Chhabra, Nikita

Rationale: Immunoglobulin (Ig) G4–related disease (IgG4-RD) reportedly has a strong relationship with adult-onset asthma and periocular xanthogranuloma (AAPOX) and may be linked to sclerosing mucoepidermoid carcinoma (MEC). We present a rare case of IgG4-RD and AAPOX occurring in a patient with resected eosinophilic or oncocytic MEC. Patient concerns: A 52-year-old woman was referred to our rheumatology clinic in 2020 to be evaluated for suspected IgG4-RD. Diagnoses: The patient had diagnoses of periorbital xanthelasmas, worsening glucocorticoid-dependent chronic rhinosinusitis and adult-onset asthma, and cervical lymphadenopathy persisting 2 years after resection of a low-grade MEC of a minor salivary gland. Interventions: Because the patient’s symptomatic relief was glucocorticoid dependent, IgG4-RD was suspected, and she was referred to our medical center. Her amylase and lipase levels were elevated. Serum IgG4 levels were initially within normal limits, but IgG4-RD was diagnosed because of the presence of lymphadenopathy and evidence of pancreatitis, which was shown on positron emission tomography/computed tomography. Furthermore, the IgG4 levels later increased without explanation. After the patient began combination therapy with a glucocorticoid (prednisone) and methotrexate, her symptoms improved but recurred when the daily oral glucocorticoid dosage decreased below 10 mg. An excisional biopsy of her right submandibular gland in 2021 yielded results consistent with IgG4-RD. In addition, AAPOX was diagnosed, given the presence of periocular edema and plaques, adult-onset asthma, and rhinosinusitis. Outcome: The patient was carcinoma free at last follow-up and was receiving medication to treat the other conditions. Lessons: The diagnosis of these 3 concomitant, uncommon entities required approximately 7 years of medical investigations. Clinicians should know that IgG4-RD, AAPOX, and MEC may occur together.

Abstract Objective To compare the artificial intelligence‐enabled electrocardiogram (AI‐ECG) atrial fibrillation (AF) prediction model output in patients with migraine with aura (MwA) and migraine without aura (MwoA). Background MwA is associated with an approximately twofold risk of ischemic stroke. Longitudinal cohort studies showed that patients with MwA have a higher incidence of developing AF compared to those with MwoA. The Mayo Clinic Cardiology team developed an AI‐ECG algorithm that calculates the probability of concurrent paroxysmal or impending AF in ECGs with normal sinus rhythm (NSR). Methods Adult patients with an MwA or MwoA diagnosis and at least one NSR ECG within the past 20 years at Mayo Clinic were identified. Patients with an ECG‐confirmed diagnosis of AF were excluded. For each patient, the ECG with the highest AF prediction model output was used as the index ECG. Comparisons between MwA and MwoA were conducted in the overall group (including men and women of all ages), women only, and men only in each age range (18 to <35, 35 to <55, 55 to <75, ≥75 years), and adjusted for age, sex, and six common vascular comorbidities that increase risk for AF. Results The final analysis of our cross‐sectional study included 40,002 patients (17,840 with MwA, 22,162 with MwoA). The mean (SD) age at the index ECG was 48.2 (16.0) years for MwA and 45.9 (15.0) years for MwoA ( p < 0.001). The AF prediction model output was significantly higher in the MwA group compared to MwoA (mean [SD] 7.3% [15.0%] vs. 5.6% [12.4%], mean difference [95% CI] 1.7% [1.5%, 2.0%], p < 0.001). After adjusting for vascular comorbidities, the difference between MwA and MwoA remained significant in the overall group (least square means of difference [95% CI] 0.7% [0.4%, 0.9%], p < 0.001), 18 to <35 (0.4% [0.1%, 0.7%], p = 0.022), and 35 to <55 (0.5% [0.2%, 0.8%], p < 0.001), women of all ages (0.6% [0.3%, 0.8%], p < 0.001), men of all ages (1.0% [0.4%, 1.6%], p = 0.002), women 35 to <55 (0.6% [0.3%, 0.9%], p < 0.001), and men 18 to <35 (1.2% [0.3%, 2.1%], p = 0.008). Conclusions Utilizing a novel AI‐ECG algorithm on a large group of patients, we demonstrated that patients with MwA have a significantly higher AF prediction model output, implying a higher probability of concurrent paroxysmal or impending AF, compared to MwoA in both women and men. Our results suggest that MwA is an independent risk factor for AF, especially in patients <55 years old, and that AF‐mediated cardioembolism may play a role in the migraine–stroke association for some patients.

External iliac vein stenosis related to cycling has rarely been reported as a cause of deep vein thrombosis. Ischemic stroke occurring in this condition due to paradoxical embolism across a preexisting patent foramen ovale (PFO) has yet to be reported. Here we report a case of embolic ischemic strokes in a young, avid cyclist with no prior known vascular risk factors. A thorough cerebrovascular workup revealed a right-to-left shunt on transesophageal echocardiogram that prompted venous thrombosis evaluation. Pelvic MR venogram demonstrated a 3.5 cm high-grade stenosis of the right external iliac vein, concerning for possible prior thrombotic disease. His strokes were deemed most likely a result of paradoxical emboli originating in the pelvis at the site of right external iliac vein stenosis. The patient ultimately opted for PFO closure for secondary stroke prevention, as he wished to continue daily cycling. This case highlights the importance of neurohospitalists considering iliac vein stenosis as a potential cause of embolic stroke of undetermined source, especially in young patients who are avid cyclists, as part of a thorough vascular workup.

Background Retinal migraine is defined by fully reversible monocular visual phenomena. We present two cases that were complicated by permanent monocular vision deficits. Cases A 57-year-old man with history of retinal migraine experienced persistent monocular vision loss after one stereotypical retinal migraine, progressing to finger-count vision over 4 days. He developed paracentral acute middle maculopathy that progressed to central retinal artery occlusion. A 27-year-old man with history of retinal migraine presented with persistent right eye superotemporal scotoma after a retinal migraine. Relative afferent pupillary defect and superotemporal visual field defect were noted, consistent with ischemic optic neuropathy. Conclusion Retinal migraine can complicate with permanent monocular visual loss, suggesting potential migrainous infarction of the retina or optic nerve. A thorough cerebrovascular evaluation must be completed, which was unrevealing in our cases. Acute and preventive migraine therapy may be considered in retinal migraine patients, to mitigate rare but potentially permanent visual loss.

ABSTRACTBACKGROUND AND PURPOSEInvestigation for patent foramen ovale (PFO) is warranted in patients with cryptogenic stroke (CS), as PFO closure is recommended in select CS patients for secondary stroke prevention. Transcranial Doppler (TCD) is noninvasive and has high sensitivity for PFO screening. However, 10% of the population has insufficient temporal bone windows to perform standard TCD monitoring of the middle cerebral arteries (MCAs). Prior reports showed similar diagnostic accuracy between the basilar artery and MCAs insonation. Our objective was to assess the accuracy of transforaminal insonation of the basilar artery (TIBA) in diagnosis of right‐to‐left shunt (RLS) in patients with inadequate temporal windows.METHODSWe performed a retrospective, single‐center, observational study of TIBA in patients with CS and inadequate temporal windows. We compared the PFO screening accuracy using TIBA versus echocardiogram, either transthoracic (TTE) or transesophageal echocardiogram (TEE).RESULTSSixteen females and four males were included (mean age 63.2). All patients underwent TTE, 10 (50%) underwent gold‐standard TEE. Nine patients had positive PFO with both TCD and echocardiogram. Two patients with negative echocardiogram had TCD‐positive shunt. Three patients had PFO present on echocardiogram, whereas TCD was negative. Six patients had absent shunt with both TCD and echocardiogram. The sensitivity and specificity of TIBA for RLS detection were 75%, positive predictive value 82%, and negative predictive value 67%.CONCLUSIONPFO screening accuracy using TIBA was 75%. Prospective evaluation of CS patients with TIBA and comparison to the gold‐standard TEE should be performed to further guide clinical practice.