Rationale: BRASH syndrome is a relatively unknown medical entity in which there is a combination of bradycardia, renal injury, hypoperfusion, and hyperkalemia. It is clinically essential to take these manifestations as a syndrome rather than isolated findings because they are interrelated and have synergistic effects. Bradycardia can result in hypoperfusion, which can cause renal injury. The resultant renal injury causes hyperkalemia (which can also be the initial trigger), which potentiates the bradycardia. Deteriorating patients with the syndrome usually do not respond to regular Advanced Cardiac Life Support resuscitation protocols. Treatment focused on the timely replacement of fluids and electrolytes gives better outcomes. It is vital to keep BRASH syndrome in diagnostic possibilities while seeing patients with refractory bradycardia, hyperkalemia, and renal injury, especially when other diagnoses are ruled out. Patient concerns: In this report, we present a 64-years-old gentleman who came with generalized fatigue, non-bloody diarrhea, vomiting, and low oral intake for the past 5 days. Diagnoses: The patient was diagnosed with BRASH syndrome. Interventions: The patient received intravenous fluids, 2 doses of atropine 0.5 mg and received dextrose 50 percent with insulin regular 10 units, and salbutamol 5 mg for hyperkalemia. He was intubated due to a low Glasgow Coma Scale and received dialysis for resistant hyperkalemia. A transvenous pacemaker was inserted due to bradycardia. Outcomes: The patient had 2 cardiac arrests and could not survive the second. Lessons: BRASH is a life-threatening yet largely underdiagnosed condition. Physicians should keep a high index of suspicion for BRASH while seeing patients with resistant and self-potentiating bradycardia, hyperkalemia, and renal failure, as a timely diagnosis is crucial in the management. Variable clinical presentations and limited literature create a diagnostic challenge. Further studies are warranted to understand the pathophysiology and develop better and accurate management algorithms. Patients’ risk of developing BRASH syndrome should be considered while prescribing causative medications (Atrioventricular nodal blocking drugs such as beta-blockers) in hospitals and outpatient settings.

Background Tolosa Hunt syndrome (THS) is a rare disease that manifests mainly as painful unilateral ophthalmoplegia. It is caused by an inflammatory process of unknown aetiology within the cavernous sinus with a rare intracranial extension. The International Classification of Headache Disorders (ICHD)- 3 diagnostic criteria aids in its diagnosis. There is limited literature on its varied presentations, diagnosis, and management. Steroids are used in the treatment of THS with varied success. Methods We conducted a single-center-retrospective-study and included all patients admitted with a diagnosis of THS from January 2015 to December 2020. Descriptive and summary statistics were used to describe the study cohort's socio-demographic parameters. Results Among 31 THS patients (predominantly Asians (18) and Arabs (9)), visual disturbance was commonest presenting complaint. Third-nerve paralysis was seen in 70.9% cases. Magnetic-resonance-imaging (MRI) was abnormal in 64.5%. 93.5% patients received steroids, with a response-rate of 70.9% and a recurrence-rate of 9.7%. A previous history of THS and female gender were associated with recurrence ( p -value 0.009 and 0.018). Recurrence was seen in 66.7% fully recovered and 33.3% partially recovered cases (p-value 0.04). Among the benign and inflammatory subtypes of THS, the ICHD-3 criteria were applicable in 85% of inflammatory THS. Conclusions THS is a rare disease with ethnic variation in presentation and response to treatment. In our cohort female gender and a previous history of THS were associated with recurrence. ICHD-3 diagnostic criteria had a higher validity in our patients compared to prior studies, especially among the inflammatory THS.

Introduction: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a well-known cause of hemolysis. It has a notable prevalence in African, Asian, and Mediterranean countries. Favism is a common trigger of oxidative stress in G6PD deficient people, which can lead to hemolysis. Additionally, fava bean ingestion can cause methemoglobinemia (MethHgb), an abnormal variation in the Hgb in which the ferrous (Fe2+) iron in heme is oxidized to the ferric (Fe3+) state. It is rare to have both G6PD deficiency and MethHgb secondary to favism at the same time. The first-line treatment for MethHgb is methylene blue. However, in G6PD deficient patients, it can potentiate hemolysis. Methods: We reviewed the literature using PubMed and Google scholar and found 6 cases of MethHgb secondary to favism in patients with G6PD deficiency. We also analyzed two cases which are still unpublished, making a total of 8 cases. Results: All 8 cases were male with median age of 18 years (1 - 56). 1 patient had a prior diagnosis of G6PD deficiency while 7 were newly diagnosed. Median Hgb was 8.3gm/dL (4.6 - 12.5) and median MetHgb was 7.8 % (3.5 - 35). 1 patient received methylene blue, and 4 received Vitamin C. All of the patients recovered and were discharged [Table 1]. Unpublished case 1: A 56-year-old male presented with MethHgb and hemolytic anemia, secondary to fava bean ingestion. Hgb on admission and lowest recorded were 9.9 and 6.5 gm/dL, respectively. He had an SPO2 of 70% on room air and 101.2 % on ABG. Methylene blue administration worsened the hemolysis as he was G6PD deficient but not diagnosed before. He got better with discontinuation of methylene blue and Vitamin C and was discharged on day 5. Unpublished case 2: A 43-year-old male, known case of G6PD deficiency presented with MethHgb and hemolytic anemia, secondary to fava bean ingestion. Hgb on admission and lowest recorded were 12.5 and 7.4 gm/dL, respectively. He had an SPO2 of 82% on room air and 100 % on ABG. He received IV vitamin C and recovered and was discharged on day 4. Discussion: Methemoglobinemia is usually acquired, secondary to oxidative stress in the body, but can rarely be congenital. Enzyme systems such as NADH methemoglobin reductase, NADPH methemoglobin reductase, ascorbic acid, and glutathione reductase systems keep a check on the accumulation of methemoglobin in the blood. However, these mechanisms can be insufficient to counter the conversion of Hgb to MethHgb, consequently promoting an oxidative state in the body. It can be due to the overproduction of methemoglobin (secondary to exposure to certain drugs, chemicals, or food items, but can sometimes be hereditary) or under conversion to Hgb due to unavailable enzyme mechanisms. One of the causes of the inability to counteract methemoglobin can be secondary to G6PD deficiency. Patients with MetHgb have a low oxygen saturation (SPO2) on pulse oximeters but a falsely high SPO2 on arterial blood gasses (ABG). The treatment depends on symptoms and the level of MethHgb. The first step is to remove any possible precipitator if present. Symptomatic patients (and asymptomatic with a level of methemoglobin >30 %) are treated with methylene blue (1-2mg/kg), which is reduced to leuko-methylene blue via NADPH dependent methemoglobin reductase. This, in turn, reduces methemoglobin back to Hgb, correcting the abnormality [Figure 1]. Rarely, patients can present with co-occurrence of MethHgb and G6PD deficiency. In such cases, caution is required while giving methylene blue as they do not have sufficient levels of NADPH to reduce it. Otherwise, a cascade of oxidative hemolysis ensues secondary to underlying G6PD deficiency, resulting in a vicious cycle of further methemoglobinemia. The most frequent cause of this co-occurrence is the ingestion of fava beans, which can simultaneously induce MethHgb and potentiate G6PD deficiency. One of our patients had a history of favism without developing any symptoms. Only this time, he ate fava beans in a larger amount, leading to hemolysis and MethHgb. Conclusion: Favism is a rare cause of the co-occurrence of methemoglobinemia and hemolysis in G6PD deficient individuals. It is vital to identify G6PD deficiency in patients presenting with MethHgb, as the initiation of methylene blue in such individuals can result in a cascade of oxidative hemolysis. A history of fava beans ingestion without any symptoms does not rule out G6PD deficiency, as it is proportional to the number of beans ingested. Disclosures No relevant conflicts of interest to declare.