Metastatic Meningioma of the Neck: A Case Report and Systematic Review

xlomafota13 shared this article with you from Inoreader Metastatic Meningioma of the Neck: A Case Report and Systematic Review Via O.R.L. Journal for oto-rhino-laryngology and its borderlands Background: Although meningiomas are the most common central nervous system neoplasms, extracranial metastases are exceedingly rare. There are even fewer reports of metastatic meningiomas to the neck. Methods: We described a patient with multiply recurrent orbital meningioma with metastasis to the neck found incidentally during neck exploration for composite resection and free tissue reconstruction. We performed a systematic review for all records pertaining to metastatic meningiomas to the cervical regions. Results: We found 9 previous reports of cervical metastatic meningiomas. Almost all cases underwent extensive local resection. There was no evidence of an association between the histological grade of the tumor and risk of metastasis to the neck. Cervical lymph node dissemination is more common in patients presenting after previous primary tumor resection. Conclusions: In the context of a neck mass, our findings suggest that metastatic meningioma should be included in the differential diagnosis, especially in patients with previous resections. ORL View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Oculo-auriculo-vertebral spectrum: new genes and literature review on a complex disease

xlomafota13 shared this article with you from Inoreader Oculo-auriculo-vertebral spectrum: new genes and literature review on a complex disease Via oto-rhino-laryngologie J Med Genet. 2022 Feb 2:jmedgenet-2021-108219. doi: 10.1136/jmedgenet-2021-108219. Online ahead of print. ABSTRACT Oculo-auriculo-vertebral spectrum (OAVS) or Goldenhar syndrome is due to an abnormal development of first and second branchial arches derivatives during embryogenesis and is characterised by hemifacial microsomia associated with auricular, ocular and vertebral malformations. The clinical and genetic heterogeneity of this spectrum with incomplete penetrance and variable expressivity, render its molecular diagnosis difficult. Only a few recurrent CNVs and genes have been identified as causatives in this complex disorder so far. Prenatal environmental causal factors have also been hypothesised. However, most of the patients remain without aetiology. In this review, we aim at updating clinical diagnostic criteria and describing genetic and non-genetic aetiologies, animal models as well as novel diagnostic tools and surgical managemen t, in order to help and improve clinical care and genetic counselling of these patients and their families. PMID:35110414 | DOI:10.1136/jmedgenet-2021-108219 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Association Between Clinical Symptoms, Electrodiagnostic Findings, Clinical Outcome, and Prodromal Symptoms in Patients With Bell's Palsy

xlomafota13 shared this article with you from Inoreader Association Between Clinical Symptoms, Electrodiagnostic Findings, Clinical Outcome, and Prodromal Symptoms in Patients With Bell's Palsy Via otol rhinol Ann Otol Rhinol Laryngol. 2022 Feb 3:34894221075107. doi: 10.1177/00034894221075107. Online ahead of print. ABSTRACT OBJECTIVE: This study aimed to determine which prodromal symptoms frequently occur in patients with Bell's palsy and evaluate the association between these symptoms and clinical severity of paresis or the severity of facial nerve injury. MATERIALS AND METHODS: The study included 86 patients with Bell's palsy between August 2018 and April 2020. Severity l evels of Bell's palsy and facial nerve damage were evaluated using the House-Brackmann (H-B) grading scale and electrodiagnostic study, respectively. Subsequently, a self-reported questionnaire on prodromal symptoms was administered. To assess the degree of recovery, the H-B grade was reported at 9 weeks and 6 months after the onset of paralysis. RESULTS: The most common prodromal symptoms were postauricular pain, sensory decline in the tongue, headache on the affected side, myalgia, facial sensory decline on the affected side, taste impairment, and dry eye. Taste impairment was significantly correlated with severe facial paralysis reported at 9 weeks after onset (P < .05) and was not related to the severity of paresis assessed at initial examination or 6 months after onset or on electrodiagnostic findings. CONCLUSIONS: The prodromal symptoms of Bell's palsy were not associated with the severity of facial nerve injury in an electrodiagnostic study. Taste impair ment was related to clinical severity of paralysis at subacute stage, 9 weeks after onset, but it was not associated with long-term prognosis. PMID:35109694 | DOI:10.1177/00034894221075107 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Replacement and Immunomodulatory Activities of 20% Subcutaneous Immunoglobulin Treatment: A Single-Center Retrospective Study in Autoimmune Myositis and CVID Patients

xlomafota13 shared this article with you from Inoreader Replacement and Immunomodulatory Activities of 20% Subcutaneous Immunoglobulin Treatment: A Single-Center Retrospective Study in Autoimmune Myositis and CVID Patients Via Traditional dysphagia therapy Front Immunol. 2022 Jan 17;12:805705. doi: 10.3389/fimmu.2021.805705. eCollection 2021. ABSTRACT BACKGROUND: Immunoglobulin (Ig) replacement therapy represents a life-saving treatment in primary antibody deficiencies. The introduction of subcutaneous Ig (SCIg) administration brings a major improvement in quality of life for patients, compared to the traditional intravenous administration. In recent years, an additional role has been proposed for Ig therapy for various inflammatory and immune-mediated diseases. Consequently, the use of SCIg has expanded from immunodeficiencies to immune-mediated diseases, such as polymyositis (PM) and dermatomyositis (DM). Given the rarity of these conditions, it is still difficult to evaluate the real impact of SCIg treatment on PM and DM, and additional data are constantly required on this topic, particularly for long-term treatments in real-life settings. AIM: This study aimed to increase the knowledg e about the anti-inflammatory and immunomodulatory effects of SCIg treatment for myositis. To this aim, a long-term evaluation of the effectiveness of 20% human SCIg treatment (20% SCIg, Hizentra®, CSL Behring) was carried out in patients with PM/DM in care at our Center. In addition, an evaluation of the 20% SCIg therapy in CVID patients was provided. This analysis, beside adding knowledge about the use of SCIg therapy in this real-life setting, was intended as a term of comparison, regarding the safety profile. RESULTS: Results support the beneficial effect and tolerability of long-term 20% SCIg therapy in PM/DM patients, reporting a significant improvement in creatine kinase levels, muscle strength, skin conditions, dysphagia, disease activity (MITAX score) and disability (HAQ-DI score). None of the patients reported systemic reactions. The duration of the reported local reactions was a few hours in 80% of the patients, and all resolved spontaneously. CVID patie nts reported an improvement in all the considered effectiveness parameters at the end of 20% SCIg therapy. The frequency of the adverse events reported by PM/DM patients was not different from what reported in CVID patients, where the use of SCIg therapy is more consolidated. CONCLUSIONS: This study suggests that 20% SCIg treatment represents a viable and safe treatment for PM/DM patients and a valid therapeutic alternative to IVIg, with important advantages for patients' quality of life. PMID:35111165 | PMC:PMC8801806 | DOI:10.3389/fimmu.2021.805705 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Surgical strategy for squamous cell carcinoma of the external auditory canal: management of locally advanced cases with skull base involvement

xlomafota13 shared this article with you from Inoreader Surgical strategy for squamous cell carcinoma of the external auditory canal: management of locally advanced cases with skull base involvement Via Journal of Neurological Surgery Part B: Skull Base J Neurol Surg B Skull Base DOI: 10.1055/a-1733-2585 Objective Surgical indications for advanced-stage squamous cell carcinoma (SCC) of the external auditory canal (EAC) are highly dependent on the skull base surgery team. The aim of this study was to evaluate the surgical outcomes in patients with SCC of the EAC and to clarify the surgical indication of far advanced cases using the T4-subclassification. Methods Patients with SCC of the EAC who underwent curative treatment from 2002-2021 were retrospectively reviewed at our hospital. Clinical and surgical results, including operative data, overall survival (OS), and disease-specific survival (DSS) were analyzed. To clarify the surgical indication for advanced stage, we proposed the T4-subclassification. Results In 46 patients, the tumors were in T1 stage in 8 patients, T2 in 10, T3 in 5, and T4 in 23. The 5-year DSS with T1, T2, T3, and T4 tumors were 100%, 85.7%, 100%, and 61.7%, respectively. No prognostic impacts for margin status were found between the 5-year OS and DSS (p = 0.23 and p = 0.13). Patients with far advanced stage (T4b) tumors were significantly associated with shorter DSS than those with early stage (T1/T2) and advanced stage (T3/T4a) tumors (p = 0.007 and p = 0.03). Conclusion The present study focused on patients with SCC of the EAC at a University hospital over a period of 20-years, especially with skull base involvement, and a T4-subclassification was proposed. Complete tumor resection in an en bloc fashion could help to achieve a good survival rate even in patients with locally advanced tumors. [...] Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany Article in Thieme eJournals: Table of contents  |  Abstract View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.