Background Huntington's disease (HD) causes dysphagia and dementia, both of which are risk factors for malnutrition. Gastrostomy is used to sustain enteral intake in neurodegenerative diseases and specifically improves outcomes in ALS, but its indications and outcomes in HD are understudied. Objective To explore the indications and outcomes for gastrostomy for HD. Methods We performed a retrospective cross-sectional analysis of all HD admissions in the National Inpatient Sample. Logistic regression models compared the patient- and hospital-level characteristics associated with gastrostomy placement in HD and the prevalence of associated diagnoses in HD vs. ALS gastrostomy patients. We also examined in-hospital mortality, length of stay (LOS), and discharge status. Results Between 2000 and 2010, 5.12% (n = 1614) of HD admissions included gastrostomy tube placement. Gastrostomy patients were more likely to be Black (adjusted odds ratio [AOR] 1.55, 95% CI: 1.09–2.21) and have Medicare coverage (AOR 1.43, 95% CI: 1.0–2.05). The most common comorbidities were aspiration pneumonia (34.1%), dementia (31.3%), malnutrition (30.3%), and dysphagia (29.5%). Dementia and delirium were associated with discharge type but not LOS. Aspiration pneumonia, sepsis, and Elixhauser comorbidity index were associated with LOS but not discharge type. Compared to 7908 ALS gastrostomy patients, those with HD more frequently had aspiration pneumonia (34.1% vs. 20.5%, p < 0.0001), sepsis (28.1% vs. 13.7%, p < 0.0001), prolonged LOS (OR 1.14, 95% CI: 1.02–1.28), and skilled nursing facility discharge (p < 0.0001, Wald chi square test). Conclusions Gastrostomy is frequently performed in HD patients with dementia and aspiration pneumonia who are at increased risk for negative hospitalization outcomes.

Background: Clinical care for Huntington's disease (HD) is often provided in experienced centers that provide multidisciplinary care. However, the value of these centers and their uptake by HD families remain unknown. Objective: To describe the services provided by a new HD center, including estimates of capture of the population served. Methods: Retrospective review of a HD Center launched in 2015, including quantitative and qualitative data on clinic visits, demographic and clinical data. Results: We observed a rapid and ongoing growth on the annual number of clinic encounters, with high demand for in-clinic multidisciplinary care. Using census data and estimates of HD prevalence, we determined that we served about 20% of local patients with HD. Most HD patients received pharmacological treatment for psychiatric symptoms, and over half were treated for chorea. About 25% of new HD diagnoses were on patients without family history of HD. Finally, the demand for predictive testing in at risk individuals significantly increased following the press release reporting the successful completion of the Ionis-HTTRx (RG 6042) trial. Conclusions: This report indicates a high demand for multidisciplinary care by HD families, supporting its value, providing a snapshot of the organization and function of a single center. Furthermore, it demonstrates how dissemination of news related to research advances influence clinical behavior. Reporting similar information from other HD centers to would provide us with a more global view of the status of HD care across multiple geographical areas.

Gap analysis: a strategy to improve the quality of care of head and neck cancer patients I n the United States, there will be an estimated 49,670 new cases of head and neck cancer for 2017. 1 Head and neck cancer (HNC) is a term used to describe a range of tumors that originate in the area of the body spanning from the lower neck to the upper nasal cavity. 2 Specifically, they are malignancies arising in the mouth, larynx, nasal cavity, sinuses, tongue, lips, and numerous glands such as the thyroid and salivary. 2 To clarify, HNC, despite the encompassing name, does not include growths of the bones, teeth, skin, brain parenchyma, and eye; therefore, such tumors will not be addressed in this article. Patients with HNC often experience fragmented and uncoordinated care that leads to delays in cancer treatment, severe distress in patients and families, and dissatisfaction with care. Literature reports that these patients face numerous stressors including aggressive cancer treatments, severe symptoms, body image concerns, loss of speech, difficulty swallowing, nutritional issues, and respiratory problems that affect their quality of life and ability to function on a day-today basis. 3,4 In addition, patients with HNC and their families are challenged to navigate the health care system and to overcome the diffi

A 67-year-old male with cervical dystonia presented to his neurologist with complaints of globus sensation and difficulty swallowing. His past medical history included essential hypertension, hypothyroid, cardiac arrest, and bilateral globus pallidus interna (GPi) deep brain stimulator (DBS) implantation. Neurology initiated work-up with a barium swallow study which showed silent aspiration. The patient was then referred to the speech-language pathologist (SLP). The SLP's clinical examination revealed multiple swallows and globus sensation with all consistencies. Instrumental assessment using modified barium swallowing study (MBS) showed incomplete laryngeal elevation, absent pharyngeal contraction, severe pharyngeal retention, and no functional epiglottic movement. There was aspiration across consistencies trialed both during and after the swallow. The patient was seen for a course of swallowing therapy including exercises to target areas of dysfunction identified via MBS. What is the Diagnosis?After the patient's initial report of dysphagia symptoms, his DBS settings were increased; however, a month later, his symptoms worsened, so he was returned to previous settings. Based on the patient's history and reports of worsening swallowing function related to increased DBS settings, the SLP re-assessed clinical swallowing function in the RIGHT DBS OFF condition which resulted in elimination of multiple swallows and patient report of decreased globus sensation. MBS was therefore repeated in the bilateral DBS ON and bilateral DBS OFF conditions. With the DBS OFF, there was a significant improvement in overall pharyngeal swallowing function not seen in the DBS ON condition, specifically complete epiglottic inversion and a reduction in pharyngeal residue. Further, there were no instances of aspiration or penetration. The patient continued to attend swallowing therapy. Another MBS was recommended as follow-up and performed in four conditions: Bilateral DBS ON, bilateral DBS OFF, LEFT OFF, and RIGHT OFF (Figure 1). There was a 5-minute washout between conditions. With the LEFT OFF condition (Online Resource 1), there was no specific improvement and function was similar to that of the DBS ON condition (Online Resource 2). With the RIGHT OFF (Online Resource 3) and DBS OFF (Online Resource 4) conditions, there were improvements in epiglottic movement (to posterior-inferior translocation, with occasional normal movement) and a reduction in pharyngeal residue. There were no instances of aspiration or penetration.These findings lead to a diagnosis of DBS-related fixed epiglottis. While other aspects of the swallow improved Electronic supplementary material The online version of this article (