chemamr
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A 44-year-old man comes to the office because of diplopia that began 10 days earlier. After the diplopia developed, he noted bilateral ptosis and gait imbalance. He had recovered from an upper respiratory infection 1 week before the onset of diplopia.
On neurologic examination, he has bilateral ptosis, severe bilateral ophthalmoplegia, mild facial weakness, and normal bulbar function. Strength in the upper and lower limbs is normal. Muscle stretch reflexes are absent, but sensation is normal. Gait is wide-based and ataxic. Cerebrospinal fluid has a mildly elevated total protein level with no cells.
what's the diagnosis? what's the treatment?
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| robin082006
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MS
Interferon beta
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| prathapdoctor
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I agree with robin, its multiple sclerosis but my doubt is why stretch reflexes are absent in this case while strength and sensations are normal.
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| chemamr
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well, the answer given is a different diagnosis, any other ideas?
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| chemamr
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but even if it were MS, the tx of an acute exacerbation would include high doses of steroids, right?
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| dr.wad
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is it Miller _FISHER syndrome ???????????????????
treatment is by plasmapheresis or immunoglobulins
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| AAzad
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GBS and or MFS they can overlap.
Clinical features
Onset
Weakness: Most often symptomatic in legs
Pain: Low back & legs
Paresthesias: Distal
Weakness
Distribution: Proximal + Distal; Symmetric
Severity: Quadriplegia in 30%; Bedbound another 30%
Respiratory failure
Vital capacity < 1 liter: Observation in ICU necessary
~33% of GBS require intubation
Indications for intubation
Vital capacity < 12 to 15 ml/kg: Especially with rapid decline
Negative inspiratory force (NIF) < 25 cm H2O
Hypoxemia: PaO2 < 80 mm Hg
Difficulty with secretions
Time of onset: 7 days
Time on respirator: 50% < 3 weeks
Usually 2° to muscle weakness
Occasionallly related to aspiration
Cranial Nerves (70%) Cranial Nerves (70%)
VII
Symmetric: Occurs early in parallel with weakness
Asymmetric
Occurs later in disease course
Other weakness may be stable or improving
Extra-ocular: Overlap with Miller-Fisher
Tongue: Symmetric; Common (50%)
Sensory
Paraesthesias: Initial symptom in 50%; Eventually occur in 70% to 90%
Pain
Prominent in 70%
Associations
Neuropathy: In back, hips & legs at onset; Myalgias; Occasional radicular
Immobility: Myalgias
Recovery phase: Distal; Legs > Hands; Dysesthesias
Loss
Distal; Symmetric
All modalities involved
Tendon reflex loss
Early in most (70%) but not all patients
Progressive reduction during 1st week
Distribution: Ankles most frequently lost; Biceps most frequently spared
Associations: Sensory loss; Weakest limbs; Distal
Spared reflexes all during disease course suggests another diagnosis
Autonomic dysfunction
Frequency: 60%
More common in more severe syndromes
Blood pressure
Transient hypertension or, less often, hypotension
Increased sensitivity to anti-hypertensive medications
Cardiac arrhythmias: Sinus tachycardia; Bradycardia
Bladder: Urinary retention; Sphincter symptoms in 10% to 15%
GI: Ileus
Test: Bilateral ocular pressure x 25 sec; Produces bradycardia (< 40 bpm)
Course
Usually improves
Edited by AAzad on 01/18/07 - 10:31 PM
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| chemamr
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correct 
it's a Miller Fischer Sx that is a variant of Guillain Barré Sx.
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| The-surgeon
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guillian barre syndrome
what goes with it
1.CSF finding is typical called(protein-cell dissociation)
2.the definition of gullian berri is post infective demylination the usual infection is respiratory as in this case
treatment lasmapherisis,IV IG,if paralyisis involve respiratory muscles assist ventillation is indicated
no rule for steroids
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