Cases from Legacy Health

Seizure-associated code-switching, hemolytic uremic syndrome, and more.

Case 1: Seizure-associated code-switching

By Manuel Lewis, DO, ACP Resident/Fellow Member, and Elizabeth Estabrook, MD

The patient

A 52-year-old woman with no relevant medical history who was raised in the United Kingdom and moved to the U.S. in her twenties presented with four weeks of intermittent episodes of severe chills, déjà vu, and sensations of impending doom. Approximately three weeks prior to her admission, she had an unwitnessed collapse and had been diagnosed with a concussion. She was unable to recall the events surrounding this episode. Her symptoms became more frequent, and during another evaluation, she was diagnosed with panic disorder and was prescribed alprazolam. Her episodes persisted and became more severe, prompting the current visit to the ED.

Figure 1 MRI of the brain axial view T2-FLAIR with increased signal within the medial right temporal lobe temporal pole hippocampus adjacent basal ganglia and right thalamus arrow
Figure 1. MRI of the brain, axial view, T2-FLAIR, with increased signal within the medial right temporal lobe, temporal pole, hippocampus, adjacent basal ganglia, and right thalamus (arrow).

On admission, she was hypertensive with otherwise normal vitals. Her examination revealed a faint right-sided frontal contusion as well as abrupt transitions from an American to a British English accent. A CT of her head without contrast revealed subtle low attenuation within the right temporal lobe, and subsequent MRIs of the brain with and without contrast showed thickening of the medial right temporal lobe, temporal pole, hippocampus, adjacent basal ganglia, and right thalamus (Figure 1). An electroencephalogram demonstrated occasional focal irregular delta activity in the right posterior temporal region. She was treated with levetiracetam for suspected partial complex seizures.

Over the following two days, her episodes resolved, including the accent changes. A brain biopsy was performed, and she was discharged on levetiracetam and dexamethasone. Biopsy results confirmed anaplastic astrocytoma. She was treated with pembrolizumab, temozolomide, and radiation. However, approximately two years later, her disease progressed, and she underwent further tumor resection, followed by laser-induced thermal therapy and another course of pembrolizumab. Despite these interventions, she developed recurrent episodes of code-switching.

The diagnosis

The patient has temporal lobe seizures caused by an underlying anaplastic astrocytoma. Her accent changes are a form of code-switching, defined as transitioning between languages or language varieties during conversations. This can be seen in people who speak multiple languages or dialects in ordinary conversation and is not normally pathological. However, in this case the patient was unaware of and did not recall the episodes of code-switching but did report a history of reverting to a British English accent when she became overly excited or stressed. Studies have shown that negative emotional arousal has been associated with code-switching to an individual's first language. The phenomenon has also been observed in multilingual individuals with dementia, who were found to utilize code-switching as a communicative resource when experiencing anomia.

It was hypothesized that this patient's code-switching was the result of strong emotional stimulation and “ictal fear” provoked by seizure activity in the limbic system. Ictal fear is a rare phenomenon described as a sensation of fear before or during seizure activity, out of context to the preceding perceptions. Her feeling of impending doom is suggestive of this. Ictal fear has been associated with seizure activity in the amygdala or anterior cingulate. The amygdala is located deep in the medial temporal lobe, and its location in this case was affected by the astrocytoma.


  • Code-switching is the alternating use of language or language varieties that can be seen in people who speak multiple languages or dialects and may be a phenomenon of underlying brain pathology.
  • Some psychological phenomena, such as the sensation of impending doom or déjà vu, may be clinical manifestations of nonconvulsive seizure activity.

Case 2: Atypical hemolytic uremic syndrome in pregnancy

By Allison Miller, MD, ACP Resident/Fellow Member, and Elizabeth Estabrook, MD

The patient

A 38-year-old pregnant woman with a history of pre-eclampsia was admitted to the obstetrics service for a vaginal delivery that was complicated by hemorrhagic cervical lacerations. A massive transfusion protocol was initiated and the patient received 15 units of blood products. She was admitted to the ICU for management of hypovolemic shock. Over the first few days of her hospitalization, an evolving acute kidney injury was noted with increasing creatinine level and was presumed to be secondary to profound volume loss. Despite adequate resuscitation, her creatinine level continued to climb, peaking at 3.6 mg/dL (reference range, 0.7 to 1.2 mg/dL).

On the fifth day of admission, she was observed to have progressive anemia with a hemoglobin level of 5.9 g/dL (reference range, 13.3 to 16.2 g/dL) and thrombocytopenia with a platelet count of 38,000 cells/mm3 (reference range, 165,000 to 415,000 cells/mm3) in addition to a precipitous drop in urine output. Her examination was notable for pallor but otherwise unremarkable for signs of active bleeding. Peripheral smear revealed moderate schistocytes. Her coagulation panel and fibrinogen level were within normal limits. ADAMTS13 level was greater than 60%. This constellation of findings was consistent with an evolving microangiopathic hemolytic anemia. Plasmapheresis was promptly initiated, but she continued to deteriorate despite multiple treatments. Eculizumab, a monoclonal antibody against C5a that inhibits further activation of the complement cascade, was started on the ninth day of her hospitalization with almost immediate stabilization and eventual resolution of her illness.

The diagnosis

The diagnosis is hemolytic uremic syndrome, which is the manifestation of anemia, thrombocytopenia, and renal dysfunction as a result of widespread endothelial injury and uncontrolled activation of the complement cascade. This syndrome is predominantly a sequela of acute Escherichia coli infection in the pediatric population. However, atypical hemolytic uremic syndrome can present in adults as a response to physiologic distress of pregnancy, infection, malignancy, or autoimmune disease. Diagnostic considerations in any postpartum microangiopathic hemolytic anemia include disseminated intravascular coagulation and HELLP (Hemolysis, Elevated Liver enzymes, and Low Platelet count). The patient's normal coagulation studies and intact hepatic function ruled out these diagnoses. It is more difficult to distinguish atypical hemolytic uremic syndrome from thrombotic thrombocytopenic purpura, although a lack of neurologic involvement and the ADAMTS13 level greater than 10% favored atypical hemolytic uremic syndrome. Ultimately, the patient's hemorrhagic delivery likely disrupted the balance of the complement system and provoked hemolytic uremic syndrome. Plasmapheresis is the initial treatment of choice in adult patients with atypical hemolytic uremic syndrome. However, eculizumab is a potentially life-saving alternative for those with refractory disease.


  • Atypical hemolytic uremic syndrome can be triggered by acute physiologic distress, and postpartum hemorrhage, especially in women with prior pre-eclampsia, raises the risk for this syndrome.
  • Although plasmapheresis has historically been the mainstay of treatment, eculizumab is increasingly used as a life-saving alternative therapy.

Case 3: Recurrent pneumonia after gastric banding

By Erin Staples, MD, BScRN, ACP Resident/Fellow Member, and Elizabeth Estabrook, MD

The patient

A 62-year-old woman with a history of recurrent pneumonia four times within the past year and bariatric surgery 10 years prior presented with fever and productive cough. She had finished a 10-day course of levofloxacin two months prior, with resolution of symptoms for a week before her cough returned, followed by fevers and dyspnea on exertion. She also reported chronic daily nonbilious vomiting, often posttussive or postprandial, as well as anorexia and fatigue. Over the previous two years she had gained back the 50 pounds of weight that she had lost after her gastric banding procedure. She was a lifelong nonsmoker. She had previously lived in Arizona and California but reported no travel in the past year. There were no identified environmental exposures beyond her long-term canine companion.

Figure 2 Barium swallow showing lap band red arrow with contrast reflux into esophagus and no contrast passing beyond the band
Figure 2. Barium swallow showing lap band (red arrow) with contrast reflux into esophagus and no contrast passing beyond the band.

Admission vital signs revealed a low-grade fever (37.9 °C) and mild hypoxia (oxygen saturation of 87% on room air). Otherwise the patient was hemodynamically stable without tachypnea. On examination, she was in mild respiratory distress, lungs were clear to auscultation with no wheezes or egophony, abdominal exam was unremarkable, and extremities were without edema or clubbing. She had a leukocytosis of 23 × 103 cells/μL (reference range, 4 to 10 × 103 cells/μL) with neutrophilia, mild thrombocytosis of 540 × 103 cells/μL (reference range, 140 to 440 × 103 cells/μL), and mild hyponatremia of 131 mmol/L (reference range, 136 to 145 mmol/L). Her chest X-ray showed bilateral patchy perihilar opacities. Her chest CT showed predominantly a bibasilar centrilobular nodular pattern, with surrounding ground glass opacities and a dilated esophagus with an air fluid level seen at the level of the thoracic inlet. Bronchoscopy was deferred and an esophagogram showed severe reflux due to laparoscopic adjustable gastric band (lap band) slippage, causing stomal stenosis and gastric obstruction (Figure 2). The patient was treated with ceftriaxone, metronidazole, and omeprazole. Five mL of saline were removed from the gastric band at bedside with rapid resolution of her symptoms. A CT of the chest at six months demonstrated complete resolution, and the patient has had no recurrence to date.

The diagnosis

The diagnosis is recurrent aspiration pneumonia secondary to gastric band slippage. Commonly recognized risk factors for aspiration include older age, neurologic disorders, altered consciousness, esophageal disorders, and vomiting. In this case, gastric band slippage caused recurrent vomiting and aspiration leading to pneumonia.

Laparoscopic adjustable gastric banding (LAGB) has been seen as a less invasive and reversible bariatric surgery option for morbidly obese patients. However, it has decreased in popularity over the last 10 years due to frequent complications and inadequate weight loss and now represents fewer than 5% of bariatric surgeries performed in the U.S. Patients who have undergone the surgery need to eat smaller meals more slowly and chew food more thoroughly. Lap band slippage can occur if a patient eats too quickly or vomits frequently. The band is pushed distally, allowing for cephalad prolapse of the stomach and gastric obstruction at the band.

Typical symptoms of stomal obstruction include acid reflux, dysphasia, nausea, vomiting, abdominal pain, and anorexia. Obstruction can be diagnosed by upper gastrointestinal series demonstrating no passage of contrast beyond the band. Mild slippage is treated by deflating the band via a subdermal port in the abdominal wall, which allows for a larger band diameter, as was achieved in this case. If deflation is unsuccessful, then the band can be removed laparoscopically and a new band could be placed if the patient chooses.

Lap band slippage is the most common complication of laparoscopic gastric banding and occurs in approximately 8% of cases. Other complications of the surgery include band erosion, ischemia, megaesophagus, and pseudoachalasia.


  • Slippage is the most common complication of gastric banding, occurring in 8% of cases, and is mainly due to nonadherence to dietary restrictions.
  • Treatment for slippage may be performed at the bedside with band deflation or laparoscopically with band removal or replacement.

Case 4: Hydrogen peroxide poisoning

By Lindsay Southgate, MD, ACP Resident/Fellow Member, and Elizabeth Estabrook, MD

The patient

Figure 3 CT of the abdomen without contrast showing extensive portal venous gas in the nondependent liver region arrow
Figure 3. CT of the abdomen without contrast, showing extensive portal venous gas in the nondependent liver region (arrow).

A 28-year-old woman presented to the ED with three hours of intractable nausea and vomiting that began immediately following accidental ingestion of approximately 30 mL of an unknown substance. There was associated abdominal pain and nausea, but no hematemesis. Physical examination demonstrated tachycardia, mild epigastric tenderness to palpation, and normal bowel sounds. Laboratory studies were notable for an aspartate aminotransferase level of 144 U/L (reference range, 14 to 50 U/L) and an alanine aminotransferase level of 109 U/L (reference range, 10 to 45 U/L). A CT of the abdomen revealed portal venous gas in the liver (Figure 3). The patient was placed in Trendelenburg position for 24 hours.

Esophagogastroduodenoscopy (EGD) revealed Grade D esophagitis with severe caustic injury. The patient was found to have ingested high-grade 35% hydrogen peroxide, which her roommate had been storing as a cleaning agent. At 24 hours, a repeat CT scan showed clearing of the portal venous air. She had no further complications and declined to stay in the hospital for further monitoring.

The diagnosis

The diagnosis is hydrogen peroxide poisoning by ingestion. Hydrogen peroxide is a clear, colorless, odorless oxidizing agent that is commonly found in households and is easy to obtain in the community. High-grade 35% hydrogen peroxide is used to clean hot tubs or industrial kitchens. Hydrogen peroxide causes direct caustic injury to tissue upon ingestion, including the oral mucosa, larynx, and gastrointestinal tract. EGD should be obtained within 24 hours of ingestion to evaluate the degree of mucosal injury.

Oxygen gas is formed when catalase in cells lining the gastrointestinal tract converts hydrogen peroxide into water and oxygen, resulting in rapid absorption across the gastric mucosa into the venous system. Gas emboli form when the concentration of oxygen exceeds solubility. In this case, 30 mL of 35% hydrogen peroxide will yield 3.5 L of oxygen, which could be sufficient to cause hollow viscous perforation. Gas emboli can travel in the venous system, causing catastrophic injury. Embolism to the brain can cause cerebral vascular accident. The emboli can also accumulate in the cardiac ventricles, leading to pulmonary embolus or cardiopulmonary arrest. Placing the patient in Trendelenburg position may prevent travel of gas emboli to the central nervous system. It is important to obtain serial imaging to confirm clearance of gas emboli with repeat imaging at 24 hours, and potentially later, as serious complications can occur up to 72 hours after ingestion. Hyperbaric oxygen therapy can decrease the size of gas emboli and counter-diffuse gas into a solution to be removed by ventilation. Although there are no specific indications for hyperbaric oxygen, it should be considered in cases with significant burden of venous gas emboli, presence of arterial gas emboli, or concern for cerebral involvement.


  • In patients who have ingested hydrogen peroxide, abdominal imaging should be performed to identify gas emboli in the venous system.
  • Upper endoscopy should be performed in the first 24 hours to evaluate degree of esophageal or gastric injury.

Case 5: Nonuremic calciphylaxis

By Stephanie Frana, MD, MPH, ACP Member, and Elizabeth Estabrook, MD

The patient

A 50-year-old man with history of chronic pain, substance use disorder treated with methadone, and hepatitis C cirrhosis presented with worsening right leg pain. He had developed multiple nonhealing wounds on his right lower extremity four months prior and had four previous admissions for suspected recurrent cellulitis, each time treated with appropriate antibiotics.

Figure 4 The right lower extremity with multiple well-demarcated ulcerations with areas of granulation and necrosis
Figure 4. The right lower extremity with multiple well-demarcated ulcerations with areas of granulation and necrosis.

Admission vital signs revealed tachycardia and tachypnea (respiratory rate of 24 breaths/min), but he was afebrile and normotensive. On examination he was ill-appearing and disheveled, with dry oral mucosa and bilateral lower-extremity pitting edema. His right lower leg was erythematous and malodorous with multiple large areas of ulceration and necrosis and with serous drainage present (Figure 4). Laboratory results revealed a white blood cell count of 26.7 × 103 cells/µL (reference range, 4 to 10 × 103 cells/µL), a lactate level of 5.3 mmol/L (reference range, 0.5 to 2.0 mmol/L), an albumin level below 1.5 mg/dL (reference range, 3.5 to 5.0 mg/dL), a calcium level of 7.9 mg/dL (reference range, 8.6 to 10.3 mg/dL), and a phosphorus level of 8.0 mg/dL (reference range, 2.7 to 4.5 mg/dL). His parathyroid hormone level was 69 pg/dL (reference range, 12 to 88 pg/dL), his blood urea nitrogen level was 48 mg/dL (reference range, 6 to 20 mg/dL), and his creatinine level was 3.41 mg/dL (reference range, 0.70 to 1.30 mg/dL). Urine and blood cultures grew Klebsiella pneumoniae.

He was admitted for management of sepsis. His renal function improved with IV fluids to his baseline creatinine level of 0.96 mg/dL. Venous vascular imaging was without abnormalities. Hypercoagulability, autoimmune, and vasculitis workup was completed to evaluate for possible causes of his ulcerative condition. Factor V Leiden mutation, cardiolipin antibody, antinuclear antibody (ANA), antineutrophilic cytoplasmic antibody (ANCA), rheumatoid factor, and cryoglobulins were all negative. Punch biopsy of his ulcers revealed vascular microcalcification deposition, fat necrosis, and fibrosis.

With sodium thiosulfate, surgical debridement, and frequent wound care, his wounds improved. Adequate pain management was especially difficult, and due to this and psychosocial factors, the patient began declining regular dressing changes, further debridement, and other treatment measures. His condition clinically deteriorated, he continued to decline treatment options, and after numerous palliative care conversations, he was transitioned to comfort measures. He ultimately died on hospital day 52 from multiorgan failure.

The diagnosis

This patient was diagnosed with nonuremic calciphylaxis. Calciphylaxis more commonly has been described among patients with end-stage renal disease (calcific uremic arteriolopathy), and incidence among patients on dialysis is 1% to 4%. Calciphylaxis also can present in patients without renal disease (nonuremic calciphylaxis) but the incidence is not clearly known due to the current lack of research and often limited clinical recognition and diagnosis among this population. The very few systematic reviews and retrospective case studies have noted possible associations with hyperparathyroidism, obesity, cirrhosis, warfarin use, and glucocorticoid use. While the exact mechanism of calciphylaxis is still not fully understood, disordered homeostasis of calcium and phosphate is often involved. Microvascular calcification leads to ischemic necrosis, presenting in a spectrum of painful skin lesions from mottling to plaques or nodules, and ultimately forms necrotic ulcers. Calciphylaxis has a very poor prognosis, as even with prompt diagnosis and treatment the average survival is less than one year; most often death is due to sepsis.

The diagnosis of nonuremic calciphylaxis is made clinically, and no confirmatory test exists, although biopsy can help support the diagnosis. Nonhealing ulcerative lesions present a wide differential diagnosis, which necessitates ruling out several etiologies (vasculitis, hypercoagulability, and autoimmune workup) prior to making a correct diagnosis of calciphylaxis.

Treatment is focused on infection control (wound care, surgical debridement, antibiotics if sepsis is present), restoring homeostasis to calcium and phosphate (diet restrictions, binders, cinacalcet, possible parathyroidectomy). Although randomized studies are limited, sodium thiosulfate, which acts as an antioxidant, vasodilator, and calcium chelator, may be helpful. Lesions are extremely painful, and complex pain management is required for patients to tolerate regular wound care. Palliative care or pain medicine consultation should occur early, as nerve blocks may be required to provide adequate pain relief.


  • Calciphylaxis is rarely seen in the absence of end-stage renal disease but should be considered in patients with nonhealing, very painful cutaneous mottling, nodules, or ulcerations.
  • Calciphylaxis has a very high mortality rate even with treatment, and advanced pain management and palliative care are critical to improving quality of life.

Other cases in this issue