Guillain-Barre- Syndrome

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25 F seen urgently after developing visual loss
OD pain behind the eye over 2 days. PMHx
negative. Visual acuity is 20/200 in the affected
eye, and some swelling of the optic disc is seen.
There is abnormal perception of the color red.
Brain MRI is normal. What treatment should be
provided?

• A. Oral prednisone
• B. IV solumedrol
• C. IVIg
• D. Baclofen
• E. thymectomy

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25 F seen urgently after developing visual loss
OD pain behind the eye over 2 days. PMHx
negative. Visual acuity is 20/200 in the affected
eye, and some swelling of the optic disc is seen.
There is abnormal perception of the color red.
Brain MRI is normal. Approximately what of
patients with optic neuritis and normal brain MRI
develop clinically definite MS in the next 10
years?

• A. 1
• B. 5
• C. 20
• D. 40
• E. 90

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20-year-old college student is seen by an
ophthalmologist 2 days after developing foggy
vision OS associated with pain on moving that
eye. Visual acuity is 20/400 and papillitis is
noted. MRI of the brain shows 2 non-enhancing
hyperintense T2 lesions. How much more likely is
this patient to develop CDMS than someone with
optic neuritis and a normal baseline scan?

• A. Equal chance of developing CDMS
• B. Slightly greater chance of developing CDMS
• C. Over 2 times as likely to develop CDMS
• D. Five times as likely to develop CDMS
• E. Ten times as likely to develop CDMS

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ONTT
100
6 m
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ONTT
100
placebo
6 m
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ONTT
100
placebo
prednisone
6 m
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ONTT
100
IVMP
placebo
prednisone
6 m
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Presence of MRI Lesions Predict Development of MS
51 gt 3 lesions
37 1-2 lesions
16 No lesions
ONTT Optic Neuritis Treatment Trial Number of
lesions present at baseline The exact
relationship between MRI findings and the
clinical status of patients is unknown Arch
Neurol 1993 Aug50(8)841-6
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Which of the following drugs is most likely to
produce clinical worsening in a patient with MS?

• A. Celecoxib
• B. Daclizumab
• C. Estriol
• D. Infliximab
• E. Phenytoin

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Patients with relapsing MS

• Can await further definition of their stage of
  illness before starting therapy
• Should be under treatment with a disease
  modifying drug
• Should consider enrolling in a MS trial
• Should investigate bee-sting therapy

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45F w/ MS 15 yrs ago optic neuritis that
resolved 6 y ago R leg weakness that resolved
over next 5 years had several more episodes, many
of which didnt improve completely. She now has
residual leg weakness and needs a
wheelchairWhich best describes her course of MS?

• Clinically isolated syndrome
• Relapsing-remitting
• Primary progressive
• Secondary progressive
• benign

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Natural History Of MSClinical and MRI Measures
Measures of brain volume Relapses and impairment
MRI Total T2 Lesion Area MRI activity
Time
Hartung HP et al. The Lancet 20023602018-2025.
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28F seen in ED w/ 2D h/o numbness weakness in
lower extremities. urinary / bowel incontinence.
Exam shows normal CN, upper extremities. Loss of
PP below umbilicus decreased vibration at toes,
Bilateral Babinsky Brisk reflexes in lower
extremities. MRI shows enhancing lesion in cord
extending from T8-T10The most appropriate
treatment at this point is

• Interferon Beta IM
• IV acyclovir
• IV methylprednisolone
• Prednisone p.o.
• Neurosurgical decompression

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45F is seen 24 h after developing acute loss of
vision OS. PMHx is negative. Family hx
significant for a sister with RA. On examination,
she only has light perception OS and an APD.
Brain MRI is normal. 6months later, she develops
numbness weakness in both legs, which
progresses to quadriplegia with bowel/bladder
involvement over the next 3 days. MRI of the C/T
spine reveals an elongated T2 hyperintense lesion
from C3 to C8. Which of the following studies
is most likely to be abnormal in this patient?

• A. Brain stem auditory evoked response
• B. CSF oligoclonal banding
• C. Neuromyelitis optica IgG Ab
• D. Repeat brain MRI
• E. Serum vitamin B12 level

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Neuromyelitis Optica

• Devics syndrome
• Optic nerve
• spinal cord spans several segments
• CSF-OCBs and brain MRI often normal
• Rx steroids, IVIg, ?Rituximab

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Guillain Barré syndrome
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Which of the following represents a criteria for
GBS?

• Stocking/glove sensory loss
• Initial distal weakness
• Hyper-reflexia Babinski sign
• Bowel Bladder dysfunction
• Cyto-albuminologic disassociation

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GBS

• Ascending paralysis
• Areflexia
• Cyto-albuminologic disassociation

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GBS - Time to Maximal Weakness
N 162
Patients
Weeks
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GBS - Onset to 1st Improvement
N 153
Patients
Weeks
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Which of the following represents appropriate
therapy for GBS?

• Plasma exchange (plasmapheresis)
• IV Steroids
• IVIg
• IVIg followed by Plasma exchange

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Plasma Exchange in GBS - French Study
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Plasma Exchange in GBS N American Study
N 245
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Dutch IVIg vs. PLEX study
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Which is the best method to monitor respiratory
function in GBS?

• match test
• VC
• Respiratory Rate
• Pulseox
• ABG

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Dysautonomia in GBS
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GBS - Treatment

• Supportive
• Immunomodulation
• Rehabilitation

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GBS - Supportive Treatment

• nutritional
• DVT prophylaxis
• pulmonary hygiene
• psychiatric
• PT, OT, Speech

• skin care
• infections
• pain management
• bowel / bladder

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