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Q: Doc, doc, got a patient with acute progression of proximal and distal weakness after the flu 3 weeks ago. Should we get NCS/EMG to confirm GBS?
A: To confirm? What are you looking for? When was the onset of weakness? If within first 2 weeks, you might get the typical demyelinating features in only 50 percent.
A: The earliest signs of neuropathy of proximal demyelination might be present - prolonged Fs, absent Hs, reduced recruitment. So while EMG/NCS findings are supportive, they aren’t required for diagnosis.
Q: How about CSF findings? Shall we look for the famous albuminocytological dissociation?
A: Well all that might be normal early on. If you see it it’s supportive certainly although not required. But there’s an important use of the CSF - to rule OUT infections or other inflammatory causes or neoplasms. So look for WCC more than 50 and be sure to check cytology.
Q: Alright, how about MRI? Surely there must be some required test for diagnosis.
A: Well you might see enhancement of the nerve roots or cauda equina. Again, it’s sensitive and supports the diagnosis but can’t tell the difference between inflammation, infiltration or leptomeningeal spread.
Q: So how DO you diagnose GBS then? Tell it to me quick and tell it to me straight.
A: As always, a good history and examination. Acute progressive sensorimotor symptoms and signs +/- radicular pain within 6 weeks after infection or vaccination. And watch out for dysautonomia and respiratory distress - and think early ICU.
Q: Alright, so we give a treatment trial I presume. What do you do?
A: Start IVIG or PLEX within 2 weeks of onset. Now if there’s a fluctuation, that is, progression within 2 months of an improvement, you can repeat the treatment. That usually means that treatment effect has worn off but the inflammation is ongoing.
A: I know the next question: No benefit of doing IVIG AND PLEX. Sounds cool, but no doesn’t work.
A: Now if there’s a deterioration more than 8 weeks after disease onset, you gotta think of CIDP cuz the management is different. It’s chronic and requires chronic immune suppression - whereas GBS is a monophasic illness and will get better on its own over time.
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