Case report: Unilateral transnasal endoscopic marsupialization of nasopalatine duct cyst.

Nasopalatine duct cyst (NPDC) is the most common type of non-odontogenic cysts of the jaw. It has been treated with complete surgical resection using a sublabial or palatine approach. However, complete removal of the cyst can be accompanied by postoperative complications including oronasal fistula. Recently, endoscopic marsupialization for the disease has been advocated, but there are still few reports regarding the surgery. Herein, we report a case of NPDC that was treated with unilateral transnasal endoscopic marsupialization. A 43-year-old man with no relevant previous medical history was referred to our hospital for the treatment of lesion occupying the right nasal cavity. A computerized tomography scan of the sinus revealed an egg-shaped lesion with a well-defined border centered on the lower half of the nasal cavity and hard palate. Based on the site of the lesion, it was considered to be NPDC. Transnasal endoscopic marsupialization was performed to diagnose and improve nasal obstruction. Histopathological examination revealed stratified squamous epithelium without atypia, which was consistent with NPDC. Although the patient noticed paresthesia of the right upper incisor area, symptoms improved 3 months after surgery. Written informed consent was obtained from the patient for the publication of any potentially identifiable images or data included in this article. Transnasal endoscopic marsupialization for NPDC is minimally invasive and useful; however, it is necessary to build evidence for an appropriate excision range based on the position and size of the lesion.

Anti-TIF1-γ antibody and cancer-associated myositis: A clinicohistopathologic study.

OBJECTIVE: We aimed to analyze the clinical and histopathologic features of cancer-associated myositis (CAM) in relation to anti-transcriptional intermediary factor 1 γ antibody (anti-TIF1-γ-Ab), a marker of cancer association. METHODS: We retrospectively studied 349 patients with idiopathic inflammatory myopathies (IIMs), including 284 patients with pretreatment biopsy samples available. For the classification of IIMs, the European Neuromuscular Center criteria were applied. Patients with CAM with (anti-TIF1-γ-Ab[+] CAM) and without anti-TIF1-γ-Ab (anti-TIF1-γ-Ab[-] CAM) were compared with patients with IIM without cancers within and beyond 3 years of myositis diagnosis. RESULTS: Cancer was detected in 75 patients, of whom 36 (48%) were positive for anti-TIF1-γ-Ab. In anti-TIF1-γ-Ab(+) patients with CAM, cancers were detected within 1 year of myositis diagnosis in 35 (97%) and before 1 year of myositis diagnosis in 1. All the anti-TIF1-γ-Ab(+) patients with CAM satisfied the dermatomyositis (DM) criteria, including 2 possible DM sine dermatitis cases, and were characterized histologically by the presence of perifascicular atrophy, vacuolated fibers (VFs), and dense C5b-9 deposits on capillaries (dC5b-9). In contrast, 39 anti-TIF1-γ-Ab(-) patients with CAM were classified into various subgroups, and characterized by a higher frequency of necrotizing autoimmune myopathy (NAM). Notably, all 7 patients with CAM classified into the NAM subgroup were anti-TIF1-γ-Ab(-) and exhibited no dC5b-9 or VFs. CONCLUSIONS: CAM includes clinicohistopathologically heterogeneous disease entities. Among CAM entities, anti-TIF1-γ-Ab(+) CAM has characteristically shown a close temporal association with cancer detection and the histopathologic findings of dC5b-9 and VFs, and CAM with NAM is a subset of anti-TIF1-γ-Ab(-) CAM.

Decremental responses to repetitive nerve stimulation (RNS) in motor neuron disease.

OBJECTIVE: To clarify the features of decremental responses following repetitive nerve stimulation in patients with motor neuron diseases (MNDs), in comparison with myasthenia gravis (MG). METHODS: The subjects consisted of 48 MND, 39 generalized MG and 19 ocular MG patients. Six muscles, both proximal and distal muscles, were tested. RESULTS: Significant decrements (>5%) in at least one muscle were observed in 83% of the MND patients, and 74% and 47% of the generalized MG and ocular MG patients, respectively. Decrements were more frequently observed in the proximal muscles both in MND and MG patients (deltoid 76% and 62%, and trapezius 71% and 51% for MND and generalized MG, respectively), suggesting lower safety factors in neuromuscular transmission in those muscles. Decrements in the nasalis were rare in MND (8%) in comparison with generalized MG (54%). CONCLUSIONS: Decremental responses were frequently observed in MND patients. There were small differences between MND and MG regarding the distribution and other features of decrements, such as the degree of the U-shape or the responses to different stimulus frequencies and to brief exercise. SIGNIFICANCE: These results imply that the underlying mechanism regulating the decrements is common to MND and MG.