Phonoarticulation in spinocerebellar ataxia type 3.

Phonoarticulation is characterized by changes in resonance, diadochokinesis, prosody, sound frequency, vocal quality, and intraoral pressure. The main aim of this study was to characterize the phonoarticulation in spinocerebellar ataxia type 3 (SCA3) and correlate it with clinical and genetic factors. Thirty-one patients with SCA3 who were subjected to spontaneous speech recordings and phonoarticulatory diadochokinesis (DDK) participated in the study. Speech analyses were performed starting after 10 s of spontaneous speech, by three experienced speech therapists, using a protocol for dysarthria adapted from the Mayo Clinic. The intra-evaluator reliability was analyzed. The lower the patient's age at disease onset was, the more frequent the occurrences of monofrequency and altered speech rhythm were. Articulation, DDK, resonance, and prosody showed a moderate correlation with the number of "CAG" triplet repeats. We conclude that the phonoarticulation of patients with Machado-Joseph disease (MJD) is characterized by mixed dysarthrophonia with cerebellar and hypokinetic components, and that there is a tendency toward higher frequency of dysarthrophonia symptoms with lower age of disease onset, longer time since onset and higher number of "CAG" triplet repeats.

Rapid maxillary expansion and obstructive sleep apnea: A review and meta-analysis.

BACKGROUND: OSAS during childhood leads to significant physical and neuropsychomotor impairment. Thus, it needs to be recognized and treated early in order to avoid or attenuate the chronic problems associated with OSAS, which are deleterious to a child's development. Adenotonsillectomy and, in select cases, continuous positive airwaypressure (CPAP) have been the preferred treatments for OSAS in children, and yet they are ineffective at fully ameliorating the disease. Minimally invasive treatments have recently been proposed, comprising intra-oral and extra-oral devices as well as speech therapy. Objetive: to conduct a meta-analysis on studies from around the world that used rapid maxillary expansion (RME) to treat OSAS in children. MATERIAL AND METHODS: We performed a meta-analysis of studies using RME for OSA treatment in children. A literature survey was conductedusing PubMed and Medline for English articles published up to December 2014 with the following descriptors: SleepApnea, Obstructive, Children, Treatment, Orthodontic, Othopaedic, Maxillaryexpansion. Studies were included in the meta-analysisif they were case-controlled studies, randomized, and involved non-syndromic children aged 0 to 12years old diagnosed with OSA by the polysomnography apnea-hypopnea index (AHI) before and after the intervention, submitted RME only. RESULTS: In all, 10 articles conformed to the inclusion criteria and were included in this meta-analysis. The total sample size across all these articles was 215 children, having a mean age of 6.7 years,of whom58.6%were male. The mean AHI during the follow-up was -6.86 (p <0.0001). CONCLUSIONS: We concluded that rapid maxillary expansion (RME) in children with OSAS appears to be an effective treatment for this syndrome. Further randomized clinical studies are needed to determine the effectiveness of RME in adults.

Randomized controlled study of a mandibular advancement appliance for the treatment of obstructive sleep apnea in children: A pilot study.

BACKGROUND: The current limited evidence may be suggestive that mandibular advancement appliance (MAAs) result in improvements in AHI scores, but it is not possible to conclude that MAAs are effective to treat paediatric OSA.There are significant weaknesses in the existing evidence due primarily to absence of control groups, small sample sizes, lack of randomization and short-term results. AIM: the objective of the present study was to evaluate MAAs in children with OSA. METHODS: Children presenting an apnea-hypopnea index (AHI) greater than or equal to one event per hour were considered to be apneic. This group of children with AHI greater than or equal to one was randomly divided through a draw into two subgroups: half of them in an experimental subgroup and half of them in a control subgroup. In the experimental subgroup, molds of each of these children's maxillary and mandibular arches were taken using standard molds and molding material. The control group did not use any intraoral device and did not undergo any type of treatment for OSAS. The MAAs used in this study had the aim of achieving mandibular advancement, thereby correcting the mandibular position and dental occlusion, and perhaps increasing the airway and treating OSAS. After 12 consecutive months of use of the mandibular advancement devices, polysomnography examinations using the same parameters as in the initial examinations were requested for both the experimental and the control subgroup. RESULTS: There was a decrease in AHI in the experimental group and an increase in the control group, with statistical significance. These data were used to calculate the sample size, which was 28 children in total in the groups. CONCLUSIONS: There was a decrease in AHI one year after implementing use of mandibular advancement devices, in comparison with the group that did not use these devices.

Value of sentinel lymph node biopsy in papillary thyroid cancer: initial results of a prospective trial.

The objectives of the study were to evaluate the performance of sentinel lymph node biopsy (SLNB) in detecting occult metastases in papillary thyroid carcinoma (PTC) and to correlate their presence to tumor and patient characteristics. Twenty-three clinically node-negative PTC patients (21 females, mean age 48.4 years) were prospectively enrolled. Patients were submitted to sentinel lymph node (SLN) lymphoscintigraphy prior to total thyroidectomy. Ultrasound-guided peritumoral injections of (99m)Tc-phytate (7.4 MBq) were performed. Cervical single-photon emission computed tomography and computed tomography (SPECT/CT) images were acquired 15 min after radiotracer injection and 2 h prior to surgery. Intra-operatively, SLNs were located with a gamma probe and removed along with non-SLNs located in the same neck compartment. Papillary thyroid carcinoma, SLNs and non-SLNs were submitted to histopathology analysis. Sentinel lymph nodes were located in levels: II in 34.7 % of patients; III in 26 %; IV in 30.4 %; V in 4.3 %; VI in 82.6 % and VII in 4.3 %. Metastases in the SLN were noted in seven patients (30.4 %), in non-SLN in three patients (13.1 %), and in the lateral compartments in 20 % of patients. There were significant associations between lymph node (LN) metastases and the presence of angio-lymphatic invasion (p = 0.04), extra-thyroid extension (p = 0.03) and tumor size (p = 0.003). No correlations were noted among LN metastases and patient age, gender, stimulated thyroglobulin levels, positive surgical margins, aggressive histology and multifocal lesions. Sentinel lymph node biopsy can detect occult metastases in PTC. The risk of a metastatic SLN was associated with extra-thyroid extension, larger tumors and angio-lymphatic invasion. This may help guide future neck dissection, patient surveillance and radioiodine therapy doses.

A prospective study of long-term dysphagia following total laryngectomy.

OBJECTIVE: To evaluate the degree of dysphagia in patients before and after total laryngectomy using the Performance Status Scale for Head and Neck Cancer Patients (PSS) and to determine the effects of post-operative radiation therapy, neck dissection, and pharyngoesophageal spasm on PSS scores. METHODS: We performed a controlled, prospective study at a tertiary referral university hospital. Twenty consecutive patients undergoing laryngectomy were included. Patients were followed for at least two years post-operative, without evidence of local, regional, or distant disease. Only patients with squamous cell carcinoma limited to the endolarynx requiring total laryngectomy with or without elective neck dissection for surgical management of cancer and with no pre-operative treatment were included. Dysphagia was evaluated by PSS prior to surgery and again two years postoperatively with an emphasis on eating in public and normalcy of diet domains. Video fluoroscopic evaluation of swallowing was performed one year after treatment. RESULTS: The relative number of patients with low mean scores in PSS (i.e. usually swallow paste of fluid food in presence of some selected persons or alone; < or = 50) increased after total laryngectomy (p = 0.04). Patients with lower scores reported more frequent spasm of the pharyngoesophageal segment (p = 0.005). Mean scores of both domains decreased after surgery (p < 0.05). CONCLUSION: Eating in public and normalcy of diet scores decreased in 50% of patients after total laryngectomy.

Laryngeal electromyography: contribution to vocal fold immobility diagnosis.

Laryngeal Electromyography (LEMG) is a diagnostic test commonly used in patients with vocal fold movement disorder. The aim of this study is to describe LEMG in patients with vocal fold immobility. A total of 55 dysphonic patients with vocal fold immobility diagnosed by laryngeal endoscopy were grouped according to probable clinical cause: 1) unknown; 2) traumatic; or 3) tumoral compression. They were submitted to LEMG by percutaneous insertion of concentric needle electrode. LEMG was conclusive in all patients and showed a majority with peripheral nerve injury. LEMG diagnosed peripheral nerve damage in 25 group 1, 12 group 2, and 11 group 3 patients. LEMG was normal in 4 patients, suggesting cricoarytenoid joint fixation. Central nervous system disorders was suggested in 2 and myopathic pattern in 1. As the major cause of vocal fold immobility is peripheral nerve damage, LEMG is an important test to confirm diagnosis.

Laryngeal electromyography in normal Brazilian population.

Quantitative analysis of normal values of motor unit action potentials duration and amplitude of muscles tireoaritenoideus (TA), cricotireoideus (CT), cricoaritenoideus lateralis (CAL), and cricoaritenoideus posterioris (CAP) was performed in 14 adult normal Brazilian volunteers. The recordings were obtained by percutaneously inserted concentric needle electrode. Different motor unit action potentials were manually selected in each muscle for quantitative computerized analysis of duration and amplitude. The mean values for duration and amplitude were respectively 3.8 ms and 413 microV for TA, 4.9 ms and 585 microV for CT 4.1 ms and 388 microV for CAL and 4.5 ms and 475 microV in CAP. There were no similar reports of normal values of motor unit action potentials in Brazilian subjects.

Laryngeal electromyography: technical features.

Laryngeal Electromyography (LEMG) is an auxiliary diagnostic method used for the comprehension and diagnosis of different neurological diseases that compromise laryngeal function. The most common LEMG technique is the percutaneous insertion of needle electrodes guided by surface anatomical references. We describe techniques for inserting needle electrodes into the tireoaritenoideus (TA), cricotireoideus (CT), cricoaritenoideus lateralis (CAL) and cricoaritenoideus posterioris (CAP) muscles; these are used at UNICAMP laryngology ambulatory, we discuss difficulties found and their proposed solutions. All patients were submitted to otorhinolaryngological, phonoaudiological and laryngeal endoscopy before LEMG. The CAP approach, by digital rotation of the thyroid cartilage was found to be the most difficult, followed by the CAL approach. TA and CT approaches gave no major problems, except with some older and obese patients. A significant complication of the TA approach via thyroid cartilage was a hematoma in one patient which partially obstructed the laryngeal lumen.

Ewing's sarcoma of the head and neck.

CONTEXT: Ewing's sarcoma is a rare neoplasm, which usually arises in long bones of the limbs and in flat bones of the pelvis, with the involvement of head and neck bones being very unusual. CASE REPORT: a case of Ewing's sarcoma occurring in the mandible of a 35-year-old female. Pain and swelling of the tumor were the main complaints. The early hypothesis was an undifferentiated malignant neoplasm, possibly a sarcoma. The CT scan depicted an expansive lesion, encapsulated, with septa and characteristics of soft tissue, involving the left side of the mandible and extending to the surrounding tissues. The patient underwent surgical excision of the lesion, the definitive diagnosis of Ewing's sarcoma was established, and the patient commenced on radiotherapy.

Neck lymph node metastases to the posterior triangle apex: evaluation of clinical and histopathological risk factors.

BACKGROUND: Dissection of posterior triangle apex (APEX) is a surgical step in supraomohyoid and lateral neck dissections. The prevalence of lymphatic metastases at this site and the clinicohistopathologic conditions that influence their occurrence have not been established. We have evaluated the prevalence and the risk factors for cervical metastases in lymph nodes of the APEX. METHODS: Sixty-two neck dissections were performed in 51 patients with squamous cell carcinoma of the oropharynx, hypopharynx, oral cavity, glottic larynx, and supraglottic larynx or with primary occult tumor. We correlated the presence of positive metastases in the APEX with the neck level involved either clinically (CLIN) or histopathologically (H/P) and with the number of CLIN- or H/P-positive neck levels with metastases. The prevalence of metastases in the APEX in elective (N0) and therapeutic (N+) neck dissections was also compared. This prevalence was also compared with that for each neck level. The histopathologic comparisons between the APEX and the neck levels were calculated for N0, N+, and all neck dissections. The primary site of tumor was correlated with the presence of H/P-positive nodes in the APEX. RESULTS: The overall prevalence of lymphatic metastases in the APEX was 6.5%. The prevalence in N0 neck dissections was 2.3% and in N+ neck dissections it was 16.7%. The prevalence of lymphatic metastases in the APEX for primary tumors of pharynx was 23.1%, for the oral cavity it was 3.6%, and it was 0% for other sites. Metastases in the APEX were not influenced by the neck level with CLIN or H/P metastases in N+ necks. The number of CLIN- or H/P-positive neck levels had no influence on histopathologic metastases in the APEX. Factors that influenced metastases in the APEX were positive histopathologic metastases at level II for N0 neck dissections and positive histopathologic metastases at level II or III for all neck dissections. All the comparisons were analyzed using Fisher's or Poisson's test. CONCLUSIONS: The prevalence of histopathologic metastases in the APEX in N+ necks is 7.3 times greater than that of N0 necks and for primary tumors of pharynx it was 6.4 times greater than for the oral cavity and significantly greater than for the larynx. Histopathologic metastases at level II for clinically N0 necks and histopathologic metastases to level II or III for all neck dissections are risk factors for metastases in the APEX. The number of positive levels did not influence the prevalence of metastases in the APEX. There are no isolated metastases in the APEX of the posterior triangle.

Granulocytic sarcoma of the larynx preceding chronic myeloid leukemia.

The authors report one case of granulocytic sarcoma infiltrating the larynx and cervical lymph nodes in a 50-year-old smoking patient. At the time of diagnosis there was no clinical and laboratory evidence of acute myeloid leukemia or chronic myeloproliferative disease. Four months after diagnosis, bone marrow morphology was consistent with chronic myeloid leukemia, accelerated phase. Cytogenetic abnormalities (Ph 1 chromosome, t(1; 12) (p36; p13), and trisomy of chromosome 20) were also found in hemopoetic cells. Granulocytic sarcoma preceding installation of chronic myeloid leukemia, as described here, seems to be a rare clinical event.