US-guided percutaneous microwave ablation (MWA) of submandibular gland: A new minimal invasive and effective treatment for refractory sialorrhea and treatment response evaluation with contrast-enhanced imaging techniques.

A 33 years' old male complained of excessive salivation with frequent swallowing and spitting, which resulted in communication disturbance, reduced quality of life, and social embarrassment for 19 years. He had been diagnosed as sialorrhea and submandibular gland hyperfunction by stomatologist, then had unilateral submandibular gland resection 13 years ago, but the symptom relief was not satisfactory. After that, he had been treated with glycopyrrolate for less than a year, which was withdrawn because of the short duration of symptomatic control after each tablet take-in and intolerable side effects. With the wish to receive a new treatment with long term effectiveness, low re-operation risk and normal preserved saliva secretion function, the patient was subject to MWA for the right submandibular gland. After systematic clinical evaluation, US-guided percutaneous MWA was successfully performed with an uneventful post-operative course. The volume of the right submandibular gland and ablated area were measured precisely by an ablation planning software system with automatic volume measurement function based on three-dimensional reconstruction of the pre-operative and post-operative enhanced magnetic resonance imaging (MRI) raw data. Finally, the ablated volume was calculated as 62.2% of the whole right submandibular gland. The patient was discharged 1 day after the operation, with symptoms relieved significantly, the mean value of whole saliva flow rate (SFR) decreased from 11 ml to 7.5 ml per 15 minutes. During the follow up by phone three months after operation, the patient reported that the treatment effect was satisfactory, whereas the SFR value became stable as 7 ml per 15 minutes, drooling frequency and drooling severity (DFDS) score decreased from 6 to 5, drooling impact scale (DIS) score decreased from 43 to 26. US-guided percutaneous MWA of submandibular gland seems to be an alternative, minimal invasive, and effective treatment for refractory sialorrhea.We described a patient with refractory sialorrhea treated successfully with ultrasound (US) guided percutaneous microwave ablation (MWA).

Relationships of drooling with motor symptoms and dopamine transporter imaging in drug-naïve Parkinson's disease.

OBJECTIVES: The aim of the present study was to determine the relationships of drooling with motor symptoms and nigrostriatal neuron loss in drug-naïve patients with Parkinson's disease (PD). We therefore examined the relationships of drooling with motor symptoms and striatal dopamine transporter (DAT) binding measured by [123-Iodine]-fluoropropyl-2beta-carbomethoxy-3beta-(4-iodophenylnortropane) dopamine transporter single-photon emission computed tomography(123I-FP-CIT SPECT). PATIENTS AND METHODS: Thirty-five untreated PD patients (14 men and 21 women with a mean age of 71.9 ±â€¯7.2 years) were included in this study. The patients were divided into a drooler group and non-drooler group. They underwent clinical assessments and 123I-FP-CIT SPECT imaging. Motor symptoms were assessed using Unified Parkinson's Disease Rating Scale (UPDRS). RESULTS: The results showed that UPDRS motor score (p = 0.002) and akinetic-rigid score (p = 0.008) were higher and that striatal DAT availability (p = 0.03) was lower in the drooler group than in the non-drooler group. However, tremor score, age, and duration of PD showed no significant differences between the drooler group and non-drooler group. CONCLUSIONS: Drooling in untreated PD is related to an increase in motor symptoms (especially bradykinesia and axial symptoms) and to reduction of striatal DAT availability.

Neurofibromatosis 1 y cavernomatosis múltiple familiar en un lactante con sialorrea profusa episódica / Neurofibromatosis 1 and multiple familial cavernomatosis in an infant with episodic profuse sialorrhoea

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Histological and immunohistochemical findings of the action of botulinum toxin in salivary gland: systematic review / Achados histológicos e imunohistoquímicos da ação da toxina botulínica em glândula salivar: revisão sistemática

Abstract The treatment of sialorrhea is necessary for the constant risks posed by hypersalivation. A new therapeutic option comes up with the application of botulinum toxin in salivary glands. However, little is known about its mechanism of action in glandular tissue. Based on the above, this work had the objective to systematically review the literature about the action of botulinum toxin on submandibular and parotid salivary glands tissues. Electronic search was performed in databases of great relevance for this study (PubMed, SciELO, HighWire, Crossref, Scopus, Science Direct, MEDLINE, OLDMEDLINE, Serials Database, NLM Catalog, LILACS and IBECS). Inclusion and exclusion criteria for articles were established, and a total number of 14 articles were selected and used. There are few publications that clarify how the salivary gland acini behave with application of botulinum toxin. Although, the immunohistochemical findings were consistent among authors, showing weak immunoreactivity in glands treated with botulinum toxin. Histometric data are divergent, requiring more detailed studies to answer the questions about the efficacy and safety of botulinum toxin in salivary glands.

Resumo O tratamento da sialorreia se faz necessário pelos constantes riscos trazidos por este estado de hipersalivação. Uma nova opção terapêutica surge com a aplicação da toxina botulínica em glândulas salivares. Entretanto, pouco se sabe sobre o seu mecanismo de ação no tecido glandular. Com base no exposto, este trabalho teve o objetivo de revisar sistematicamente na literatura a ação da toxina botulínica sobre o tecido das glândulas salivares submandibular e parótida. Foi realizada uma busca eletrônica em bases de dados de grande relevância para este estudo (PubMed, SciELO, HighWire, Crossref, Scopus, Science Direct, MEDLINE, OLDMEDLINE, Serials Database, NLM Catalog, LILACS e IBECS). Foram estabelecidos critérios de inclusão e exclusão para os artigos, e um "n" de 14 trabalhos foram selecionados e utilizados. São poucas as publicações que esclarecem como os ácinos das glândulas salivares se comportam mediante aplicação da toxina botulínica. Apesar de os achados imunohistoquímicos entre os autores serem concordantes, com imunorreatividade mais fracas nas glândulas tratadas com a toxina botulínica, os dados histométricos são divergentes e há questionamentos metodológicos, necessitando de mais estudos pormenorizados para responder as questões sobre a eficácia e segurança da toxina botulínica nas glândulas salivares.

[Interests of the ultrasound-guidance in the treatment of drooling through botulinum toxin injections, our experience over 10 years]. / Intérêt de l'échoguidage dans le traitement du bavage par injections de toxine botulique, notre expérience sur 10 ans.

OBJECTIVES: To report about our 10 years' experience about the treatment of drooling by ultrasound guided botulinum toxin injections. MATERIAL AND METHODS: Retrospective monocentric study including all the patients suffering from drooling and treated by ultrasound-guided botulinum toxin injections into the salivary glands between 2004 and 2015. The etiology of r drooling, the doses of toxin, the injected glands, the size of the glands measured by ultrasonography, the effectiveness of the treatment and the side effects were assessed. RESULTS: Two hundred and ninety-two injections sessions were performed in 61 patients. Exactly 70.5 % of patients reported an improvement after the first session. Parkinson's disease was the main etiology of drooling (43 % of the patients). Eleven patients reported side effects. The salivary gland volume reduced after treatment in 46 % of the patients. DISCUSSION: The interest of ultrasound-guidance is to make sure about the intraglandular injection, to lower the risk for extraglandular diffusion of the toxin responsible for swallowing disorders and to allow for an adaptation of the doses to the volume of the salivary glands, which may vary during treatment.

Histological and immunohistochemical findings of the action of botulinum toxin in salivary gland: systematic review.

The treatment of sialorrhea is necessary for the constant risks posed by hypersalivation. A new therapeutic option comes up with the application of botulinum toxin in salivary glands. However, little is known about its mechanism of action in glandular tissue. Based on the above, this work had the objective to systematically review the literature about the action of botulinum toxin on submandibular and parotid salivary glands tissues. Electronic search was performed in databases of great relevance for this study (PubMed, SciELO, HighWire, Crossref, Scopus, Science Direct, MEDLINE, OLDMEDLINE, Serials Database, NLM Catalog, LILACS and IBECS). Inclusion and exclusion criteria for articles were established, and a total number of 14 articles were selected and used. There are few publications that clarify how the salivary gland acini behave with application of botulinum toxin. Although, the immunohistochemical findings were consistent among authors, showing weak immunoreactivity in glands treated with botulinum toxin. Histometric data are divergent, requiring more detailed studies to answer the questions about the efficacy and safety of botulinum toxin in salivary glands.

Análise de fatores clínico-demográficos, psicológicos, hormonais e genéticos na síndrome da ardência bucal e ardor bucal secundário / Analysis of clinical and demographic, psychological, hormonal and genetic factors in burning mouth syndrome and secondary burning mouth

A síndrome da ardência bucal (SAB) é uma condição clínica pouco esclarecida caracterizada por sensação espontânea de ardência, dor ou prurido na mucosa oral, sem alterações locais ou sistêmicas identificáveis. Sua etiopatogenia é incerta, não havendo até o momento uma padronização dos critérios utilizados para o seu diagnóstico. O presente estudo objetivou verificar a associação de fatores psicológicos, hormonais e genéticos com a SAB no sentido de propor uma melhor caracterização de sua natureza. Além de uma análise descritiva da amostra estudada, os aspectos analisados foram especificamente os níveis de estresse e sua fase, depressão, e ansiedade, compondo os fatores psicológicos; mensuração dos níveis séricos de cortisol e desidroepiandrosterona (DHEA); bem como a verificação sobre a ocorrência de polimorfismos no gene da Interleucina-6 (IL6). Foram realizadas análises comparativas entre um grupo de pacientes com SAB e um grupo composto por indivíduos com ardor bucal secundário (AB). Os resultados revelaram diferenças estatisticamente significativas entre os dois grupos com relação aos seguintes aspectos: xerostomia (p=0,01) e hipossalivação em repouso (p<0,001), que foram mais prevalentes no grupo SAB; sintomas de depressão (p=0,033), também mais presentes no grupo SAB; e dosagem de DHEA, que apresentou níveis mais reduzidos no grupo SAB (p=0,003). A dosagem desse hormônio mostrou-se amplamente sensível e específica para o diagnóstico da síndrome em estudo, sendo verificado que níveis séricos de DHEA abaixo de 0,37µg/mL para mulheres, utilizando-se os procedimentos propostos na pesquisa, possuem um Odds Ratio de 4,0 95 por cento IC (0,37 a 2,71)]. Foi verificado ainda que o alelo C do polimorfismo rs2069849 da IL-6 pode representar um alelo de risco para a ocorrência de ardor bucal em ambos os grupos, no entanto, não se pode garantir sua real implicação nos processos inflamatórios da SAB

Os presentes resultados sugerem uma provável influência da depressão, bem como de níveis diminuídos do hormônio DHEA na SAB. (AU)

The burning mouth syndrome (BMS) is a clinical condition characterized by spontaneous burning sensation, pain or itching in the oral mucosa without identifiable local or systemic changes. Its pathogenesis is uncertain, with no observable standardization in previous literature of the criteria used for its diagnosis. The present study aimed to determine demographic, psychological, hormonal and genetic factors in patients with BMS and secondary burning mouth to propose a better characterization of the nature and classification of this condition. Besides a descriptive analysis of the sample of 163 individuals, were analyzed the levels of stress and its phase, depression and anxiety; measurement of serum levels of cortisol and dehydroepiandrosterone (DHEA), as well as checking on the occurrence of polymorphisms in the gene of interleukin-6 (IL6). Comparative analysis between a group of patients with BMS and a group of individuals with secondary burning mouth (BM) were performed. The results revealed statistically significant differences between the two groups with respect to the following aspects: xerostomia (p=0.01) and hyposalivation at rest (p<0.001), which were more prevalent in the BMS group; symptoms of depression (p=0.033), more present in the BMS group, and dosage of DHEA, which showed lower levels in BMS patients (p=0.003). The dosage of this hormone was largely specific and sensitive for the diagnosis of the studied syndrome, and was verified that serum levels of DHEA below 0.37 pg/mL in women, using the procedures proposed in this research, have an Odds Ratio of 4.0 95 per cent Cl (0.37 to 2.71)]. These results suggest a possible influence of depression and decreased levels of the hormone DHEA in the pathogenesis of BMS. (AU)

Tratamiento de la sialorrea en niños con patología neurológica / Drooling therapy in children with neurological disorders

Introducción. La sialorrea es la incapacidad para retener la saliva dentro de la boca y su progresión al tracto digestivo, y es un problema frecuente en pacientes pediátricos con patología neurológica, por lo que se están utilizando diferentes medidas para su tratamiento. Objetivo. Evaluar la eficacia y seguridad del trihexifenidilo, la escopolamina y la infiltración de toxina botulínica en el tratamiento del babeo en niños con patología neurológica. Pacientes y métodos. Es un estudio de tipo abierto y prospectivo. Incluye pacientes atendidos en el servicio de neurología que presentaban babeo excesivo, con repercusión en su calidad de vida, entre 2009 y 2013. Resultados. En 46 pacientes se indicó tratamiento con trihexifenidilo oral, y se obtuvo buena respuesta en 15 (32,6%), tres con efecto transitorio y el resto mantenido. Presentaron efectos secundarios tres pacientes (6,5%). De los 11 pacientes a los que se indicaron parches de escopolamina, se halló efecto beneficioso en cuatro (36,36%), uno fue retirado por falta de eficacia y seis por efectos secundarios. Veinticinco pacientes fueron infiltrados con toxina botulínica, con disminución significativa del babeo en 16 (64%) tras la primera infiltración. No observamos cambios significativos en nueve casos. Sólo uno presentó efectos secundarios (disfagia leve). Conclusiones. Por no haber una opción terapéutica totalmente eficaz para los pacientes con sialorrea, recomendamos iniciar el tratamiento con trihexifenidilo; como segunda opción, los parches de escopolamina, y como tercera opción, la toxina botulínica. La infiltración de toxina botulínica en glándulas salivales se muestra como una alternativa eficaz y segura según nuestra serie (AU)

Introduction. Drooling is the inability to retain saliva in the mouth and its progression to the digestive tract, being a common problem in pediatric patients with neurological disorders. Three different treatment options are available. Aim. To assess the effectiveness and safety of trihexyphenidyl, scopolamine and botulinum toxin infiltration in the treatment of drooling in children with neurological disorders. Patients and methods. This is an open and prospective type study. We include patients treated in the Neurology Service that present excessive drooling, affecting their quality of life, between 2009 and 2013. Results. We enrolled 46 patients in the study. The treatment with oral trihexyphenidyl was indicated in 46, obtaining good result in 15 (32.6%), three with temporary effect and the rest with lasting effect. Three patients presented side effects (6.5%). Four out of 11 (36.36%) patients treated with scopolamine patch had beneficial effects. One was withdrawn due to lack of efficacy and six due to side effects. Twenty-five patients were infiltrated with botulinum toxin, with a significant decrease of drooling in 16 patients (64%) after the first injection. We observed no significant changes in nine patients. Only one out of 25 showed side effects (mild dysphagia). Conclusions. Currently there is not a fully effective therapeutic option for drooling. We recommend starting treatment with trihexyphenidyl. A second option could be the scopolamine patch and botulinum toxin as a third option. Botulinum toxin infiltration in salivary glands is shown as an effective and safe alternative in our study (AU)

Botulinum toxin for salivary disorders in the treatment of head and neck cancer.

BACKGROUND/AIM: During the treatment of head and neck cancer (HNC), salivary problems may impair a patient's healing process. Botulinum toxin (BoNT) is accepted as an effective treatment option for reducing salivary flow. We aimed to describe the features of patients treated with BoNT to determine the effects of BoNT. PATIENTS AND METHODS: Twenty-five patients over a five-year period were retrospectively included. The patients suffered at different stages of oncologic treatment. The cohort primarily had larger primary tumors that required complex oncological treatment. RESULTS: The condition improved in more than three quarters of the 19 patients with functional hypersalivation. Four of six cases suffering from a salivary fistula demonstrated an obvious reduction in symptoms. CONCLUSIONS: Injection of BoNT, to temporarily reduce saliva flow, is a safe tool in the treatment of HNC even in situations involving repeated therapy or high dosage. The main clinical side-effect of BoNT is insufficient reduction of the salivary problem.

Evaluation of ethanolamine oleate sclerotherapy on the submandibular glands of canines as a potential therapy for sialorrhea.

BACKGROUND: Sialorrhea can have major negative effects on the physical and social well-being. Sclerotherapy may be useful in patients with sialorrhea by decreasing the amount of saliva production. The aim of this study was to test the effect of ethanolamine oleate (EO) in an experimental model as a preliminary step for its application in humans. METHODS: Histopathological and morphometric analysis of submandibular glands from thirteen dogs was preformed. A total of 25 glands were injected with 1ml of 2.5% EO (n=5), 1ml of 5% EO (n=5), 5ml of 2.5% EO (n=5) and 5ml of 5% EO (n=5). Five glands were used as control. RESULTS: EO significantly induced a dose dependent scaring of the gland ending in lobular transformation (salivary gland cirrhosis). Morphometric measurements showed that 1ml of 2.5% or 5% EO significantly induced fibrosis compared to normal glands (p=0.014 and 0.021, respectively). Fibrosis significantly increased and was more apparent when a dose of 5ml of 2.5% EO or 5% EO were injected [by semi-quantitative evaluation (p=0.016 and 0.002, respectively) and morphometric measurements (p=0.016 and 0.008, respectively)]. This scarring effect was significantly associated with reduction of area of acinar cells when a dose of 1ml-5%, 5ml-2.5% or 5ml-5% EO were applied (p=0.03. 0.012 and 0.004, respectively). Moreover, ductal injury was only significant when a dose of 5ml of 5% EO was used (p=0.034). This dose and concentration (i.e. 5ml-5% EO) had a significant synergetic effect [p=0.0119]. CONCLUSION: In this model, treatment with EO proved to permanently reduce the acinar area through induction of progressive, irreversible and dose dependant scarring (medical sialoadenectomy).

[Sensory neuronopathy of Sjögren's syndrome. A diagnostic challenge]. / Sensible Neuronopathie bei Sjögren-Syndrom. Eine diagnostische Herausforderung.

Sjögren's syndrome is an important differential diagnosis in patients with sensory neuronopathy because immunosuppressive therapy may prevent progressive degeneration of sensory fibres, ganglions and axons. Due to the challenges in the diagnostic process the diagnostic criteria have repeatedly changed over the past few years. In patients with negative antibodies (SSA, SSB antibodies) biopsy of the salivary glands of the lip and the parotid gland can be useful to diagnose Sjögren's syndrome. We report on four patients in whom biopsy of the salivary gland was helpful in establishing the diagnosis of Sjögren's syndrome and consequently immunosuppressive therapy was initiated. One of these patients suffered from hypersalivation. This was probably due to denervation hypersensitivity. To our knowledge this has not been reported yet.

Surgical treatment of macroglossia in patients with Beckwith-Wiedemann syndrome: a 20-year experience and review of the literature.

Macroglossia is observed in the majority of paediatric patients diagnosed with Beckwith-Wiedemann syndrome and surgical treatment may be indicated. A 20-year retrospective study was performed to evaluate all patients with Beckwith-Wiedemann syndrome who underwent tongue reduction surgery at the authors' institution. A literature review was performed. Surgical treatment was indicated and carried out in 23 patients with a yearly average varying from 0 to 3 subjects. The mean follow-up time was 7 years. Primary indications for surgical treatment of macroglossia included significant tongue protrusion in 30% (n=7), and a combination of clinical problems in 70% (n=16). In all cases, the procedure was performed by the same surgeon using an anterior, V-shaped, wedge resection technique. Two patients had direct postoperative complications. No recurrence of macroglossia was observed in this study. Reported clinical outcome during follow-up demonstrated a satisfactory resting tongue position in all patients. Review of the literature demonstrated variability in surgical indications and techniques, and postoperative outcome. The results of this study indicate that the anterior wedge resection is a simple, effective and safe technique in the surgical treatment of paediatric patients, diagnosed with Beckwith-Wiedemann syndrome, suffering from macroglossia.

Early-onset form of benign childhood epilepsy with centro-temporal EEG foci--a different nosological perspective from Panayiotopoulos syndrome.

PURPOSE: We have studied the clinical differences between early-onset benign epilepsy with centro-temporal spikes (early-onset BECT) and Panayiotopoulos syndrome (PS) to investigate the hypothesis that BECT and PS nosologically constitute age-dependent benign childhood seizure susceptibility syndromes based on a common etiopathogenesis. SUBJECTS AND METHODS: The subjects were 24 patients with BECT and 62 patients with PS, who satisfied the following definitions: 1) onset of epilepsy before 5 years of age; 2) the BECT and PS seizures started mainly with orofacial focal motor attacks and emetic symptoms followed by focal seizures, respectively; 3) follow-up examinations for longer than 2 years. We compared the various clinical features between these two groups. RESULTS: In children with early-onset BECT, the seizures at times manifested with hypersalivation, vomiting, and focal motor seizures, but the vomiting that developed in the middle of seizures was different from the initial vomiting observed in patients with PS. Although the seizures recurred more frequently in patients with early-onset BECT, the incidence of status epilepticus as well as prolonged seizures was higher in those with PS. The patients demonstrating below borderline IQ scores and mild developmental behavioral disorders were more frequently seen in early-onset BECT than PS, accounting for 37.5 and 14.6% (P<0.05), and for 8% and 21%, respectively (P<0.05). DISCUSSION: Early-onset BECT and PS have heterogeneous clinical characteristics, except for the same onset age, and appear to be nosologically different epileptic syndromes. The former seems to develop in combination with other acquired disturbances based on a BECT predisposition, while the latter develops based on a PS predisposition and involves a better prognosis.

Dietary L-tyrosine supplementation in nemaline myopathy.

Nemaline myopathy is defined by the presence of nemaline bodies, or rods, on muscle biopsy. Facial and bulbar weakness in nemaline myopathy cause chewing and swallowing difficulties, recurrent aspiration, and poor control of oral secretions. This article discusses 5 patients (4 infants and 1 adolescent) with nemaline myopathy who received dietary supplementation with L-tyrosine (250 to 3000 mg/day). All 4 infants were reported to have an initial decrease in sialorrhoea and an increase in energy levels. The adolescent showed improved strength and exercise tolerance. No adverse effects of treatment were observed. Dietary tyrosine supplementation may improve bulbar function, activity levels, and exercise tolerance in nemaline myopathy.

Successful treatment of drooling in children with neurological disorders with botulinum toxin A or B.

Drooling is a common and severe problem in children with neurological disorders and is caused by a disturbed coordination of orofacial and palatolingual muscles. Botulinum toxin could be a successful option to reduce excessive sialorrhea in children with neurological disorders. In 30 children with cerebral palsy or neurodegenerative disorder we injected under ultrasound guidance either botulinum toxin A or botulinum toxin B into the parotid and submandibular glands on both sides. All injections were well tolerated without general anaesthesia. Drooling severity at baseline and reduction of sialorrhea during treatment was measured using a parent's questionnaire and rated using the Teachers Drooling Scale (TDS). Reduction of sialorrhea was achieved two weeks after injection, with a positive effect lasting about three to four months in most children. 83% showed a good response to botulinum toxin after first injection, but only in 50% treatment was continued. We found no significant differences between botulinum toxin A or B. Side effects were observed in 5 children with viscous saliva and in one child a unilateral parotitis was observed. Treatment of drooling with botulinum toxin into the salivary glands is a safe and easy therapeutic option for children with neurological disorders to improve life quality.

Scintigraphic analysis of the parotid glands in patients with sialorrhea and Parkinson's disease.

Since sialorrhea was initially described, it has been associated with Parkinson's disease (PD) but until now little is known about its pathophysiology. The authors studied parotid gland activity using scintigraphic analysis on 14 PD patients with sialorrhea and in eight healthy persons with matching ages. There was no difference between uptake and intra-glandular distribution by the parotid gland in the two groups but the parotid excretion speed in the PD patients was greater than that observed in healthy individuals. Our results reject the hypothesis of PD productive sialorrhea and point to retention sialorrhea due to the increase of saliva excretion velocity.

[Sialorrhea--causes and treatment options]. / Sialorrhö. Ursachen und Behandlungsmöglichkeiten.

Sialorrhea (drooling) is the involuntary spillage of saliva from the mouth. Drooling of saliva appears to be the consequence of a dysfunction in the coordination of the swallowing mechanism, resulting in excess pooling of saliva in the anterior portion of the oral cavity and the unintentional loss of saliva from the mouth. There are specific and symptomatic approaches to manage this condition. Treatment options range from conservative (i.e., observation, postural changes, biofeedback, motoric therapy of the mouth, acupuncture) to more aggressive measures such as medication, radiation, and surgical therapy.