Differential diagnosis Differential diagnosis of LETM in adultsof LETM in adults

Christopher Eckstein, Stephanie syc, Shiv saidha,Christopher Eckstein, Stephanie syc, Shiv saidha,John hopkins university,BaltimoreJohn hopkins university,Baltimore

European Neurological journal,Oct,2010European Neurological journal,Oct,2010

• 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 AND2.Acute myelitis AND3. 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,vas

culitis• Variable CSF and MRI findings• Steroids,Cyclophosphamide,rituximab,p

lasmapheresis

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