Case of the Month – October 2017 – Answer

We had only one response to this most recent edition of Case of the Month, and Dr. Birnbaum hit the nail right on the head.

Check here for the complete vignette.

To summarize:

The patient is a 28-year-old man with a history of systemic lupus erythematous with numerous complications, and recent medication non-compliance who presented to the ED with non-traumatic back pain refractory to over the counter medications, and during his ED course, developed rapidly ascending paralysis with concurrent urinary retention. His exam was notable for absent sensory, motor function, and reflexes below his xiphoid process; imaging studies indicated an inflammatory or demyelinating process within his spinal cord.


What is your differential diagnosis for this patient?

The patient’s clinical presentation is consistent with neuromuscular weakness, which is either due to pathology affecting the upper motor neuron, lower motor neuron, neuro-muscular junction, or the muscles themselves. His symptoms are not related to frequency of muscle use, eliminating neuromuscular junction disorders such as myasthenia gravis from the differential diagnosis. The presence of sensory symptoms eliminates pure a muscular disorder from the differential. Thus, his pathology can be attributed to dysfunction with his lower motor neurons and/or his upper motor neurons. The sensory and motor deficits, the lack of reflexes, and autonomic dysfunction generate a broad differential, including an inflammatory polyneuropathy like Guillain-Barre syndrome (GBS), transverse myelitis, multiple sclerosis, malignancy, cord compression, or infection [Ganti, 2016]

The constellation of symptoms, ascending paralysis with numbness, is most consistent with classical Guillain-Barre syndrome (as opposed to the Miller-Fisher variant which progresses in a proximal to distal direction) [Ganti, 2016]. By contrast, transverse myelitis usually causes hyperreflexia and a positive Babinski sign along with neurologic symptoms depending on the involved spinal cord level, though atypical cases may be characterized by areflexia, rapid ascending paralysis, and sensory deficits, with symptom progression occurring between 4 hours and 21 days [Frohman, 2010]. This confusion between GBS and transverse myelitis has implications for therapy (more on this later).


What, if any, further tests would you perform on this patient?

MRI is the most important immediate diagnostic test to order as it can evaluate for cord compression and help with determining disposition. The lack of cord compression on MRI eliminates any indication for neurosurgical intervention, and points towards a demyelinating (like GBS), inflammatory (like an autoimmune disorder like MS or transverse myelitis), or less likely, malignancy.

A close second is measurement of the pulmonary function tests, including his negative inspiratory force (NIF) and forced vital capacity (FVC) because they can indicate the severity of the patient’s presentation, and point you towards his or her ultimate disposition. The general rule is a NIF (which measures the maximum negative pressure the patient can generate when inhaling) greater than -30 cm H20 (e.g. a NIF of -15 cm H2O) or a FVC (which measures the largest volume that a patient can exhale) less than 20 mL/kg correlate with an increased risk of respiratory failure [Lawn, 2011].

That said, these parameters should be serially measured, and used in conjunction with the patient’s overall presentation, including work of breathing, vital signs, and overall clinical picture [Ibid; Farkas, 2015]. In short: if the clinical gestalt gives you any concern for impending respiratory failure, then you should strongly consider intubating the patient and consulting the ICU.

While a lumbar puncture would help with diagnostic differentiation between GBS and other inflammatory myelopathies, it will not determine this patient’s disposition, and could be performed after patient disposition. Though the classic USMLE Step 1 buzzword for Guillain-Barre is “albuminocytologic dissociation,” or elevated protein without elevated cell count, this may not be the case during the first week of the illness, let alone the first few hours like with our patient. Unfortunately, transverse myelitis can have similar LP findings of elevated protein and immunoglobulins [Ganti, 2016].


How would you manage this patient?

The first step is the same as with every patient: assess the ABCs! If there is any concern about respiratory compromise, then consider intubating the patient and mechanical ventilation. If the clinical gestalt is suggestive of neurogenic shock, then pursue the appropriate supportive care with vasopressors.

In general, non-invasive positive pressure ventilation should be avoided in patients with concern for or impending neurogenic respiratory failure, as patients who have decompensated to the point where their diaphragms are too weak to expand the lungs will likely be afflicted for days to weeks. Additionally, the possibility of concomitant neurogenic autonomic dysfunction will make that crash intubation, when the patient has failed NIPPV, much more dicey [Rubinstein, 2016].

Treatment of the underlying primary process depends on what you think the primary process is. If the clinical presentation is more suggestive of Guillain Barre syndrome (based on the symptomatology described above, the classic LP findings of albuminocytologic dissociation), then the gold standard treatments are intravenous immunoglobulin (IVIG), and plasmapheresis, as demonstrated by two recent Cochrane reviews, with similar efficacy [Chevret, 2017; Hughes 2014]. A separate Cochrane review demonstrated that corticosteroid monotherapy did not demonstrate any improvement with the rate of recovery [Hughes, 2016].

For transverse myelitis, the primary goal is to halt and reverse the acute inflammation. The first line therapy is high dose corticosteroids, either oral or intravenous methylprednisolone, oral prednisone, or oral dexamethasone. Plasmapheresis can be considered for those cases refractory to corticosteroids [Frohman, 2010].


What is the ultimate disposition for this patient?

Again, the key decision-making tool is the patient’s performance on pulmonary function tests. If there is any concern for impending respiratory compromise, then the patient should go straight to the ICU for close monitoring and intervention.


Back to our patient…

Based on the rapid progression of his symptoms, a NIF was measured and the ICU was consulted.  Though he was able to generate a NIF of -50, he was accepted to the ICU for close monitoring, where he consistently demonstrated NIFs of -50 or greater and thus never required intubation. Subsequent to his admission, an LP was performed, with a protein of 795 mg/dL and 1160 WBCs.  He received stress dose steroids, and received broad spectrum antimicrobials based on his initial LP results, though these were discontinued when the cultures were negative. His labs were notable for significantly elevated anti-NMO titers, and on that basis he was diagnosed with NMO transverse myelitis in his C6-L1 spinal cord. He experienced no improvement with corticosteroids, and underwent several days of plasmapheresis with gradual regaining of sensation in his T4-L1 dermatomes, though he never regained urinary or fecal continence. He was eventually discharged to a subacute rehab facility, with mild improvement noted in his last follow-up appointment with Neurology about 1 month ago.

Stay tuned for our next edition of Case of the Month!



Ganti, Latha, and Vaibhav Rastogi. “Acute Generalized Weakness.” Emergency Medicine Clinics of North America, Nov. 2017, pp. 233–249

Frohman, Elliot M., and Dean M. Wingerchuk. “Transverse Myelitis.” New England Journal of Medicine, vol. 363, no. 6, May 2010, pp. 564–572.

Farkas, Josh. “Five pearls for the dyspneic patient with Guillain-Barre Syndrome or Myasthenia Gravis.” EMCrit Project, 3 June 2017,

Hughes RAC, Brassington R, Gunn A, van Doorn PA. Corticosteroids for Guillain-Barré syndrome | Cochrane,

Chevret S, Hughes RAC, Annane D. Plasma exchange for Guillain-Barré syndrome | Cochrane,

Hughes RAC, Swan AV, van Doorn PA. Intravenous immunoglobulin for Guillain-Barré syndrome | Cochrane,

Lawn ND, Fletcher DD, Henderson RD, Wolter TD, Wijdicks EF. Anticipating mechanical ventilation in Guillain-Barre syndrome.  Archives of Neurology. June 2001, 58(6) 893-898

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Bored Review – Neonatal Jaundice

Seeing neonates in the Emergency Department makes me nervous. Hopefully this review can simplify one of the more common neonatal presentations. Following are the key points emergency clinicians need to know (for life and for the boards) about neonatal jaundice.

  1.    Neonatal jaundice is often physiologic, not pathologic

Almost all newborns will develop a bilirubin level above the normal cutoff for adults (1 mg/dL) within the first week of life. This is due to increased bilirubin production and decreased hepatic bilirubin clearance. Typically, levels will gradually increase, peak around 6-8 mg/dL on the third day of life, and then decline to normal within 2 weeks. Jaundice often occurs at bilirubin levels >5 mg/dL, and many infants will have visible jaundice at some point. This normal process is termed physiologic jaundice of the newborn.


  1.    The second most common cause of neonatal jaundice is breast milk jaundice

Breast milk jaundice refers to a prolonged hyperbilirubinemia beyond the first week or two of life. It can be thought of as a persistent physiologic jaundice. The mechanism of breast milk jaundice is unclear. Neonates will often have bilirubin levels above 5 mg/dL for several weeks that typically returns to normal by 12 weeks. Patients should continue to have their bilirubin levels monitored during this time, however they should continue to breast feed as normal. If levels rise significantly above normal or if they develop a conjugated hyperbilirubinemia, they should be evaluated for pathologic causes of jaundice.


  1.    There are numerous pathologic causes of neonatal jaundice

Pathologic causes of neonatal jaundice should be suspected for:

  1. Jaundice within the first 24 hours of life
  2. Any elevated direct bilirubin level
  3. Rapidly rising or persistently elevated bilirubin levels
  4. Bilirubin levels approaching exchange transfusion cutoffs (see below)

Jaundice within the first 24 hours of life suggests ABO incompatibility between mother and neonate resulting in significant hemolysis. Typically this will occur with a mom with O type blood and a child with either A or B.


Elevated direct (conjugated) bilirubin suggests an obstructive process such as biliary atresia.

Other causes of pathologic hyperbilirubinemia include INFECTION/SEPSIS, breast feeding failure and dehydration, inherited red blood cell disorders, genetic defects in bilirubin metabolism, and reabsorption of cephalohematoma. In the Emergency Department, finding the exact cause of hyperbilirubinemia may not be as important as identifying it in the first place and initiating treatment. Unless, of course, the cause is sepsis.


  1.       Evaluation and treatment of hyperbilirubinemia is relatively straightforward

Every neonatal jaundice patient presenting to the Emergency Department should have bilirubin levels checked, both direct and indirect. If the patient is well-appearing, this may be all that is necessary. Further testing is dictated by patient’s circumstances. A jaundiced neonate <1 day old should have  a CBC and Coombs testing. Direct Coombs will look for presence of maternal antibodies on the baby’s red blood cells. Ill-appearing patients should have a full sepsis workup. Patients with elevated direct bilirubin may require surgical consultation, given the possibility of biliary tract obstruction (i.e. biliary atresia) and cholestasis.

The treatment of hyperbilirubinemia is primarily focused on preventing bilirubin-induced neurologic dysfunction (BIND) and its related, long-term neurologic complication, known as kernicterus. Bilirubin levels greater than 20-25 mg/dL place patients at increased risk of BIND. The two treatments available are phototherapy and exchange transfusion.

The American Academy of Pediatrics have two simple graphs that function as guidelines for when to use phototherapy and exchange transfusion. Patients are first divided into a risk category (low, medium, high) based on several clinical factors including prematurity. Their total bilirubin level is then plotted on the graph for their specific age. Those above the specified threshold should be treated appropriately. A simple online version of this is available at
































It should be noted that these graphs are not based on robust evidence, therefore treatment with exchange transfusion can be initiated based on clinical judgment. For example, jaundiced neonates with symptoms of BIND should proceed with exchange transfusion.


In summary:

  1.       Neonatal jaundice is often physiologic
  2.       Direct hyperbilirubinemia is always pathologic – think biliary atresia
  3.       Hyperbilirubinemia in the first 24 hours of life is always pathologic – think ABO incompatibility
  4.       Ill-appearing patients require more extensive testing – think sepsis
  5.       In well-appearing patients with jaundice, use the bilirubin graph to guide treatment with phototherapy and exchange transfusions



This is primarily a board review post and was not based upon an up to date literature review.




Maloney, Patrick. Gastrointestinal Disorders. In: Walls R, Hockberger R, Gausche-Hill M. Rosen’s Emergency Medicine: Concepts and Clinical Practice, Ninth Edition Philadelphia, PA: Elsivier Inc; 2018.


Wong, Ronald, Vinod Bhutani. 2017. Pathogenesis and etiology of unconjugated hyperbilirubinemia in the newborn. UpToDate. Accessed January 2, 2018.


American Academy of Pediatrics Subcommittee on Hyperbilirubinemia: Management of hyperbilirubinemia in the newborn infant 35 or more weeks of gestation. Pediatrics 114:297–316, 2004.

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How US Health Spending Grew $1 Trillion

US health care spending has had a notorious reputation for high spending and low outcomes.1 A new study in November’s JAMA tackles from a high level perspective the question of why the US is such an outlier with its high health care spending. Dieleman et al (2017)2 identified the most prominent cost drivers from 1996-2013 across type of care (ambulatory, inpatient, pharmaceuticals, nursing facility, emergency), demographic trends (growth, aging), disease prevalence, service utilization, and service price and intensity.


Service price and intensity accounted for more than half of the nearly $1 trillion increase in US health care spending, according to their study (red dot, top figure).2 Among the different care locations, emergency and dental care spending increases were almost entirely driven by price and intensity (last two rows, bottom figure).2


Given limitations of the data, the authors could only separate some measures of service. Service utilization (e.g. number of visits) is measured separately from price (cost per visit). However, the data could not separate price from service intensity – the amount or level of service (e.g. E/M code level) rendered in a particular transaction. For example, if the price of an inpatient hospitalization increases to $2,000 from $1,000, the data cannot differentiate whether the price went up because the same services were twice as expensive (price) or more tests were ordered (intensity), or a combination of the two. While patients may be getting more per service, many studies demonstrate that the same care in the US is simply more expensive.3,4


Knowing where the spending is growing may provide objective guidance on where to go from here. The fact that service price and intensity drives over half of the spending makes other often-cited spending drivers secondary. Such drivers may include more hospitalizations or doctor visits, population aging, medical technology, high levels of comorbidity, and lack of primary care. All of these things matter, but focusing on those areas may not be where we are going to get the most bang for our buck.


Controlling growth of price and intensity requires thinking about system-level factors. Consider, for example:

  • The role of government: How much should the government intervene to regulate prices? Currently, Medicare cannot legally negotiate for lower drug prices.5
  • Market consolidation: What is the right level of bargaining power of the buyers and sellers? Insurance companies and hospitals are in a race for consolidation – growing in size by mergers or acquisitions – to have an upper hand in negotiating payments. It is unclear if this trend will make prices higher or lower.3,6
  • Care rationing: How desirable are price increases from more extensive workups? Did the patients in their last year of life in 2011 want all of the $205B worth of interventions they received?7


This study2 points to which types of policies can have the most impact on spending increases. We should recognize that if policy makers are looking towards the role of service price and intensity, that is where physicians need to actively engage in as well. Rising prices can seem distant from our daily clinical work, but if we do not spend time shaping the policies that dictate prices, other people will. If we do not responsibly self-regulate the intensity of our work-ups, other people will.


For more posts, please visit my blog Margin and Mission.


  1. Mahon M. US Spends More on Health Care Than Other High-Income Nations But Has Lower Life Expectancy, Worse Health. Commonw fund 2015;1–4.
  2. Dieleman JL, Squires E, Bui AL, et al. Factors Associated With Increases in US Health Care Spending, 1996-2013. Jama [Internet] 2017;318(17):949–54. Available from:
  3. Anderson GF, Reinhardt UE, Hussey PS, Petrosyan V. It’s the prices, stupid: Why the United States is so different from other countries. Health Aff 2003;22(3):89–105.
  4. Squires D, Anderson C. Issues in International Health Policy U.S. Health Care from a Global Perspective: Spending, Use of Services, Prices, and Health in 13 Countries. Commonw Fund 2015;15:1–16.
  5. Cubanski J, Neuman T. Searching for Savings in Medicare Drug Price Negotiations. 2017;Available from:
  6. Dafny L. The Risks of Health Insurance Company Mergers. Harv Bus Rev [Internet] 2015;(September). Available from:
  7. Aldridge MD, Kelley AS. The myth regarding the high cost of end-of-life care. Am J Public Health 2015;105(12):2411–5.












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