Successful rituximab therapy in adult-onset IgA vasculitis with diffuse alveolar hemorrhage and renal failure: a case report.

Background: Immunoglobulin A vasculitis (IgAV) is a rare condition that most commonly presents during childhood. Patients with adult-onset IgAV are more likely to exhibit severe symptoms at presentation with worse renal outcomes. Pulmonary manifestations of adult-onset IgAV have been described rarely in the literature and often indicate higher morbidity and mortality. Given the rarity of alveolar hemorrhage in IgAV, the literature describing the clinical entity and offering management recommendations is insufficient. Case Description: We describe a patient with known adult-onset IgAV who presented with one month of abdominal pain, bloody stools, new skin lesions, and progressive shortness of breath. She developed rapidly worsening hypoxic respiratory failure associated with a hemoglobin drop and diffuse pulmonary infiltrates on imaging. Bronchoscopy demonstrated progressively hemorrhagic effluent on bronchoalveolar lavage (BAL) consistent with a diagnosis of diffuse alveolar hemorrhage (DAH). She developed acute renal failure requiring the initiation of emergent renal replacement therapy. Given concomitant DAH and acute renal failure, methylprednisolone and rituximab (RTX) therapy were initiated. With this treatment regimen, she exhibited marked improvement in respiratory function and complete renal recovery. Conclusions: This case highlights the importance of considering DAH as a rare and life-threatening pulmonary manifestation of adult-onset IgAV. Our case demonstrates the novel and successful use of RTX in combination with steroids to treat a patient with adult-onset IgAV presenting with concomitant DAH and renal failure.

Clinical impact of magnetic resonance neurography in patients with brachial plexus neuropathies.

OBJECTIVE: To study the impact of brachial plexus MR neurography (MRN) in the diagnostic thinking and therapeutic management of patients with suspected plexopathy. METHODS: MRN examinations of adult brachial plexuses over a period of 18 months were reviewed. Relevant data collection included-patient demographics, clinical history, pre-imaging diagnostic impression, pre-imaging treatment plan, post-imaging diagnosis, post-imaging treatment plan, surgical notes and electrodiagnostic (ED) results. Impact of imaging on the pre-imaging clinical diagnosis and therapeutic management were classified as no change, mild change or substantial change. RESULTS: Final sample included 121 studies. The common aetiologies included inflammatory in 31 (25.6%) of 121 patients, trauma in 29 (23.9%) of 121 patients and neoplastic in 26 (21.5%) of 121 patients. ED tests were performed in 47 (38.8%) of 121 patients and these showed concordance with MRN findings in 31 (66.0%) of 47 patients. Following MRN, there was change in the pre-imaging clinical impression for 91 (75.2%) of 121 subjects, with a mild change in diagnosis in 57 (47.1%) of 121 patients and a substantial change in 34 (28.0%) of 121 patients. 19 (15.7%) of 121 patients proceeded to therapies that would not have been performed in the same manner without the information obtained from MRN. CONCLUSION: MRN of the brachial plexus significantly impacts clinical decision-making and should be routinely performed in suspected brachial plexopathy. Advances in knowledge: MRN significantly impacts the diagnostic thinking and therapeutic management of patients with suspected brachial plexopathy. MRN not only provides concordant information to ED tests in majority of cases, but also supplements with additional diagnostic data in patients who are ED negative.

Repetition suppression in the medial temporal lobe and midbrain is altered by event overlap.

Repeated encounters with the same event typically lead to decreased activation in the medial temporal lobe (MTL) and dopaminergic midbrain, a phenomenon known as repetition suppression. In contrast, encountering an event that overlaps with prior experience leads to increased response in the same regions. Such increased responding is thought to reflect an associative novelty signal that promotes memory updating to resolve differences between current events and stored memories. Here, we married these ideas to test whether event overlap significantly modulates MTL and midbrain responses-even when events are repeated and expected-to promote memory updating through integration. While undergoing high-resolution functional MRI, participants were repeatedly presented with objects pairs, some of which overlapped with other, intervening pairs and some of which contained elements unique from other pairs. MTL and midbrain regions showed widespread repetition suppression for nonoverlapping pairs containing unique elements; however, the degree of repetition suppression was altered for overlapping pairs. Entorhinal cortex, perirhinal cortex (PRc), midbrain, and PRc-midbrain connectivity showed repetition-related increases across overlapping pairs. Notably, increased PRc activation for overlapping pairs tracked individual differences in the ability to reason about the relationships among pairs-our behavioral measure of memory integration. Within the hippocampus, activation increases across overlapping pairs were unique to CA1 , consistent with its hypothesized comparator function. These findings demonstrate that event overlap engages MTL and midbrain functions traditionally implicated in novelty processing, even when overlapping events themselves are repeated. Our findings further suggest that the MTL-midbrain response to event overlap may promote integration of new content into existing memories, leading to the formation of relational memory networks that span experiences. Moreover, the results inform theories about the division of labor within MTL, demonstrating that the role of PRc in episodic encoding extends beyond familiarity processing and item-level recognition. © 2016 Wiley Periodicals, Inc.

Endoscopic Endonasal Management of Olfactory Neuroblastoma: A Retrospective Analysis of 10 Patients with Quality-of-Life Measures.

OBJECTIVE: Anterior craniofacial resection has served as the traditional surgical treatment of olfactory neuroblastoma (ON). With the development of extended endonasal approaches, the opportunity exists for using minimal access techniques for management of select tumors. This study assesses the impact of endoscopic resection on ON and patient outcomes and quality of life. METHODS: A retrospective review identified 10 patients with ON (3 women, 7 men; mean age 49.1 years) who underwent endoscopic resection during the period 2010-2013. Modified Kadish staging divided the cohort into 3 stage B patients (30%), 5 stage C patients (50%), and 2 stage D patients (20%). Outcome measures included extent of resection, complications, recurrence, and preoperative and postoperative Sino-Nasal Outcome Test-20 scores. RESULTS: Gross total resection was achieved in all patients, with negative margins in 9 patients. One patient had negative frozen section pathology but was noted to have a positive posterior dural margin on final pathology. There was a 20% complication rate (pneumocephalus, ethmoid meningoencephalocele). Neoadjuvant chemotherapy and radiation were performed in 2 patients (Kadish stage C and D). Adjuvant chemotherapy and radiation were performed in 5 patients (4 Kadish stage C and 1 stage D). Postoperative radiation alone was administered in 3 patients (Kadish stage B). Analysis of postoperative Sino-Nasal Outcome Test-20 scores demonstrated no significant change relative to preoperative Sino-Nasal Outcome Test-20 scores. At the most recent follow-up examination, there was no evidence of recurrent disease in patients who underwent endoscopic resection. One patient (Kadish stage D) died during the follow-up period. Mean follow-up duration was 21.1 months. CONCLUSIONS: This series adds to the growing body of literature that suggests equivalent or improved outcomes of purely endonasal resection for select patients. Given the advanced Kadish stage of most of our patients, longer follow-up is required to determine the full applicability of purely endoscopic approaches to the treatment of ON.