Precision treatment of post pneumonectomy unilateral laryngeal paralysis due to cancer.

Aim: The aim of this study is to assess the efficacy of external laryngeal medialization acquired through a Gore-Tex implant in a 45 patients affected by unilateral vocal fold paralysis in abduction after pneumonectomy. Method: The cohort of patients was made up of 30 male (73.1%) and 11 female patients (26.9%) with an average age of 66.7 years in a range between 46 and 78 years. Results: The results were analyzed with the objective and subjective analysis of voicing and swallowing. In 95.2% of cases, voice and swallow improvement with statistically significant evidences. Conclusion: We can conclude that Gore-Tex implantation is a simple, reproducible and minimally invasive procedure for management of selected cases of vocal fold unilateral paralysis in the abductory position.

Management of dysphagia in multiple sclerosis: current best practice.

INTRODUCTION: Multiple sclerosis (MS) is characterized by extreme variability in both severity and clinical course. It can show severe disabling symptoms, and among them dysphagia is frequently described. However, its management still represents a challenge in the daily care. Areas covered: In this review, we will focus on the clinical recognition and therapeutic strategies to identify and manage dysphagia in people with MS. In the view of a personalized approach, different interventions should be tailored to every single patient. Expert commentary: Multidisciplinary evaluation is mandatory in MS management, and dysphagia represents a perfect model of taking care of a disabling symptom in a chronic disease. Further research is required to better organize a personalized and long-term management of dysphagia phenomenon, through the different subtypes of MS.

Multi-disciplinary clinical protocol for the diagnosis of bulbar amyotrophic lateral sclerosis / Protocolo clínico multi-disciplinar para el diagnóstico de la esclerosis lateral amiotrófica bulbar

Introduction and objectives: The objective of this study was to examine the role of different specialists in the diagnosis of amyotrophic lateral sclerosis (ALS), to understand changes in verbal expression and phonation, respiratory dynamics and swallowing that occurred rapidly over a short period of time. Materials and methods: 22 patients with bulbar ALS were submitted for voice assessment, ENT evaluation, Multi-Dimensional Voice Program (MDVP), spectrogram, electroglottography, fiberoptic endoscopic evaluation of swallowing. Results: In the early stage of the disease, the oral tract and velopharyngeal port were involved. Three months after the initial symptoms, most of the patients presented hoarseness, breathy voice, dysarthria, pitch modulation problems and difficulties in pronunciation of explosive, velar and lingual consonants. Values of MDVP were altered. Spectrogram showed an additional formant, due to nasal resonance. Electroglottography showed periodic oscillation of the vocal folds only during short vocal cycle. Swallowing was characterized by weakness and incoordination of oro-pharyngeal muscles with penetration or aspiration. Conclusions: A specific multidisciplinary clinical protocol was designed to report vocal parameters and swallowing disorders that changed more quickly in bulbar ALS patients. Furthermore, the patients were stratified according to involvement of pharyngeal structures, and severity index

Introducción y objetivos: El objetivo de este estudio fue examinar la función de los diferentes especialistas en el diagnóstico de la esclerosis lateral amiotrófica (ELA), para comprender qué cambios se produjeron en cuanto a expresión verbal, dinámica fonatoria y respiratoria, y deglución, en un breve periodo de tiempo. Materiales y métodos: Se sometió a 22 pacientes con ELA bulbar a evaluación de la voz, examen ORL, programa de voz multi-dimensional (MDVP), espectrograma, electroglotografía y evaluación de la deglución mediante endoscopía de fibra óptica. Resultados: En la etapa temprana de la enfermedad, el tracto oral y el cierre velofaríngeo se vieron comprometidos. Transcurridos 3 meses de los síntomas iniciales, la mayoría de los pacientes presentó ronquera, voz entrecortada, disartria, problemas de modulación de la afinación y dificultades de pronunciación de las consonantes oclusivas, velares y linguales. Los valores del MDVP se vieron alterados. El espectrograma reflejó un formante adicional, debido a la resonancia nasal. La electroglotografía mostró una oscilación periódica de las cuerdas vocales únicamente durante el ciclo vocal corto. La deglución se vio caracterizada por debilidad y descoordinación de los músculos orofaríngeos, con penetración o aspiración. Conclusiones: Se diseñó un protocolo clínico multidisciplinar específico para reportar los parámetros vocales y los trastornos deglutorios que experimentan un cambio más rápido en los pacientes con ELA bulbar. Además, se estratificaron los pacientes con arreglo al compromiso de las estructuras faríngeas y el índice de gravedad

Multi-disciplinary clinical protocol for the diagnosis of bulbar amyotrophic lateral sclerosis.

INTRODUCTION AND OBJECTIVES: The objective of this study was to examine the role of different specialists in the diagnosis of amyotrophic lateral sclerosis (ALS), to understand changes in verbal expression and phonation, respiratory dynamics and swallowing that occurred rapidly over a short period of time. MATERIALS AND METHODS: 22 patients with bulbar ALS were submitted for voice assessment, ENT evaluation, Multi-Dimensional Voice Program (MDVP), spectrogram, electroglottography, fiberoptic endoscopic evaluation of swallowing. RESULTS: In the early stage of the disease, the oral tract and velopharyngeal port were involved. Three months after the initial symptoms, most of the patients presented hoarseness, breathy voice, dysarthria, pitch modulation problems and difficulties in pronunciation of explosive, velar and lingual consonants. Values of MDVP were altered. Spectrogram showed an additional formant, due to nasal resonance. Electroglottography showed periodic oscillation of the vocal folds only during short vocal cycle. Swallowing was characterized by weakness and incoordination of oro-pharyngeal muscles with penetration or aspiration. CONCLUSIONS: A specific multidisciplinary clinical protocol was designed to report vocal parameters and swallowing disorders that changed more quickly in bulbar ALS patients. Furthermore, the patients were stratified according to involvement of pharyngeal structures, and severity index.

Multicenter research into the quality of life of patients with advanced oropharyngeal carcinoma with long-term survival associated with human papilloma virus.

The treatment of advanced-stage oropharyngeal squamous cell carcinoma may utilize various modes, including combining surgery with chemoradiotherapy (CTRT), or primary CTRT followed by rescue surgery. In previous literature it has been revealed how patients treated with combined modes report a low quality of life (QoL) and severe consequences following surgery, radiotherapy and chemotherapy, in the short and in the long-term. The decrease in the QoL of patients treated with high-intensity multi-modal strategies highlights the necessity of modifying treatments, particularly for young HPV-positive patients, where an increased survival rate has already been reported. The modified treatment for HPV-positive tumors in the tonsils and at the base of the tongue is based on the deintensification of therapies aiming to reduce toxicity and thereby improve QoL in the long term, whilst still maintaining therapeutic effectiveness. The aim of the present study was to evaluate the QoL in patients with a long-term survival, who were treated with combined therapy for squamous cell tumors in the tonsils and at the base of the tongue, and to compare the results observed in HPV-positive and HPV-negative patients. According to statistical analysis, differences in the general QoL and in the single scales of the European Organization for the Research and Treatment of Cancer questionnaires were not correlated with the type of therapy selected for the particular patient. QoL considered the presence of HPV, the type of treatment, the subregion of the tonsils vs. the base of the tongue and the disease stage at the time of diagnosis, and was determined to be non-influential with regard to these specific variables.

Expression pattern of estroprogestinic receptors in sinonasal inverted papilloma.

Inverted papilloma (IP) is a locally destructive, benign neoplasm of the nose and paranasal sinuses with a high tendency for recurrence, a significant potential for malignancy, and an etiology that today is still uncertain. The expression of hormonal receptors in neoplastic tissues has been the focus of intensive research for its potential diagnostic, prognostic, and therapeutic significance. The aim of this study was to assess the potential estroprogestinic receptor expression in patients undergoing sinus surgery for IP. A retrospective study was carried out, on surgical specimens of 73 patients who underwent endoscopic sinus surgery for first manifestation of sinonasal IP (primitive IP group) and in 21 subjects who had developed a recurrence (relapsed IP group). The results of the immunohistochemical analysis of the first group showed the absence of receptor expression for PGR in all cases analyzed and the presence of a low positivity for ER in 11 cases (P > 0.082). Similarly, in the second group the results showed a low presence of ER receptors in 3 of the 21 cases (P > 0.068), while there was no evidence of PGR receptors in the examined samples. In addition, in 11 of the cases only 3 were considered positive (27.2%) showing a recurrence during follow-up (P > 0.068).Our results suggest that the sinonasal IP is a benign tumor independent of estrogen and progesterone, and the receptors for these hormones are therefore unsuitable as predictors of relapse or possible prognostic indicators and therapeutic targets.

The watch-pat in pediatrics sleep disordered breathing: Pilot study on children with negative nocturnal pulse oximetry.

OBJECTIVES: The main purpose of this study was to determine the efficacy of Watch-PAT in Pediatric Sleep Disordered Breathing (PSDB) diagnosis in children with symptoms suggestive of PSDB, in which the nocturnal pulse oximetry was negative according to the Brouilette criteria. METHODS: We enrolled 28 patients aged between 5 and 12 years (mean age: 7.75 ± 1.69), who underwent the registration with Watch-PAT, that utilizes the Peripheral Arterial Tone (PAT), AHI, RDI, body position, snoring, pulse oximetry and actigraphy. RESULTS: Recording Watch-PAT was indicative of PSDB in 10/28 (35.7%) patients; when it was placed the threshold of AHI > 1 the number of positive patients for PSDB increased to 17/28 (60.7%). Exists a positive correlation between pat-RDI (rho = 0.798, p = 0.005) and the snoring > 40% of the time (rho = 0.656, p < 0.001) were correlated with the pat-AHI values. CONCLUSION: The recording Watch-PAT appears to permit the defection of a certain number of SDB that might escape to the clinical evaluation and pulse oximetry only.

Autoimmune Ear Disease: Clinical and Diagnostic Relevance in Cogan's Sydrome.

The autoimmune inner ear disease is a clinical syndrome with uncertain pathogenesis that is often associated to rapidly progressive hearing loss that, especially at the early stages of disease, may be at monoaural localization, although more often it is at binaural localization. It usually occurs as a sudden deafness, or a rapidly progressive sensorineural hearing loss. In this study a particular form of autoimmune inner ear disease is described, Cogan's syndrome. Cogan's syndrome is a chronic inflammatory disorder that most commonly affects young adults. Clinical hallmarks are interstitial keratitis, vestibular and auditory dysfunction. Associations between Cogan's syndrome and systemic vasculitis, as well as aortitis, also exist. We report a case of a young woman who presented audiological and systemic characteristics attributable to Cogan's syndrome. In the description of the case we illustrate how the appearance and evolution of the disease presented.

Therapeutic approaches to pediatric pseudotumor cerebri: New insights from literature data.

The pseudotumor cerebri syndrome (PTCS), also known as idiopathic intracranial hypertension (IIH), is characterized by signs and symptoms of increased intracranial pressure such as headache and cranial nerve palsies, in the absence of any space-occupying mass. This condition commonly affects overweight women of childbearing age, even if it is also frequent in men and children. Children with PTCS may present with atypical signs and symptoms, with a different prognosis compared to adults. However, the treatment is the same for both children and adults, even if there are no strict treatment guidelines in regards. All treatment strategies in children are based on retrospective studies and none has been evaluated in prospective or randomized controlled trial studies. This review focuses on literature data on PTCS treatment in children.

The senile functional evolution of the larynx after supracricoid reconstructive surgery.

Supracricoid partial laryngectomy is a relevant organ-preserving surgical technique used for the treatment of early or locally advanced laryngeal tumors with an oncologic outcome comparable with that of total laryngectomy. This reconstructive surgery is certainly technically feasible also in the elderly, but the problem is postoperatively as the patient may lack the will and strength to be successfully rehabilitated. The goal was to evaluate functional outcomes, in a maximum follow-up period of ten years from the end of the postoperative follow-up, in a cohort of elderly patients to look at the senile evolution of the laryngeal post surgical function comparing the amount of resection and grade of possible impairment of swallowing and phonatory parameters. A group of 33 patients were selected for a retrospective study aimed at evaluating the long-term evolution of laryngeal functionality after surgery, with the baseline at 5 years from the end of follow-up. All the patients had three-monthly visits for a period up to ten years, during which the objective and subjective swallowing and phonatory parameters were recorded, analyzed and, at the end of the study, compared with baseline. The results suggest that long-term post surgical functional problems may develop also in the neolarynx where the effects of the reconstructive surgery, on speech and swallowing, are largely stabilized. The presence of arytenoid resection had significantly impacted on the occurrence of aspiration even if the overall number of dysphagic patients was not statistically significant compared to baseline.

Assessment of oxidative stress markers and hearing thresholds in patients with obstructive sleep apnea-hypopnoea treated with cysteine and superoxide dismutase therapy.

BACKGROUND AND AIM OF THE WORK: In OSAHS, the hypoxia and reoxygenation cicles, maintain a state of oxidative stress, which seems to cause a change in the oxidative balance. Our aim is to compare the markers of oxidative stress with audiological findings and OSAHS severity, in OSAHS patients untreated and also treated ones, with cysteine and superoxide dismutase. METHODS: 65 patients (42 Men, 23 Women) with 30-65 years age range have been enrolled, with a mean age of 52.6 ± 13.3 years with moderate OSAHS. We have analyzed plasma and lymphocyte markers of oxidative stress (glutathione, thioredoxin and heat shock protein) and they were underwent tonal audiometry. Patients were divided in two groups: Group A (32 patients) included patients treated for 8 weeks with cysteine and superoxide dismutase; Group B (33 patients) included patients untreated. RESULTS: The research showed a significant increase in reduced glutathione levels (p<0.05) in OSAHS patients treated; conversely, it showed a decrease of oxidized glutathione level (p<0.05) in treated patients than OSAHS untreated ones. The thioredoxin values, in untreated OSAHS patients, appear to be reduced than in OSAHS patients treated (p<0.05), and that the heat shock protein values were more elevated in untreated OSAHS patients (p<0.05). Finally, it was found that a correlation exists between the severity of OSAHS and auditory dysfunction. CONCLUSIONS: The study of the oxidative stress markers has produced results which lead to support the idea that, in a personalized therapy context, the use of antioxidant therapy can cooperate effectively the first choice treatment.

Heat shock proteins and hormesis in the diagnosis and treatment of neurodegenerative diseases.

Modulation of endogenous cellular defense mechanisms via the vitagene system represents an innovative approach to therapeutic intervention in diseases causing chronic tissue damage, such as in neurodegeneration. The possibility of high-throughoutput screening using proteomic techniques, particularly redox proteomics, provide more comprehensive overview of the interaction of proteins, as well as the interplay among processes involved in neuroprotection. Here by introducing the hormetic dose response concept, the mechanistic foundations and applications to the field of neuroprotection, we discuss the emerging role of heat shock protein as prominent member of vitagene network in neuroprotection and redox proteomics as a tool for investigating redox modulation of stress responsive vitagenes. Hormetic mechanisms are reviewed as possibility of targeted therapeutic manipulation in a cell-, tissue- and/or pathway-specific manner at appropriate points in the neurodegenerative disease process.

A Selective Mutism Arising from First Language Attrition, Successfully Treated with Paroxetine-CBT Combination Treatment.

After immersion in a foreign language, speakers often have difficulty retrieving native-language words and may experience a decrease in its proficiency, this phenomenon, in the non-pathological form, is known as first language attrition. Self-perception of this low native-language proficiency and apprehension occurring when speaking is expected and, may sometimes lead these people to a state of social anxiety and, in extreme forms, can involve the withholding of speech as a primitive tool for self-protection, linking them to selective mutism. We report an unusual case of selective mutism arising from first language attrition in an Italian girl after attending a two-year "German language school", who successfully responded to a paroxetine-cognitive behavioral treatment (CBT) combination treatment.

The usefulness of immunotherapy in pediatric neurodegenerative disorders: A systematic review of literature data.

Immunotherapeutic strategies to treat neurodegenerative disorders have inspired the scientific community. The aim of our review is to address the translational aspects of neuroimmunology to describe the efficacy of immunotherapy in the treatment of pediatric neurodegenerative disorders. In the studies we analyzed IVIG were found to be efficient in the treatment of post-streptococcal neurodegenerative disorders, even if in PANDAS, plasma-exchange (PE) showed a higher efficiency. IVIG were also successfully used in ADEM and Guillan-Barré syndrome. In Sydenham Chorea the use of methylprednisolone was found in most cases as efficient as IVIG, while in Tourette's Syndrome, Colecoxib was successfully used in one patient. Pediatric Multiple Sclerosis seems to respond better to immunosuppressant agents (Mitoxantrone, Cyclophosphamide, Natalizumab), as well as Neuromyelitis optica (Rituximab, Mycofenolate). The importance of this review relies in the attempt to draw standardized guidelines for immunotherapy in pediatric neurodegeneratve disorders.

Epilepsy and innate immune system: A possible immunogenic predisposition and related therapeutic implications.

Recent experimental studies and pathological analyses of patient brain tissue samples with refractory epilepsy suggest that inflammatory processes and neuroinflammation plays a key-role in the etiopathology of epilepsy and convulsive disorders. These inflammatory processes lead to the secretion of pro-inflammatory cytokines responsible for blood-brain-barrier disruption and involvement of resident immune cells in the inflammation pathway, occurring within the Central Nervous System (CNS). These elements are produced through activation of Toll-Like Receptors (TLRs) by exogenous and endogenous ligands thereby increasing expression of cytokines and co-stimulatory molecules through the activation of TLRs 2, 3, 4, and 9 as reported in murine studies.It has been demonstrated that IL-1ß intracellular signaling and cascade is able to alter the neuronal excitability without cell loss. The activation of the IL-1ß/ IL-1ß R axis is strictly linked to the secretion of the intracellular protein MyD88, which interacts with other cell surface receptors, such as TLR4 during pathogenic recognition. Furthermore, TLR-signaling pathways are able to recognize molecules released from damaged tissues, such as damage-associated molecular patterns/proteins (DAMPs). Among these molecules, High-mobility group box-1 (HMGB1) is a component of chromatin that is passively released from necrotic cells and actively released by cells that are subject to profound stress. Moreover, recent studies have described models of epilepsy induced by the administration of bicuculline and kainic acid that highlight the nature of HMGB1-TLR4 interactions, their intracellular signaling pathway as well as their role in ictiogenesis and epileptic recurrence.The aim of our review is to focus on different branches of innate immunity and their role in epilepsy, emphasizing the role of immune related molecules in epileptogenesis and highlighting the research implications for novel therapeutic strategies.

Relationship between radiotherapy and gastroesophageal reflux disease in causing tracheoesophageal voice rehabilitation failure.

OBJECTIVE: The objective was to analyze the association of radiotherapy with gastroesophageal reflux as determinant of fistula related pathology, in voice prosthesis patients. STUDY DESIGN: Retrospective study. METHODS: Sixty-one laryngectomy patients were enrolled between 2005 and 2012. All patients underwent phonatory rehabilitation with voice prosthesis, along with evidence of gastroesophageal reflux disease, for which proton pump inhibitors (PPIs) were prescribed. We analyzed the occurrence of fistula-related problems among patients who received postoperative radiotherapy and those patients who did not. RESULTS: We observed a higher rate of failure of speech rehabilitation in laryngectomy patients with gastroesphageal reflux: this occurred when they had a history of postoperative radiotherapy (45%) compared with patients who did not (17%) (P < 0.05), although all patients were treated with PPIs. CONCLUSION: Our results seem to confirm the importance of postoperative radiotherapy with gastroesophageal reflux for the determinism of fistula-related problems.

Mucosal leishmaniasis: an underestimated presentation of a neglected disease.

We present a review of current knowledge about mucosal leishmaniasis (ML). Although involvement of mucous membranes is classically admitted in New World leishmaniasis, particularly occurring in infection by Leishmania (L.) braziliensis species complex, ML is also a possible presentation of Old World leishmaniasis, in either L. donovani or L. major species complex infections. Thus, ML has to be considered not only as a Latin American disease but as an Old and New World disease. We describe ML epidemiology, pathogenesis, clinics, diagnosis, and therapy. Considering both its highly disfiguring lesions and its possible lethal outcome, ML should not be underestimated by physicians. Moreover, leishmaniasis is expected to increase its burden in many countries as sandfly vector distribution is widespreading towards non-endemic areas. Finally, the lack of clear understanding of ML pathogenesis and the absence of effective human vaccines strongly claim for more research.

Isolated laryngeal leishmaniasis in immunocompetent patients: an underdiagnosed disease.

We describe a case of isolated primary laryngeal leishmaniasis in an immunocompetent Italian patient with a previous medical history negative for visceral or cutaneous leishmaniasis, presenting with hoarseness. We also summarize the epidemiological, clinical, and diagnostic features and the therapeutic management of other cases of laryngeal leishmaniasis in immunocompetent subjects, described in the literature. Considering the insidious and nonspecific clinical presentation, the increasing number of different forms of mild or underestimated immunosuppressive conditions, and the number of people travelling in endemic zones, along with the ability of Leishmania amastigotes to survive for a long period in the body, we believe it is important for pathologists and clinicians to be aware of this unusual form of leishmaniasis in order to avoid delayed recognition and treatment. The rarity of the presentation and the lack of guidelines on mucosal leishmaniasis may contribute to the potential undiagnosed cases or delayed diagnosis, the possible relapses, as well as the correct pharmacological and/or surgical therapeutic approach.