicmanifestations,thrombosis,vasculitis
    • Variable CSF and MRI findings
    • Steroids,Cyclophosphamide,rituximab,plasmapheresis
  • Behcets disease
    • Chronic relapsing inflammatory disease
    • Neurologic 5-50%
    • M: F-4:1,japan,eastern mediterranean
    • Artropaty,colitis,thrombosis
  • Diagnostic criteria for behcet’s
    • 1.mouth sores at least 3 times in 12 months
    • 2.two of the
    • a)recurring genital sores
    • b)eye inflammation with loss of vision
    • c) Characteristic skin lesions
    • d) Positive pathergy (skin prick test)
    • Csf-pleocytosis with lymphocyte/ neutophilic predominance, rised protein
    • MRI-LETM,3-6 spinal segments, gray and white matter, confluent lesions in basal ganglia and brain stem
    • Steroids,azathiprine,infliximab,methotrexate
  • Sarcoidosis
    • Chronic granulomatous, multi systemic
    • Lungs, heart,eyes,skin
    • Neurological-5-15%
    • Myelopathy is subacute, <40, monophasic, relapsing, progressive
    • CT thorax, CT-PET,ACE levels,biopsy
    • MRI-6 vertebral segments, centrally located, cervical or thoracic, gado enhancing
    • CSF-elevated protein, pleocytosis, hypoglycorrachia, ACE 50%
    • Steroids,methotrexate,mycophenolate
  • Primary CNS vasculitis
    • Vasculitis of brain and spinal cord
    • 5-14% spinal involvement
    • Acute/ subacute,30-50 yrs
    • Diagnosis of exclusion
    • No systemic features
    • CSF-pleocytosis, protein elevation
    • MRI-central, thoracic, mostly single segment, brain scattered lesions, gado enhancing masses, meningeal enhancement.
    • Biopsy-50-75% sensitive
    • Angiography normal in 50-60%
  • HIV
    • Infectious,vasculitic,neoplastic,direct
    • Incidence of myelitis in postmortem 11-30%
    • Insidious, progressive over years, painless, early bladder and erectile dysfunction
    • Spongy degeneration and myelin loss
    • MRI-cervical cord atrophy,non enhancing lesions
    • HAART
    • Viral
    • HTLV-1
    • Cytomegalo virus
    • Herpes simplex
    • Varicella zoster
    • Bacterial
    • M.Tuberculosis
    • Treponema pallidum
    • Fungal
    • cryptococus
    • Parasites
    • Toxoplasma gondii
    • Non infectious
    • Neoplasm
    • plasmacytoma
    • Spinal astrocytoma
    • Vasculitis
  • HTLV
    • High risk,endemic southern japan, Africa, South America
    • Chronic progressive myelopathy, early bladder, sensory symptoms
    • PCr viral load
    • Hypergammaglobulinemia
    • False positive VDRL
    • CSF-lymphocytic pleocytosis, elevated protein, Anti HTLV ab
    • MRI-thoracic cord atrophy, subacute course gado enhancement in posterior and lateral column, periventicular and subcortical brain lesions
    • Steroids for rapidly progressive ilness
  • syphilis
    • Tabes dorsalis, meningo myelitis, spinal vascular myelitis, compression by gumma
    • Serologic and csf treponemal antibody assay
    • MRI-cord atrophy, extensive intrinsic cord hyperintensities
  • Other infections
    • Schistosomiasis
    • radicular pain, paraparesis and auotonomic, acute onset
    • Lower thoracic, lumbar, conus, intramedullary ,3-5 segments
    • Toxocara, echinococcus granulosus, taenia solium
  • Non inflammatory
    • Cord ischemia-1% of all strokes, ,varied etiology,apoplectic onset, anterior cord syndrome
    • Spinal AV fistula-acquired malformations, chronic ischemic state,>40 yrs, thoracic cord, pain exacerbated by pain, fluctuating course
    • MRI-centrally located extensive cord abnormalities, dilated intradural vessels as flow voids
    • Myelography
    • Spinal angiography
    • Intramedullary spinal cord tumours-ependymomas, astrocytomas, oligodendrogloma, gangliogloma, lymphoma
    • Enlargement of cord with variable gado enhancement
    • Radiation myelopathy-delayed,1-2 yrs post exposure, initial sensory symptoms, painless
    • Enlargement of cord, gado enhancement
    • LETM does not universally represent a diagnosis of NMO
    • High index of suspicion
    • Difficult to determine underlying etiology
    • rewarding
    • Thank you