Today a patient presented with b/l LE weakness, something we've all probably seen a couple times in the south side and something that is ofter dismissed by ED providers. One thing to consider and evaluate for is Guillain Barre Syndrome, it is a extremely common MISSED diagnosis.
Only 25% of patients were accurately diagnoses their first visit, with the average of 2 visits needed to be correctly diagnosed!!!
The team this morning, thought of GBS, which of course is the first step, and astutely worked up the patient appropriately. So lets talk GBS!
What is it? Acute immune-mediated polyneuropathy
- Acute monophasic paralyzing illness approx 88% provoked by a preceding infection
- Mcc: C, jejuni, CMV, EBV, HIV, and now Zika!
- Can also be from immunization, trauma, surgery and bone-marrow transplantation
Variants:
Symptoms?
- We all learn the ascending b/l LE weakness, but can start in the arms in 10% of patients
- 90% of pts will have decreased or absent reflexes in UE or LE
- CAN BE PAINFUL! This is due to nerve root inflammation
- Up to 10-30% develop respiratory failure requiring intubation
- Also a/w dysautonomia and SIADH
- 50% have facial palsies or oropharyngeal weakness
Clinical Course:
- Prodromal illness--> 5-7days then onset of neuromuscular symptoms
- Rapidly progressive weakness/parethesias ( can be difficulty climbing stairs or walking)
- Plateau 2-4 weeks, often bedridden
- Recovery
- Can be rapidly progressive form with quadriplegia and resp failure within 48hrs!
- 4-15% mortality
- Complications include: sepsis, ARDS, PE, cardiac arrest
Workup:
- PE: AREFLEXIA!
- Basic labs for evaluation of other etiologies of complaints
- LP w/ CSF showing albuminocytologic dissociation (high protein normal wbc)
- EMG testing as inpatient
Management:
- 25% require intubation!
- Think of the 20-30-40 Rule when deciding to intubate:
- FVC <20
- Max Inspiratory pressure <30
- Max Expiratory pressure <40
- Plasma exchange and IVIG are most efficacious if started within the first 2 weeks of symptoms
- Get CSF first!
- NO ROLE FOR STEROIDS
Sources: EMDocs, Uptodate, EMRAP