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Bobbye Thompson, MD University of Texas Medical Branch, Galveston Division of Neurosurgery. A Case Presentation & Discussion. History. 51yo F w/several months progressive BLE weakness; pain greater in LLE Bilateral numbness just above breasts & inferiorly

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bobbye thompson md university of texas medical branch galveston division of neurosurgery
Bobbye Thompson, MD

University of Texas Medical Branch, Galveston

Division of Neurosurgery

A Case Presentation & Discussion

history
History
  • 51yo F w/several months progressive BLE weakness; pain greater in LLE
  • Bilateral numbness just above breasts & inferiorly
  • Muscle aches cramps spasms falls
  • Urinary incontinence & saddle anesthesia
  • Constipation 2wks (manual disimpaction)
  • No history of spinal operation, injury, or trauma
  • No F/C/N/V
physical exam
Physical Exam
  • Sensory deficit inferior to T1 bilaterally
  • Decreased rectal tone
  • Motor: LLE 2/5, RLE 5/5
  • Increased tone/spasticity
  • Hyperreflexia
  • Babinski: Bilateral upgoing toes
  • Gait: Able to stand with asisstance; drags LLE, circumduction
labs csf
Labs/CSF
  • Gram stain & culture: rare monocytes, no organisms isolated
  • CSF glucose: 69
  • CSF protein: 31
  • Oligoclonal bands: negative
differential diagnosis
Differential Diagnosis
  • Tumor
    • Astrocytoma
    • Ependymoma
  • Demyelinating lesion:
    • Multiple sclerosis
    • Neuromyelitis optica
    • Transverse myelitis
    • ADEM
impression
Impression
  • Progressive LE weakness
  • Imaging consistent with Intramedullary tumor at T1
  • Oligoclonal bands negative
  • Probable tumor, likely glioma
  • Proceed with C6-T2 laminectomy with biopsy and/or resection
  • Midline myelotomy firm, tan tissue
  • Frozen section: gliosis vs. low-grade glioma
      • Inflammatory infiltrate
diagnosis
Diagnosis
  • Inflammatory demyelinating lesion
  • Multiple Sclerosis
  • No further surgical intervention
  • Neurology for management of MS
  • Follow up in clinic 2 months postop
    • Motor LLE improved
    • MRI Brain & C-spine
slide14
Multiple Sclerosis (MS)

Neuromyelitis Optica (NMO)

Transverse Myelitis

Acute Disseminated Encephalomyelitis (ADEM)

Demyelinating Diseases Mimicking Tumors

multiple sclerosis
Multiple Sclerosis
  • Relapsing-remitting MS (RRMS):
    • Most common
    • 85% of MS initially diagnosed
    • Partial or total recovery between attacks
  • Secondary-progressive MS (SPMS):
    • RRMS course, but becomes gradually progressive
    • Attacks & partial recoveries may continue to occur
    • Over 60% initially RRMS progress to SPMS in 10 yrs
multiple sclerosis1
Multiple Sclerosis
  • Primary-progressive MS (PPMS):
    • Progressive from onset
    • Symptoms generally do not remit
    • Progressive disability w/o acute attacks
    • 15% of MS initially diagnosed
  • Primary-relapsing MS (PRMS):
    • Same as PPMS, but with acute attacks
multiple sclerosis2
Multiple Sclerosis
  • Clinical: Episodic, relapsing-remitting neurologic symptoms in a young adult (typically)
    • Neurological symptoms disseminated in time & space
    • Common presentations: monocular visual disturbances (optic neuritis), paresthesias/weakness (myelitis), incoordination (cerebellar), and/or diplopia (brainstem)
  • Labs: Oligoclonal bands positive, MBP elevated
    • Not specific, new tests improving sensitivity/specificity
    • Early MS vs Clinically definite
    • MBP elevated in various disease processes
  • MRI: T2 intense foci in white matter (UBOs), juxtacortical (G-W junction involving U-fibers), periventricular lesions, involve corpus collosum (perpendicular extensions)
neuromyelitis optica devic
Neuromyelitis Optica/Devic
  • Clinical: Visual loss eye(s) w/myelopathy, usually over several months; may occur simultaneously. Course can be monophasic, but ~2/3 relapsing.
    • In children: usually monophasic & preceded by infection. Recovery is often complete. ADEM variant.
  • Labs: Oligoclonal bands negative. NMO-IgG serum can be sent (indirect immunofluorescence assay)
  • MRI: Spinal cord lesions extend > 3 levels, w/o brain lesions. SC lesions are cavitating, necrotic, w/acute axonal pathology.
transverse myelitis
Transverse Myelitis
  • Clinical: focal inflammatory disorder of spinal cord; acute or subacute combination of motor, sensory, and autonomic deficits.
    • Up to 50% pt will have LE paralysis/paresis
    • >80% have numbness, paresthesias, or dysesthesias, often with a well-defined sensory level
    • vast majority experience impaired bladder function.
    • 1/3 complete recovery, 1/3 moderate disability, 1/3 severe disability.
  • Labs/MRI/Treatment: Variable
transverse myelitis1
Transverse Myelitis
  • Findings that herald diagnosis of MS:
  • asymmetric clinical findings
  • predominance of sensory deficits
  • MRI lesions <3 spinal cord segments,
  • MRI Brain: occult white matter lesions (“dirty white matter”)
  • CSF: Oligoclonal bands positive
acute disseminated encephalomyelitis adem
Acute Disseminated Encephalomyelitis(ADEM)
  • Clinical: “Postinfectious Encephalomyelitis”
    • Presents as ACUTE neurological symptoms
    • Fever, nausea, vomiting, positional vertigo, and altered consciousness (drowsy or lethargic)
    • Children following viral infxn or immunization
  • Labs: ESR, CRP, CSF pleocytosis
  • MRI: extensive lesions, many enhance w/gad (ring-enhancing lesions)
    • Basal ganglia and/or thalami involvement
    • Spares corpus collosum
  • Molecular mimicry: viral epitope & myelin epitope
other differentials
Other Differentials
  • Sjögren’s syndrome: mimics MS by producing recurrent multifocal neurological manifestations; white-matter lesions on MRI and oligoclonal bands in CSF.
  • Differentiate by: systemic manifestations (sicca-dry eyes, mouth & rheumatic features), abnormal serology (antinuclear, SSA-Ro, or SSB-La antibodies), and findings of inflammatory foci on salivary gland biopsy.
other differentials1
Other Differentials
  • Behçet’s disease: Most common CNS presentation is a subacute brainstem syndrome.
  • Differentiate by: recurrent oral ulcerations (at least 3 times in 12 months), MRI in Behçet’s disease: brain abnormalities tend to be large, diffuse lesions confined to the brainstem.
red flags
Red Flags

Atypical features that may portend alternative diagnosis (not MS):

  • Onset: too early/late (before 15-20y, after 50y)
  • Early onset may point to a genetic etiology
  • Family history: less likely MS (weak genetic assoc)
  • Normal CSF, MRI, and/or Evoked potential studies
  • Systemic signs
    • Anemia
    • Angiokeratomas
    • Cardiomyopathy
    • Proteinuria
    • Metabolic acidosis
summary1
Summary
  • History & Physical Exam, Labs & MRI findings, AND exclusion of alternative diagnoses.
  • ~90% of patients ultimately diagnosed with MS initially present with a clinically isolated syndrome (CIS), such as optic neuritis.
  • MRI can be valuable in differentiating.
  • American Academy of Neurology: on value of MRI predicting eventual conversion of a clinically isolated syndrome to clinically definite MS:
    • >3 white-matter lesions in T2 MRI is sensitive predictor (>80%) of clinically definite MS within 7-10 years
    • In Normal MRIs, <20% will have 2nd attack w/in 10y
the future
The Future
  • Development of more highly specific criteria for this spectrum of diseases.
  • Development of more reliable biomolecular markers.

Always consider demyelinating diseases in the differential diagnosis of enhancing intramedullary lesions.

    • If index of suspicion is high, further testing may be warranted.
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References