Love means never having to say you have a polyneuropathy
Gillian Bowshard is a 2 y/o presenting the ED with hypotonia, fever and worsening over the last week - is it GBS?
FEVER should NOT be present in isolated GBS. Useful in younger children vs. infectious conditions
Geff Binnysash is 8 y/o presenting with 3 weeks of progressive lower limb weakness complicated by radicular pain and inability to fully close lips, bradycardiac but afebrile. Additional exam finding reveals hyporeflexia DTRs b/l lower extremities.
What is Likely !
This case has the four pseudocardinal symptoms of GBS - B/l lower limb weakness with hyporeflexia, dysautonomia (bradycardia), radicular pain, and facial/bulbar palsy.
Giddyup B. Sucklehoney is a 6 y/o presenting with 2 months of progressive u/l right leg weakness and urinary incontinence. Afebrile.
What is Unlikely !
This has multiple signs that make it unlikely to be GBS - time course (2-4 weeks to peak of symptoms) is a feature that is relatively preserved over all cases with a few exceptions. Additionally, the weakness is unilateral.
In children with GBS, LMWH may be indicated
What is Yes !
If they have significant lower extremity weakness, DVT ppx is an important part of treatment
6 y/o with extreme and hyperacute abdominal pain, with spontaneous resolution. At bedside pt. is now lethargic. Of note, mom points out a petechial rash around both ankles.
What is HSP
Clinical criteria: MUST have rash + 2/4 of the following: abdominal pain, arthralgias, hematuria/proteinuria, biopsy signs consistent with HSP
10% of children present with a lethargic variant. This is thought to be related to intussusception, as seen. The current theory is there is a very robust physiologic pain control response that essentially snows the child
George Blatsnout is a 17 y/o with 3 months of progressive but asymmetric weakness of b/l lower leg weakness
The TIME COURSE for GBS is clinically distinctive - usually the nadir of symptoms is at 2 weeks, 90% of patients have started to have reached peak symptoms by 4 weeks
Granny Bigblocks is a 7 y/o arriving with features concerning for GBS without fever. LP performed with the following results
Lymphocytic pleocytosis to 25, elevated protein to 500.
What is Could Be !
Albuminocytologic dissociation is classic buzzword for GBS, meaning cells <5 but significantly elevated protein, upwards of 1000. However, a minority of pts can have <50 cells, and are generally lymphocytic
Gumper Ballingstone is a 12 y/o presenting over 2 weeks with radicular pain with loss of sensation in all extremities. Afebrile, normocardiac. Exam pertinent for diffuse hyporeflexia.
What is - Could be !
Pure sensory GBS is a known variant. Although muscle weakness will be mild/not present, pt.'s generally will still exhibit the same timeline and hypo/areflexia.
Gallus Blugsby is a 9 y/o male. When he was three, he had influenza, which was followed by some leg pain and mild weakness. Parents are unsure of the details. He is presenting to your office now with pain his hands and feet that has been slowly increasing of the last 2 years.
What is Could Be !
CIDP is a possible complication of GBS. The pathology is thought to be in the context of individuals who are genetically predisposed to autoimmunity creating anti-myelin antibodies that are not as acute as AIDP. Typical symptoms include proximal AND distal weakness, ataxia, painful paresthesias.
1 month old with fevers, palpable splenomegaly, lethargy, and hyperferritinemia.
What is pediatric HLH -
pHLH classically presents before the age of 1. This is because genetics plays a role: the most common mutation is perforin, which is related to cell mediated apoptosis. If not functional, the innate immune system can essentially become a liquid malignancy.
Hyperferrtinemia >10,000 has >90% sense and spef for HLH in adults, the point being the higher the ferritin the more likely this condition
Greg B. Slackskin is a 5 y/o male presenting tachycardiac but afebrile with b/l neuropathic pain over the last week with hyporeflexive DTRs b/l lower legs
What is Likely
The 4 "cardinal (most common)" symptoms seen in GBS are hyporeflexia, progressive b/l lower extremity weakness starting distally, dysautonomia, and radicular pain
Gilly B. Sinbox is a 4 y/o male with symptoms consistent with GBS or autoimmune encephalitis with polyneuropathy. Primary team initiates high dose corticosteroids with significant improvement in symptoms
What is Unlikely !
Despite being an autoimmune polyneuropathy (an alternative term for GBS), multiple trials have shown NO benefit (and possible worsening of disease). First line therapy is IVIG.
Gall Breckingsill the 4th is a 6 y/o with hyporeflexia, muscular weakness, and radicular pain developing over 2 weeks. Primary team is concerned for GBS and gather an LP -
No CSF abnormalities identified.
What is Could Be !
Up to 20% of pts. with GBS will have a NORMAL csf at 2 weeks.
Gim BuSash is a 12 y/o with hx of GBS that occurred approximately 8 weeks after getting the yearly influenza shot. At his WCC, his mother is concerned about getting meningitis and HPV vaccine.
What do you recommend?
The estimated cases of GBS linked to vaccines is 1-2 in a million, and may be incidental given the rarity. There is no contraindication to continuing vaccines.
16 y/o with x1 week of worsening abdominal pain, RUQ. Imaging, US/CT, negative. LFTs reveal ALT of 900, AST 100, normal bilirubin/albumin. ANA is 1:80. IgG is elevated. Viral hepatitis panel is negative, no EToH use, and is on no medications.
What is Autoimmune Hepatitis
This is an important reminder that ANA positivity is wildly non-specific. Autoimmune hepatitis can have varying serological markers, including monoclonal IgG proliferation, ANA+, AMA+, Smooth Muscle AB +, ANCA+. Clinical criteria: ALT predominant transaminitis, one of the both serological markers, exclusion of other common causes (Etoh, drugs, viral hep)
ANA can be positive in essentially ANY autoimmune phenomenon, including organ specific causes: hepatitis, pancreatitis, thyroiditis, etc.
Gilza Busbank is a 8 y/o with worsening left arm and right leg weakness distal to proximal over the last 2 weeks, afebrile, accompanied by new dysphagia
What is Could Be
Children, like with many diseases, can present ATYPICALLY. It is not unknown for there to be asymmetric weakness. The dysphagia could be a bulbar palsy, which is associated commonly with GBS.
Giraldino Blimps is a 7 y/o presenting with dysautonomia, dyspnea, radicular nerve pain and b/l LE weakness over 2 weeks. Who would you like to speak to?
Dr. Sadof, Dr. Pineda, Dr. Chang or Dr. Rosen?
What is CAROL
ANY dyspnea with a pt. presenting with GBS symptoms is an indication for immediate PICU admission. GBS is known to cause a very rapid decline in respiratory function, upwards of 30% over 24 hours.
Goofus B Sump is a 7 y/o presenting afebrile but tachycardiac with the inability to close his eye lids completely, dysphonia and radicular pain presenting over 3 weeks. Exam pertinent for hyporeflexia
What is Likely !
Although this pt. seems to have symptoms that from a surface level does not seem like GBS, but it is important to remember bulbar and facial palsies are very common. This pt. has CN V and CN X involvement.
Provider A sees a pt. with radicular pain and lower extremity weakness that has progressed over 2 weeks, exam pertinent for areflexia. LP revealing 100 protein and 4 lymphocytes. An MRI is ordered to evaluate for GBS
Provider B sees a pt. with urinary retention, right hand and right leg weakness, LP revealing 25 lympocytes and 800 protein, an MRI is ordered to evaluate for GBS
Which of these is inappropriate and why?
Provider A
GBS when apparent by clinical exam and LP is diagnostic - MRI is recommended ONLY if pt.'s case COULD be consistent with GBS but it is unclear. MRI will reveal edema and hyperintensity of dorsal nerve roots.
3-month-old arriving with reports of 1 week of high fever, cervical lymphadenopathy, and rash. Labs reveal a low albumin, elevated platelets, significantly elevated inflammatory markers, and a leukocytosis. Blood cultures with no growth x24 hours. You recommend a Ua by straight cath to eval for possible UTI, but your attending actually recommends a clean catch over a cath.
What is Incomplete Kawaski's
Incomplete Kawaski's is more common outside the normal age range (<3 months or > 5 years). Defined as having SOME kawaski's symptoms with elevated inflammatory markers and three of the following: Anemia, thrombocytosis, hypoalbuminemia, ALT transaminitis, leukocytosis, or pyuria. Notably - this is DISTAL pyuria. If a straight cath is performed there is actually a high chance of missing it.
Gigi Barnacles is a 15 y/o with x3 weeks of ataxia, complicated by encephalopathy and eye muscle weakness, hemodynamically stable and afebrile. Exam reveals truncal ataxia and ophthalmoplegia.
What is Likely !
Bickerstaff Encephalitis is a GBS variant characterized by the triad of encephalitis, ophthalmoplegia and ataxia. GBS symptoms can present but muted (i.e. limb weakness, normal appearing reflexes).
Gus Bits'o'Honey is a 10 y/o presenting with 2 weeks of radicular pain, documented ileus, vomiting, diarrhea, and urinary incontinence with bradycardia and fever. Exam pertinent for hyporeflexia and mild abdominal pain.
What is Could Be !
Pandysautonomia is another variant of GBS, where the presentation is primarily signs of autonomic dysfunction (I.E. fever/hypothermia, hyper/hypotension, diarrhea/constipation, increased/decreased salivary functions etc etc.). GBS symptoms can be present but are mild in comparison.
Gabby BlipSlip is a 11 y/o presenting afebrile with ataxia, eye weakness over 2 weeks. Exam pertinent for areflexia. This case be diagnosed via serology
What is Likely !
Miller Fisher Syndrome (Ataxia, opthalmoplegia, and areflexia) and Bickerstaff Encephalitis (MFS + encephalopathy) are GBS variants characterized by GQ1b antibodies, which are theorized to target myelin. These be present nearly 100% of the time with these syndromes.
Fever, areflexia, radicular pain, inability to close eyelids, jaundice, symptoms progressing to worst point over 2 weeks, respiratory depression, glevus nessasarium, ileus, continued progression of symptoms at 2 months, tachycardia, dysphagia
Fever, areflexia, radicular pain, facial/bulbar palsy, dysautonomia, and progression of symptoms over 2 weeks - all consistent. The remainder are inconsistent. Glevus Nessarium is particularly inconsistent because I made it up while writing this
The following symptoms, if not easily explainable by another process, should prompt evaluation for this disorder, particularly in older adolescents:
Spontaneous VTach, New AV-block, parotid swelling, hepatosplenomegaly, CN VII palsy, isolated elevation in ALP, anterior uveitis, and hypercalciuria.
What is Sarcoidosis
Pearl: Sarcoid is effectively on every differential. There are findings EXTREMELY suggestive of sarcoid (b/l hilar LAD +/- u/l paratracheal LAD also known as the 1-2-3 sign, lupus perinio, optic neuritis, elevated ACE levels) and then are findings that should raise a red flag - those mentioned in the question.