Huntington's disease with severe neck infection.

OBJECTIVE: To present a case of Huntington's disease (HD) with severe neck infection. BACKGROUND: Huntington's disease (HD) is a progressive neurodegenerative genetic disorder and is often accompanied by dysphagia. MATERIALS AND METHODS: The patient was a 64-year-old man with HD. The severe neck infection was considered as one of the symptoms of HD. CONCLUSION: Clinicians should remember that patients with HD are apt to delay dental consultation, resulting in serious oral infections.

Sonographic evaluation of the treatment response in patients with immunoglobulin G4-related disease of the submandibular glands.

OBJECTIVES: To evaluate the usefulness of sonography for monitoring the response to glucocorticoid treatment in patients with immunoglobulin G4 (IgG4)-related disease. METHODS: We conducted a retrospective study using sonography in 12 patients with bilateral swollen submandibular glands who had a diagnosis of IgG4-related disease based on an elevated serum IgG4 level (>135 mg/dL) and histopathologic findings between January 2010 and December 2012. Among these patients, 6 were treated with prednisolone, and the other 6 were placed under observation. B-mode sonographic examinations of the submandibular glands were performed with or without color Doppler imaging at the initial examination and 6 months later. Findings were compared between the groups (treated and untreated), and their relationship with the treatment response of the primarily involved organs was investigated. RESULTS: In the treated group, the submandibular glands of all 6 patients decreased in both size and volume after treatment (average volume ± SD, 27,449.7 ± 24,227.6 to 4609.7 ± 1911.4 mm(3); P = .004). The internal echo texture, characterized by multiple hypoechoic foci scattered against a heterogeneous hyperechoic background of submandibular tissue with demarcated hyperechoic lines, with or without hypoechoic tumor formation, disappeared or was obscured in all cases. In addition, the blood flow signals were reduced in all 3 patients who underwent color Doppler sonography, and the response observed on sonography was found to correlate with the IgG4 level and recovery of specific organ involvement. In contrast, in the untreated group, the submandibular glands showed a tendency to increase in both size and volume (average volume, 9326.3 ± 3054.8 to 12,217.4 ± 4605.5 mm(3); P= .2) without a decrease in the blood flow signals. CONCLUSIONS: Sonography is considered useful for evaluating the response to glucocorticoid therapy in patients with IgG4-related disease of the submandibular glands.

Clinical features and treatment outcomes of immunoglobulin g4-related sclerosing sialadenitis.

Idiopathic enlargement of salivary glands used to be confusing in diagnosis until immunoglobulin G4 (IgG4)-related sclerosing sialadenitis was proposed as a possible answer. In this case series, we reported the clinical features and management outcomes in 16 patients with IgG4-related sclerosing sialadenitis. We retrospectively studied 16 patients in clinical examination, serology, pathology, and sonography features. All patients were treated by corticosteroids and followed up for at least 3 months. The results of clinical features showed that all of the patients presented persistent, symmetric bilateral swelling of the salivary glands, elevated levels of serum IgG4, and/or IgG4-positive plasmacytes infiltration and tissue fibrosis. The results of all autoantibody tests were negative. The typical sonographic manifestation revealed multiple hypoechoic foci with an irregular netlike diffuse lesion in salivary glands. Most patients showed excellent response to steroids treatment. We conclude that, for patients who present (1) symmetric swelling of bilateral salivary glands for more than 3 months, (2) elevated serum IgG4 level (>135 mg/dL), and (3) enlargement in bilateral salivary glands with multiple hypoechoic areas (irregular netlike appearance) in the sonography, the diagnosis of IgG4-related sclerosing sialadenitis should be considered. A comprehensive understanding of the medical condition and appropriate pathology examination are the key to diagnose. Steroids treatment is effective, and a treatment plan should be set up and followed in the long-term.

Glycyrrhizic acid ameliorates submandibular gland oxidative stress, autophagy and vascular dysfunction in rat model of type 1 diabetes.

The burden of diabetes mellitus (DM) and associated complications is increasing worldwide, affecting many organ functionalities including submandibular glands (SMG). The present study aims to investigate the potential ameliorative effect of glycyrrhizic acid (GA) on diabetes-induced SMG damage. Experimental evaluation of GA treatment was conducted on a rat model of type I diabetes. Animals were assigned to three groups; control, diabetic and GA treated diabetic groups. After 8 weeks, the SMG was processed for assessment of oxidative stress markers, autophagy related proteins; LC3, Beclin-1 and P62, vascular regulator ET-1, aquaporins (AQPs 1.4 and 5), SIRT1 protein expressions in addition to LC3 and AQP5 mRNA expressions. Also, parenchymal structures of the SMG were examined. GA alleviated the diabetes-induced SMG damage via restoring the SMG levels of oxidative stress markers and ET-1 almost near to the normal levels most probably via regulation of SIRT1, AQPs and accordingly LC-3, P62 and Beclin-1levels. GA could be a promising candidate for the treatment of diabetes-induced SMG damage via regulating oxidative stress, autophagy and angiogenesis.

[Sphingomonas paucimobilis infection with underlying submandibular sialolithiasis]. / Submandibüler sialolitiazis zemininde Sphingomonas paucimobilis enfeksiyonu.

Sphingomonas paucimobilis (S. paucimobilis), is a gram-negative, aerobic, non-fermentative, oxidase (+) and catalase (+) bacterium. Although S. paucimobilis is isolated very rarely, it can cause both nosocomial and community-acquired infections. A patient admitted to our clinic had a complaint of swelling in the right mandibular region and pain increasing while eating for the previous week. Bimanual palpation revealed a painful swelling of 1x1x1 cm in size inside the right Wharton's duct. Via massage over the right submandibular gland, a purulent drainage came up from the opening of the Wharton's duct and it was cultivated and S. paucimobilis was isolated. There was no ultrasonographic evidence of calculi in the duct or in the gland, but during the right submandibular gland massage, two stones came out through the Wharton's duct. The infection was observed to be eradicated with a 14-day ampicillin-sulbactam treatment. In the control examination after six months, there were no pathological signs or symptoms and the ultrasonogram was normal. As far as we know, this is the first sialadenitis case with underlying sialolithiasis where S. paucimobilis was isolated.

[Alpha-1-blockers (alfuzosin) for obstructive salivary gland diseases]. / Les Alpha-1-Bloquants (alfuzosine) en pathologie salivaire obstructive.

INTRODUCTION: Alpha-blockers are used in urology to treat stenosis and lithiasis. The pathophysiology is similar in salivary glands. We had for aim to assess the safety and effectiveness of an alpha-blocker (Alfuzosin) in patients with ductal stenosis, allergic pseudo-parotitis or sialolithiasis after lithotripsy. PATIENTS AND METHODS: Three hundred and fifty-two patients were included, 194 of whom presented with sialolithiasis fragmented by extracorporeal lithotripsy (112 parotidic and 82 submandibular). Sixty-nine presented with ductal stenosis, and 89 with allergic pseudo-parotitis. This retrospective study lasted 3 years (January 2005 to January 2008) with a mean follow-up of 33 months (18 months to 4 years). Male patients were given 2.5mg tid of the alpha-blocker Alfuzosin and female patients 2.5mg bid for 3 to 24 months. After 6 months and up to 2 years of treatment, patients were assessed every 3 months by US and with a questionnaire on symptoms. RESULTS: Results were similar in male and female patients. Eighty percent of patients with colic-like pain due to stenosis reported a significant improvement after treatment. 78.6% of patients with allergic pseudo-parotitis felt they had improved and noted a sharp decrease of pruritus. Sixty-seven of the patients with residual parotid lithiasis after extracorporeal lithotripsy presented with less ductal lithiasis and fragments were evacuated more rapidly in the two months following lithotripsy. Forty-two percent of the patients treated for residual submandibular lithiasis reported a significant functional improvement and faster evacuation of fragments. Twelve patients out of 352 (3.4%) reported adverse effects. The incidence of orthostatic hypotension was 2.2%. DISCUSSION: A significant improvement of symptoms was observed in patients treated with Alfuzosin for obstructive salivary gland diseases. The drug was well tolerated. These preliminary results are good in terms of effectiveness and inocuity. They should be confirmed with a prospective controlled study.

Neonatal suppurative submandibular sialadenitis.

Neonatal sialadenitis of the submandibular gland is a very rare clinical entity. Information about the etiopathogenesis and management of the disease is very limited. Prematurity, prolonged gavage feeding and dehydration are the frequent causes. This report presents a rare case of isolated suppurative submandibular sialadenitis in a full-term newborn without any risk factors. Possible etiology, diagnosis and management of this uncommon disease are discussed.

Sarcoidosis involving the lacrimal, submandibular, and parotid glands with panda sign.

Sarcoidosis is a granulomatous inflammation of unknown origin that varies in severity and distribution. We present a case of sarcoidosis with involvement of the skin, lacrimal, parotid, and submandibular glands. The patient had been previously misdiagnosed as having angioedema and allergic contact dermatitis; he had the rare finding of panda sign on gallium scintigraphy.

[Submandibular sialadenitis in a 10 days old newborn].

We report a case of acute submandibular sialadenitis in a ten days old newborn. The disease was diagnosed at an early stage, therefore rapid antibiotic treatment was initiated, and no surgical intervention was needed. In a long term follow-up, a full recovery was observed. The authors reviewed the literature and describe the common etiologies and recommended treatment options for submandibular sialadenitis.

Coexistence of actinomycosis and sialolithiasis in the submandibular gland.

Actinomycosis of the submandibular gland is unusual. A 55-year-old male presented with a right, painless submandibular mass of four-year duration. Physical examination revealed a 4 x 3-cm mass in the submandibular region. Ultrasonography-guided fine needle aspiration from the lesion showed microorganisms compatible with actinomyces colonies in the glandular tissue. The diagnosis was made as actinomycosis of the right submandibular gland accompanied by sialolithiasis. The patient was treated with penicillin G (10 million U/day) for three months, followed by 3 g/day oral penicillin for six months. After this medication, physical examination showed no decrease in the size of the mass and the clinical features remained unchanged. Submandibular gland excision under general anesthesia was performed.

Treatment of iatrogenic submandibular sialocele with botulinum toxin. Case report.

We report the case of a 40-year-old woman with a submandibular sialocele (diagnosed by ultrasonography and magnetic resonance sialography) after sialoadenectomy for sialolithiasis. Type A botulinum toxin was injected percutaneously under colour Doppler ultrasonographic guidance into the sialocele and the residual salivary gland. Five months later the submandibular swelling had gone, and we gave a second injection of botulinum toxin to block any residual secretory activity. There were no side effects. This is, as far as we know, the first published report of the use of botulinum toxin to treat an iatrogenic submandibular sialocele.

Effect of cevimeline on radiation-induced salivary gland dysfunction and AQP5 in submandibular gland in mice.

The aim of this study was to clarify the effects of the muscarinic receptor agonist, cevimeline, on saliva flow and expression of aquaporin5 (AQP5) in submandibular gland after X-ray irradiation. Using a previously established radiation-induced xerostomia model mouse, saliva flow from at 7 days before irradiation to at 28 days after irradiation was investigated in mice that were treated with cevimeline before or after irradiation. Radiation caused a significant decrease in saliva flow compared with nonirradiated salivary glands. Cevimeline post-treatment also caused a significant decrease in saliva flow. In contrast, cevimeline pre-treatment did not significantly decrease saliva flow. Expression of AQP5 fluorescent intensity and mRNA were also analyzed. Irradiation significantly decreased expression of AQP5 in submandibular gland. However, pre-treatment with cevimeline prevented this decrease in AQP5 expression. These data suggest that pretreatment with cevimeline prevents radiation-induced xerostomia and radiation-induced decrease in expression of AQP5 in submandibular gland.

Severe life threatening maxillofacial infection in pregnancy presented as Ludwig's angina.

BACKGROUND: Ludwig's angina is a rapidly spreading cellulitis that may produce upper airway obstruction often leading to death. There is very little published information regarding this condition in the pregnant patient. CASE: A 24-year old black female was admitted at 26 weeks gestation with tooth pain, submandibular swelling, severe trismus, and dysphagea, consistent with Ludwig's angina. Her treatment included emergent tracheostomy, incision and drainage of associated spaces, teeth extraction, and antibiotic therapy. CONCLUSIONS: During a life threatening infectious situation such as the one described, risks of maternal and fetal morbidity include both septicemia and asphyxia. Furthermore, the healthcare provider must consider the risks that the condition and the possible treatments may cause the mother and her unborn child.

Neonatal suppurative submandibular sialadenitis.

Neonatal sialadenitis of the submandibular gland caused by Staphylococcus aureus is described in 2 full term newborns without any risk factors. Fifteen other newborns with isolated submandibular sialadenitis have been reported in the English language literature. Sialadenitis was caused by S. aureus in 87% and occurred primarily in premature gavage-fed neonates (87%).

[Malakoplakia of the submandibular gland in a renal transplant patient]. / Malakoplakie de la glande sous-mandibulaire chez un patient transplanté rénal.

INTRODUCTION: Malakoplakia (MP) is a rare granulomatous disease, usually occurring in immunocompromised patients, linked to Escherichia coli infection. The lesions are usually located in the genitourinary tract, but there is a great variability in the topography and the clinical presentation. CASE REPORT: A 70-year-old diabetic kidney transplant patient under immunosuppressive treatment presented with a voluminous submandibular chronic lesion, involving the skin, associated with a burgeoning lesion of the oral mucosa. Histological examination of biopsies concluded to MP and bacteriological samples were positive for E. coli. Antibiotic treatment allowed for the regression of the lesion before surgical removal. Histological examination of resected material confirmed the diagnosis of invasive MP of the submandibular gland. DISCUSSION: The diagnosis of MP relies on histological examination, showing the presence of von Hansemann's cells and Michaelis- Gutmann bodies. The treatment is based on active antibiotics targeted against intracellular bacteria, possibly associated with surgery. We report the first case of MP involving the submandibular gland.

Neonatal suppurative sialadenitis: an important clinical diagnosis.

Neonatal suppurative sialadenitis is a condition causing infection of the salivary glands, most frequently caused by Staphylococcus aureus. The vast majority of cases reported in the literature have been in infants with recognised risk factors. This report presents two cases of neonatal sialadenitis in siblings, neither of whom had any predisposing characteristics. The aetiology, diagnosis and treatment of this condition are discussed. This report will also highlight the need for awareness of this condition and its inclusion in considered differentials for neck swelling in all infants.

Acute Submandibular Swelling Complicating Arteriography With Iodide Contrast: A Case Report and Literature Review.

Iodide mumps is an uncommon condition induced by iodide-containing contrast. We present the first reported case of iodide mumps in mainland China, which occurred after carotid artery intervention.The patient, a 65-year-old Chinese male, had a history of dizziness, hypertension, diabetes, and right arm weakness. He had no history of allergies and had never previously received iodide-containing contrast. The patient's kidney function and other laboratory findings were normal. He underwent stenting of the left internal carotid artery (LICA) opening and received approximately 250 mL of a nonionic contrast agent (ioversol). Approximately 5 hours after angioplasty, bilateral local swellings were noted near the mandible; the masses were moderately firm and nontender.Iodide mumps was diagnosed in the patient. Intravenous dexamethasone (10 mg) was administered. The submandibular glands had shrunk by 11 hours after angioplasty, and they gradually became softer. The mandibular salivary glands had completely recovered by 5 days after surgery.Iodide mumps represents a rare late reaction to iodine-containing contrast media. This condition can occur in any patient receiving any iodinated contrast agent and may recur upon repeated exposure, but self-resolution can be expected within 2 weeks. All clinicians who use contrast media or iodide should be aware of this condition.

Streptococcal submandibular cellulitis in young infants.

Six infants with streptococcal submandibular cellulitis and bacteremia were managed in our institution during a seven-month period. Five uncomplicated cases were caused by group B beta-hemolytic Streptococcus, and one rapidly progressive case of Ludwig's angina was caused by group A Streptococcus. Recognition of this characteristic clinical presentation of group B streptococcal infection may be beneficial in the management of such patients.