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

The post Case of the Month – October 2017 – Answer appeared first on The Original Kings of County.

Case of the Month – October 2017


Get your thinking caps ready folks, for it’s time for the newest edition of Case of the Month.


The patient is a 28-year-old man with a history of SLE complicated by lupus nephritis, who presents with pressure-like mid-back pain since last night. He experienced no relief with oral naproxen, resulting in his presentation to the emergency department.  His last bowel movement and urination were approximately 8 hours prior to presentation and normal.  He denies any trauma, focal weakness, new numbness or tingling, fevers/chills, N/V, or any bowel or bladder complaints.  He does admit to not taking his lupus medications for about a month.  His only recent travel was to Georgia two months ago and Florida four months ago.  His lupus was discovered when he had nephrotic syndrome as a child, and he has never had a lupus flare, and he has not been on corticosteroids for the last year.


No known drug allergies

Medications: hydroxychloroquine 200 mg twice daily, mycophenolate mofetil 1g twice daily

PMH/PSH: As above, pulmonary embolism at age 15; bilateral hip surgery for avascular necrosis secondary to chronic steroids

SH: No toxic habits; denies previous sexual activity

ROS: As per HPI.


Physical Exam:

Vitals: T 97.4 (F), HR 63/min RR 22/min BP 127/73 mm Hg, SaO2 100% on room air

General: Young man sitting in bed, in no acute distress

HEENT: Sclera anicteric, no oropharyngeal erythema or exudates, moist mucous membranes

CV: Normal S1S2; no murmurs, rubs, or gallops

Pulm: Clear to auscultation bilaterally; no wheezes, rales, rhonchi, or crackles

Abdomen: Soft, mild/diffuse tenderness to palpation, dull to percussion

Rectal exam: Normal, no saddle anesthesia

Back: mild bilateral CVA tenderness

Neuro: CN2-12 intact.  Motor exam 5/5 bilateral upper and lower extremities; no sensory deficit; normal reflexes; no gait abnormality.




Urinalysis: negative leukocyte esterase and nitrates, no WBCs or bacteria on micro

CMP: within normal limits

CBC: WBC 2.78 K/uL Hgb 13.5 g/dL Hct 39.2% Platelets 159 K/uL


EKG: Normal sinus rhythm, rate = 61/min

CXR: normal

Lumbar and Sacral Spine X-rays: Questionable L5 spondylolysis


After you give the patient APAP/oxycodone 5/325 mg once, he begins to complain of worsening weakness in his lower extremities, and requires assistance to walk to the bathroom. Once there, he finds he is unable to urinate.  A Foley catheter is placed, and he drains approximately 400 mL of urine. You order a CT of his head to rule out an acute stroke or intracranial hemorrhage.  Over the next three hours, the patient becomes unable to move his lower extremities, loses his bilateral patellar reflexes, and develops saddle anesthesia.  You consult neurology who recommend an MRI spine.  Over the following three hours, his paralysis rises to just below his xiphoid process.



CT Head: No acute process

MRI Thoracic and Lumbar spine: No evidence of herniation, stenosis, or foramen narrowing.  Partially visualized high signal in the spinal cord to the level of the conus medularis which may represent demyelinating or inflammatory process, though primary vs metastatic malignancy cannot be excluded.



  • What is your differential diagnosis for this patient?
  • Which, if any, further tests would you perform on this patient?
  • What therapeutic strategy would use for this patient?
  • What is his ultimate disposition? Is there any additional information that you need prior to making this disposition decision?

Good luck!

The post Case of the Month – October 2017 appeared first on The Original Kings of County.

Case of the Month – July 2017 – Answer

We had three wonderful answers to last month’s COTM, but Dr. Segarra’s comprehensive response takes the cake.


For a complete review of the case, please click here.


Just to recap:


50-year-old woman with DM2, HTN, GERD, recently completed 6 months of TB therapy, presented with abdominal pain, N/V, weight loss, and anorexia. Her exam was notable for a diffusely tender abdomen, most pronounced in the right lower quadrant, and bilateral costovertebral angle tenderness. Labs were notable for a leukocytosis and bandemia of 11%, thrombocytopenia, hyponatremia of 116 (uncorrected), anion gap of 17 (20 when corrected for Albumin of 2.8), hyperglycemia of 526, creatinine of 3.23, large leukocyte esterase in the urine. CT imaging was notable for massively enlarged kidneys, ascites, hepatomegaly, and a large bulky uterus. The patient was initially tachycardic and hypotensive but after a 2L bolus, the hemodynamics improved, creatinine decreased to 2.89, glucose decreased to 435, and sodium decreased to 107 (uncorrected).


What is your differential diagnosis for the patient’s presentation?

Dr. Segarra gave a wide differential diagnosis and went through the process of paring down the possibilities. For this patient, labs ruled out DKA (no ketonemia), and CT excluded appendicitis, pancreatitis, hepatitis, cholecystitis, and bowel obstruction. The remaining diagnoses included sepsis (patient had a leukocytosis/bandemia and tachycardia), hematologic disorders, endocrine disorders such as SIADH and adrenal insufficiency, and infiltrative diseases. All of our respondents included extra-pulmonary tuberculosis as part of their differential diagnosis. The treatment regimen for drug-susceptible renal and disseminated tuberculosis is the same as for pulmonary tuberculosis, and as such, our patient had recently completed treatment (Nahid, 2016).


What antibiotics would you choose?

It all depends on what is being treated and which organisms may be responsible. Piperacillin/tazobactam was the initial choice in the ED based on the suspicion for an intra-abdominal infection as it offers coverage for both Gram negatives and anaerobic organisms (in addition to pseudomonas which was not likely to be a culprit in this case). The only risk factor that she had for TB is her previous infection; otherwise, she had no previous history of incarceration (recall that she works in the police department), and she had been adherent to her therapy (this was confirmed by the medicine team with the DOH). So, her risk for multidrug resistant TB was deemed to be low. Nevertheless, in such patients, the use of a fluoroquinolone, despite its broad-spectrum activity, would be controversial as it also is effective against Mycobacteria species and is generally given as part of a multi-drug regimen to avoid development of resistance. Piperacillin/tazobactam is an acceptable choice, but ceftriaxone with metronidazole would be most appropriate for the treatment of community-acquired intra-abdominal sepsis.

Does this patient need to be on isolation?

Based on CDC guidelines, respiratory isolation is not required. The patient had very recently completed a treatment regimen and did not have any respiratory symptoms.

How would you characterize the sodium disturbances, both on initial presentation and after the initial interventions?  How would you address them?

The sodium derangements are somewhat masked by the hyperglycemia. Her initial value indicates some degree of pseudohyponatremia, with the corrected value being either 123 or 126 depending on whether you use 1.6 or 2.4 as your correction factor. That the serum sodium decreased to 107 (112 or 115 depending again on choice of correction factor) despite infusion of crystalloid containing 154 mmol/L indicates that she is suffering from either too much free water retention or from too much loss of salt. Her corrected values still represent hyponatremia, but it is important to recognize that the concomitant hyperglycemia has an impact.

As Dr. Segarra noted, it’s helpful to break down hyponatremia based on serum osmolality and then on volume status (Spasovski, 2014). Calculation of this patient’s serum osmolarity would be affected by her hyperglycemia, but her downtrending creatinine (3.23 to 2.89 after 2L of fluids), and initial vital sign abnormalities all point towards an initial hypovolemic state. The resolution of her tachycardia/hypotension and downtrending creatinine also indicate that her initial hypovolemia had improved, and she was now closer to a euvolemic state. Euvolemic hyponatremia is usually caused by endocrine derangements such as hypothyroidism, adrenal insufficiency, and syndrome of inappropriate antidiuretic hormone (SIADH).

Further diagnostic work-up for the exact etiology of her hyponatremia would likely include serum cortisol, serum TSH, serum osmolarity, urine electrolytes, urine osmolarity, and urine creatinine. However, much of this work-up typically continues after the patient has left the ED.

Treatment of hyponatremia requires assessing whether the patient is symptomatic, characterizing the time-course during which it had developed, and clarifying the underlying etiology. Acute (e.g. developing within 48 hours) severe (Na < 125 mmol/L) hyponatremia has severe complications including altered mental status, hallucinations, seizures, coma, decerebrate posturing, respiratory arrest, and ultimately, death (Berl, 2007). While the available information does not definitively tell us how long the patient’s hyponatremia had been present, the lack of any of these signs/symptoms points towards a more chronic picture. Chronic hyponatremia may cause milder or even no neurologic symptoms (like our patient).

Assessment of time-course and presence (or absence) of symptoms will determine the treatment plan (Berl, 2007). A patient with documented acute (e.g. <48 hours) severe hyponatremia or chronic severe hyponatremia with symptoms, should received 3% hypertonic saline at 2 mL/kg/hr and IV furosemide, with a goal of 2 mmol/L/hr increase in serum sodium (Berl, 2007). Spasovski et. al describes alternative dosing regimens for treatment of symptomatic severe hyponatremia.  Patients may require ICU level of care for monitoring and administration of hypertonic saline (Berl, 2007).


A patient with an unclear time-course and moderate symptoms (symptoms not including coma or seizures) should get 0.9% saline with IV furosemide, with a goal of 0.5-2 mmol/L/hr increase and a goal of 8-10 mmol/L increase in serum sodium in the first 24 hours (Berl, 2007). Alternatively, the patient may receive a single bolus of 2 mL/kg 3% hypertonic saline over 20 minutes, cause-specific therapy, and discontinuation of any inciting factors (Spasovski, 2014).

An asymptomatic patient should have fluid restriction with dietary salt or urea (at 0.25-0.5 g/kg). Use of ADH antagonists is controversial, and based on 2014 European guidelines, it is not recommended due to the fear that it may increase the rate of sodium correction (via free water excretion) beyond a safe level (Spasovski, 2014).

In the event of overcorrection of serum sodium (e.g. a rise in >10 mmol/L during the first 24 hours, or >8 mmol/L in any 24 hour period afterwards), the saline treatment should be discontinued, and the prospect of starting D5W at 10 mL/kg over 1 hour should be discussed (Spasovski, 2014).


Fluid restriction would be appropriate for treatment of our asymptomatic patient’s hyponatremia. However, this creates a dilemma because she also has an AKI (suggested by the fact that her creatinine is downtrending after the initial 2L bolus). As such, it may also be reasonable to give 0.9% saline as detailed above, withholding the furosemide (which would counteract the goal of increasing her intravascular volume). The key is to avoid over-correction as it can cause Osmotic Demyelination Syndrome.

What is the ultimate disposition for this patient?

Ultimately the disposition depends on the treatment plan. Continuous administration of 3% hypertonic saline would likely require placement of a central line and close observation that would only be possible in an ICU. If treatment is fluid restriction, then a stepdown unit (as we have at UHB) would be appropriate as it would allow for closer observation than a floor bed, in case the patient then develops the significant symptoms of hyponatremia.


Clinical Course

This patient was admitted to the stepdown unit and initially treated with maintenance normal saline.  After approximately 12 hours, her sodium decreased to 114 (corrected). Her treatment was switched to fluid restriction and oral salt, and her home insulin regimen was restarted. The Department of Health reported that she had drug-susceptible TB and corroborated that she had completed antimycobacterial therapy. Urine AFBs were negative. Urine studies were indicative of a pre-renal etiology behind her AKI, and ruled out renal sodium loss as the etiology behind her hyponatremia. Renal, Hematology/Oncology, and Infectious Diseases were consulted. Her hyponatremia was attributed to SIADH; her leukocytosis/bandemia and initial hemodynamic instability was attributed to sepsis. Urine and blood cultures ended up growing ESBL E. coli, and her antibiotics were switched to IV meropenem. After approximately 48 hours of fluid restriction and oral salt, the serum sodium increased to 120 mmol/L (corrected). Maintenance fluid was restarted and oral salt continued, and after one week, the sodium was 130 mmol/L and remained stable until discharge.

The patient experienced dramatic improvement in her urine output during her hospitalization, and an abdominal ultrasound done prior to discharge demonstrated marked reduction (but not resolution) of her ascites. There were 24-hour intervals during which maintenance fluids were discontinued, but it was quickly restarted after the patient experienced a rise in her creatinine. Eventually, her serum creatinine decreased and remained stable at 1.5. The patient was discharged home after completion of her antibiotic course.

Unfortunately, the ultimate etiology behind the patient’s enlarged kidneys, organomegaly, and ascites was never fully elucidated during her hospitalization. Therefore, the patient was discharged with follow up appointments with gynecology (for further characterization of her large bulky uterus), nephrology (for possible biopsy of her massively enlarged kidneys), and hepatology (for her hepatomegaly and ascites).

Thanks to our respondents!  Stay tuned for the next installment of Case of the Month!



Berl, D. E. (2007). The Syndrome of Inappropriate Antidiuresis. New England Journal of Medicine, 1064-1072.

Nahid, D. A. (2016). Official American Thoracic Society/CDC/IDSA Clinical Practice Guidelines: Treatment of Drug-Susceptible Tuberculosis. Clinical Infectious Diseases, e147-e195.

Spasovski, V. (2014). Clinical Practice Guidelines on Diagnosis and Treatment of Hyponatraemia. Nephrology Dialysis Transplantation, 1-39.

The post Case of the Month – July 2017 – Answer appeared first on The Original Kings of County.