Ocular vestibular evoked myogenic potential in patients with myasthenia gravis

Publication date: Available online 26 June 2017
Source:Auris Nasus Larynx
Author(s): Mohammad El-Sayed Mojahed, Elsaeid Mohamed Thabet, Mohamed Galal El-Khateeb, Ashraf Elsayed Morgan
ObjectiveMyasthenia gravis (MG) is an archetypic disorder of neuromuscular junctions (NMJs) and autoantibody-mediated disease causing fatigable weakness of skeletal muscles with an ocular onset in up to 85%. The aim of this study was to detect extra ocular muscles (EOMs) abnormalities in MG patients using ocular vestibular evoked myogenic potential (oVEMP) n10 response.MethodsThe oVEMP was performed on 40 myasthenia gravis patients that were divided into three groups: newly diagnosed (10 patients), uncontrolled on treatment (15 patients) and controlled on treatment (15 patients) groups in addition to a control group of 10 subjects. Also a comparison of oVEMP response was held between patients with generalized and ocular MG.ResultsThe oVEMP n10 showed significant difference between the 3 study groups and the control. The n10 showed no significant difference between the newly diagnosed group and the other 2 groups. There was also significant difference between uncontrolled and controlled on treatment group and between generalized and ocular types of myasthenic patients.ConclusionThe oVEMP can be usefully used in diagnosis of new MG patients as regard n10 amplitude, threshold and AR except n10 latency with no therapeutic or monitoring value of oVEMP in MG.



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A novel strategy with combined assays for detection of anti-neutrophil cytoplasmic antibody (ANCA) in clinically ANCA-negative granulomatosis with polyangiitis patients

Publication date: Available online 27 June 2017
Source:Auris Nasus Larynx
Author(s): Kaori Tateyama, Satoru Kodama, Kan Kishibe, Yasuaki Harabuchi, Masashi Suzuki
ObjectiveGranulomatosis with polyangiitis (GPA) that is localized to the upper airway presents a diagnostic challenge because of a tendency towards anti-neutrophil cytoplasmic antibody (ANCA)-negativity. The purpose of this study was to investigate whether positivity of ANCA detection might be elicited with combined use of enzyme-linked immunosorbent assay (ELISA) kits.MethodsTwenty-nine serum samples obtained from GPA patients were used in this study. In addition to routine biochemical investigation for ANCA, tests for detecting PR3-, MPO-ANCAs, and minor ANCAs were performed with commercially available ELISA kits. Cytoplasmic (C)-ANCA and perinuclear (P)-ANCA were evaluated using the indirect immunofluorescence (IIF) technique.ResultsTwelve patients were positive for PR3- or MPO-ANCA in the clinical laboratory test, and 17 patients were negative for both ANCAs. Of the 17 ANCA-negative patients, four were positive for PR3- or MPO-ANCA, and three were positive for minor ANCA according to results obtained from six different ELISA kits. These findings indicated that performing detection tests with six different ELISA kits might improve the positivity of ANCA and might contribute to establishing the diagnosis of ANCA-associated vasculitis. Together with results from IIF, the samples of eight patients with clinically ANCA-negative results (8/17, 47.1%) were converted to ANCA-positive results, and the ANCA detection rate was significantly improved from 12/29 (41.4%) to 20/29 (69.0%, p=0.03).ConclusionsAdditional detection techniques should be used to confirm the results of clinically ANCA-negative samples, particularly when vasculitis is suspected. Minor ANCAs should also be evaluated with detection tests when PR3- and MPO-ANCA are negative.



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Residual Pure Intralymphatic Breast Carcinoma Following Neoadjuvant Chemotherapy is Indicative of Poor Clinical Outcome, Even in Node-Negative Patients.

Residual carcinoma confined to lymphovascular spaces following neoadjuvant chemotherapy (NAC) for invasive breast carcinoma is an uncommon finding. We studied pathologic features and outcome for patients with pure intralymphatic carcinoma (PIC) following NAC, a pattern of residual disease reported to have a poor outcome in the only previously published series of this entity. Six of 284 (2.1%) patients treated with NAC were studied. All 6 patients had axillary lymph node involvement before NAC. Tumors were triple-negative (n=3) and HER2+ (n=3: 2 ER+, 1 ER-). Two patients presented with clinical findings of inflammatory carcinoma. Three of 5 pre-NAC core biopsies showed lymphovascular invasion. Three patients showed complete clinical response to NAC, and 3 showed partial response. Post-NAC surgical specimens showed foci of intralymphatic carcinoma in the breast spanning an extent of 0.5 mm to 0.5 cm. Residual ductal carcinoma in situ was present in 2 cases. Four of 6 patients converted to node-negative following NAC. One patient had distant metastasis at presentation and 1 patient died of pulmonary embolism 2 months after surgery. Three of the 4 remaining patients developed distant metastasis, of which 2 first recurred locally (in mean follow-up of 46.5 mo). Patients with PIC had significant greater risk for relapse (hazard ratio, 10.18 [1.97, 52.58]; P=0.006) compared with other NAC-treated patients, after controlling for residual lymph node involvement, tumor size, tumor subtype, histologic grade, and age. Residual PIC following NAC is associated with poor outcome, including in patients that are node-negative following NAC. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.

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DICER1 Mutations are Consistently Present in Moderately and Poorly Differentiated Sertoli-Leydig Cell Tumors.

Ovarian Sertoli-Leydig cell tumors (SLCTs) are uncommon sex cord-stromal tumors associated with both germ-line and somatic DICER1 mutations, the frequency of which has varied widely in different studies (0% to 62.5%). The current World Health Organization Classification includes 3 histologic types of SLCTs (well-differentiated, moderately differentiated, and poorly differentiated); heterologous elements and/or retiform patterns may be present in moderately and poorly differentiated neoplasms. We investigated the frequency of DICER1 mutations in a series of 38 ovarian tumors initially diagnosed as SLCTs, and explored whether identified mutations were associated with specific morphologic features. Specialist pathology review performed blinded to molecular results confirmed 34 tumors to be SLCTs (22 moderately differentiated, 8 poorly differentiated; 4 well-differentiated), while the remaining 4 neoplasms were considered not to represent SLCTs. Of the 34 cases diagnosed as SLCTs, 30 (88%) harbored >=1 DICER1 mutation. All 30 moderately differentiated/poorly differentiated SLCTs contained mutations, but we did not find deleterious DICER1 mutations in the 4 well-differentiated SLCTs. Our study reports the highest DICER1 mutation frequency to date in SLCTs, with 100% of moderately differentiated and poorly differentiated tumors being DICER1-mutated. This suggests that DICER1 mutation may be a defining feature of these neoplasms. Although the number of cases is limited, well-differentiated SLCTs appear to be DICER1-independent. Moderately differentiated and poorly differentiated SLCT components often coexist with each other and form part of a spectrum, while well-differentiated SLCTs usually occur in pure form, suggesting that fundamentally, these represent 2 separate and independent tumor types with a different pathogenesis. We suggest that all patients with ovarian SLCTs undergo germ-line DICER1 mutation testing. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.

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Frozen shoulder and risk of cancer: a population-based cohort study

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Second-line pazopanib in patients with relapsed and refractory small-cell lung cancer: a multicentre phase II study of the Hellenic Oncology Research Group

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CBF1 is clinically prognostic and serves as a target to block cellular invasion and chemoresistance of EMT-like glioblastoma cells

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