Welcome to PICU Doc On Call, A Podcast Dedicated to Current and Aspiring Intensivists.
I'm Pradip Kamat and I'm Rahul Damania. We are coming to you from Children's Healthcare of Atlanta - Emory University School of Medicine.
Welcome to our episode, A Three-Year-Old with recent cough and leg weakness.
Here's the case presented by Rahul.
A 3-year-old previously healthy female presented to the hospital with a 2-week history of productive cough and congestion and the new 1-day onset of bilateral weakness. Today, the mother noticed weakness and inability to stand/walk following after shower as well as her voice becoming hoarse. She also noticed her lying more limp sitting on her lap, unable to sit up fully without her mother supporting her. She had no trouble holding up her head. The mother endorses increased fussiness but is able to be consoled. Decreased p/o intake, last meal was yesterday. About 1-2 weeks prior to this patient also had non-bloody diarrhea that resolved spontaneously after a few days.
UOP normal with 2-3 wet diapers. No difficulty breathing. No history of head trauma or trauma to lower extremities, no erythema/swelling to joints. No pain associated with leg movement. No previous difficulty with walking - developing normally otherwise. No fever, recent travel, H/O sick contact at home (sibling with URI). No allergies, immunization UTD. CMP largely unremarkable. CBC with leukocytosis to 19.72 with L shift and platelets of 647. CRP 0.3, ESR 12.
Afebrile, RR 24/min, HR 130, BP 140/86.
On PE: Patient was coughing, had a hoarse voice heart and lung exam was normal. Normal abdominal exam. No rash
Neurological exam: PERRL, (A+O) X3, 3-4/5 strength at ankles and knees and 5/5 in arms, +UE DTR's but none at patella or ankles. Has a wide-based ataxic gait and needs to hold on to the wall/furniture to ambulate.
Rahul, to summarize key elements from this case, this patient has:
Let's transition into some history and physical exam components of this case?
Any patient with acute ascending lower extremity flaccid paralysis with CSF showing acellular protein predominance should be considered to have Guillain-Barré syndrome unless proven otherwise. MRI brain spine is necessary to rule out any other etiologies such as brain tumor or spinal pathologies. Features strongly supporting the diagnosis of Guillain-Barré syndrome include a progression of onset over several days to less than 4 weeks, symmetrical involvement, painful onset, mild/absent sensory symptoms, cranial nerve involvement, autonomic dysfunction, absence of fever, and recovery 2 to 4 weeks after the onset of peak or plateauing of symptoms.
PFT measurement in GB syndrome is remembered as the 20/30/40 rule: A vital capacity < 20ml/kg, a maximum inspiratory pressure less negative than -30cm H2O, or maximum expiratory pressure of ≤ 40cm H2O. Serial measurements are required.
Rahul, what is the pathogenesis of Guillain-Barré Syndrome?
The exact pathogenesis is unknown. An immune trigger such as infection, vaccine, etc affects peripheral nerve components due to molecular mimicry. A gastrointestinal or upper respiratory tract illness within 4 weeks of presentation triggers the onset of Guillain-Barré Syndrome. Possible viral agents include cytomegalovirus (detected in 26%), Epstein-Barr virus, influenza, and human immunodeficiency virus, and bacterial triggers include *Mycoplasma*, *Haemophilus*, and, most commonly, *Campylobacter jejuni*, which accounts for 20% to 30% of US and European cases. Although rare, vaccination (influenza), surgery, trauma, transplant, lymphoma, and systemic lupus erythematosus have also been associated with GBS. Recently GBS after exposure to Zika virus has been described with most patients having a complete recovery.
Rahul, can you comment on the Guillain-Barré syndrome variants?
Acute inflammatory demyelinating polyneuropathy (AIDP) is considered synonymous with Guillain-Barré syndrome and has the best prognosis. Most prevalent form in Europe and North America.
AMAN (acute axonal motor neuropathy): Has no to minimal sensory symptoms and predominantly presents with progressive flaccid ascending quadriparesis complicated by respiratory failure. (slow recovery and high mortality rate). More prevalent in South East Asia
ASMAN: acute motor-sensory axonal polyneuropathy. Both sensory, as well as motor fibers, are involved. It’s a form of axonal GBS and is considered a variant of AMAN.
Miller Fisher Variant: The patient presents with a triad of areflexia, ataxia, and ophthalmoplegia and can progress to AIDP in some cases.
Rahul can you comment on the autonomic dysfunction in Guillain-Barré syndrome
1/2 patients diagnosed with **Guillain-Barré syndrome** will present with autonomic dysfunction such as diarrhea/constipation, bradycardia (15% of patients), followed by hyponatremia, SIADH. Others such as cardiomyopathy, syncope, urinary retention, BP instability syncope, reversible cardiomyopathy, and Horner syndrome are rarely seen. Cranial neuropathies (seen in 60% of patients) in form of bulbar weakness, facial palsy, ophthalmoplegia, and hypoglossal nerve palsy. Bradycardia may be difficult to treat and may require pacing. Patients can have excessive sweating and light-fixed pupils as a part of their autonomic dysfunction. A small percentage of patients can have paresthesias/numbness and pain.
Rahul, what are the Key Objectives and Takeaways?
More information can be found
This concludes our episode on Guillain-Barre Syndrome. We hope you found value in our short, case-based podcast. We welcome you to share your feedback, subscribe & place a review on our podcast! Please visit our website picudoconcall.org which showcases our episodes as well as our Doc on Call management cards. PICU Doc on Call is hosted by myself Pradip Kamat and my cohost Dr. Rahul Damania. Stay tuned for our next episode! Thank you!