Longitudinal Changes of Tongue Thickness and Tongue Pressure in Neuromuscular Disorders.

BACKGROUND: Swallowing dysfunction is related to major cause of adverse events and an indicator of shorter survival among patients with neuromuscular disorders (NMD). It is critical to assess the swallowing function during disease progression, however, there are limited tools that can easily evaluate swallowing function without using videofluoroscopic or videoendoscopic examination. Here, we evaluated the longitudinal changes in tongue thickness (TT) and maximum tongue pressure (MTP) among patients with amyotrophic lateral sclerosis (ALS), myotonic dystrophy type 1 (DM1), and Duchenne muscular dystrophy (DMD). METHODS: Between 2010 and 2020, TT and MTP were measured from 21 ALS, 30 DM1, and 14 DMD patients (mean ages of 66.9, 44.5, and 21.4 years, respectively) at intervals of more than half a year. TT was measured, by ultrasonography, as the distance from the mylohyoid muscle raphe to the tongue dorsum, and MTP was determined by measuring the maximum compression on a small balloon when pressing the tongue against the palate. Then we examined the relationship between these evaluations and patient background and swallowing function. RESULTS: Mean follow-up periods were 24.0 months in the ALS group, 47.2 months in the DM1group, and 61.1 months in the DMD group. The DMD group demonstrated larger first TT than the other groups, while the DM1 group had lower first MTP than the ALS group. The ALS group showed a greater average monthly reduction in mean TT than the DM1 group and greater monthly reductions in mean body weight (BW) and MTP than the other groups. Significant differences between the first and last BW, TT, and MTP measures were found only in the ALS group. CONCLUSIONS: This study suggests that ALS is associated with more rapid degeneration of tongue function over several years compared to DMD and DM1.

Therapeutic Efficacy of Twin-Block and Fixed Oral Appliances in Patients with Obstructive Sleep Apnea Syndrome.

PURPOSE: To compare the efficacy of twin-block (i.e., allows mouth opening) and fixed (i.e., maintains mouth closure) mandibular advancement splints (MASs) for the treatment of obstructive sleep apnea-hypopnea syndrome (OSA). MATERIALS AND METHODS: From 2011 to 2013, 23 patients with OSA in the twin-block group, and from 2013 to 2015, 29 patients in the fixed MAS group were included. All patients underwent polysomnography before and after 3 months of treatment. The two sets of polysomnographic and cephalometric variables were compared. RESULTS: A significant difference (p < 0.001) was observed in the apnea-hypopnea index before and after MAS treatment in both groups (twin-block group: 20.6 ± 11.5 vs. 14.7 ± 9.4; fixed group: 21.4 ± 15.2 vs. 11.2 ± 9.7). In the twin-block group, 5 patients (21.7%) were complete responders, 9 (39.1%) were fair responders, and 9 (39.1%) were nonresponders; the corresponding figures for the fixed group were 14 (48.3%), 9 (31.0%), and 6 (20.7%) patients. A significant between-group difference was observed in the distribution of responders (p = 0.046). The fixed group showed a significant improvement in the snoring index (p = 0.003), arousal index (p = 0.036), and desaturation rate (p = 0.012). Finally, the change in incisal overjet was larger in the fixed group than in the twin-block group (p < 0.001). CONCLUSIONS: These results suggest that fixed oral appliances are superior in treating OSA, based on their ability to prevent mouth opening and reduce incisal overjet.

Weight loss in Parkinson's disease: A retrospective comparison between oral medication and device-assisted therapies.

OBJECTIVE: Weight loss (WL) is the most common symptom among patients with Parkinson's disease (PD) and has been reported to start several years before the diagnosis of PD. The relationship between WL and PD treatment is complex. This study aimed to characterize the impact of PD treatment on WL and find clues to establish the administration of nutrition for patients with PD. MATERIALS AND METHODS: Eighty-two patients with PD (mean age, 58.4 ± 10.2 years; mean Hoehn and Yahr stage, 3.2 ± 0.7) were recruited. Their treatments included deep brain stimulation (DBS) therapy (n = 34), levodopa/carbidopa intestinal gel (LCIG) therapy (n = 13), and oral medication alone (n = 35). Based on the medical records, the age of onset, disease duration, treatment options, videofluoroscopic dysphagia scale, blood test results, and weight change were collected. RESULTS: The median WL per year and rate of WL were -1.0 ± 2.8 kg and -1.9 ± 4.7 %, respectively. Most patients (93 %) were classified into normal nutrition and mild malnutrition groups by their CONUT scores. The median WL of the DBS group was significantly lower than that of the oral medication alone group (p < 0.01). The rate of WL showed a significant negative correlation with the age of onset (rho = -0.328, p = 0.003), but showed a significant positive correlation with the disease duration (rho = 0.231, p = 0.04). CONCLUSION: These results highlighted WL in the early stages of PD and suggested the need for adequate monitoring for patients undergoing device-aided therapy as well as oral medicine-treated patients with greater WL.

Factors that impact dysphagia and discontinuance of oral intake in patients with progressive supranuclear palsy.

Objective: To evaluate the swallowing function in the advanced stages of progressive supranuclear palsy (PSP) and clarify the factors that lead to adjustment of food consistency and discontinuation of oral intake. Methods: A total of 56 patients with PSP were recruited. Based on medical records, information about the basic attributes, clinical features (including axial rigidity and dementia), food intake, the results of a videofluoroscopic swallowing study (VFSS), and the timing of nasogastric tube transition and gastrostomy were extracted. From the VFSS images, the presence or absence of aspiration and retrocollis were assessed. Results: The average age at the onset, diagnosis, and the final follow-up examination were 67.6 ± 6.4 years, 71.6 ± 5.8 years, and 75.4 ± 5.6 years, respectively. The average duration of illness was 64.6 ± 42.8 months. Twenty-four individuals (42.9%) were continuing oral intake, while 32 were tube-fed, among whom 16 (50.0%) underwent gastrostomy tube placement. There were significant differences in the duration from the disease onset to tube feeding between the patients with and without cognitive decline at the time of the diagnosis (p < 0.01) and in the duration from the initial VFSS to tube feeding between the patients with and without aspiration on the initial VFSS (p < 0.01). There were significant differences in the duration from the diagnosis to tube feeding and from the initial VFSS to tube feeding between patients with and without axial rigidity at the time of the diagnosis (p < 0.05 and p < 0.05, respectively). Additionally, there was a significant association between axial rigidity and retrocollis (p < 0.01). Conclusion: Cognitive decline, axial rigidity and retrocollis, which are associated with the deterioration of dysphagia in PSP, are the highest risk factors for the discontinuation of oral intake. The early identification of these factors associated with the progression of dysphagia can contribute to the improvement of patient care and management.

Association between denture use and food form in older adults requiring long-term care: A multicenter cross-sectional study.

PURPOSE: This study aimed to determine whether denture use contributes to maintaining and improving food forms in long-term care facility (LTCFs) residents. METHODS: In 888 residents of 37 LTCFs in Japan, the following were investigated: nutritional intake status, food forms, age, sex, Barthel index (BI), clinical dementia rating (CDR), number of teeth present, number of occlusal supports, swallowing function, and use of dentures. Among all residents, those who were well-nourished and had ≤9 occlusal supports were analyzed. Based on standardized criteria, the food forms consumed by the subjects were divided into two groups: dysphagia and normal diet, which were further classified into four levels. Analysis was performed using a generalized estimation equation with the four levels of food forms as dependent variables and age, sex, BI, CDR, presence of dysphagia, number of teeth present, and use of dentures as independent variables. RESULTS: The final analysis included 622 (70.0%) residents. Of these, 380 (61.1%) used dentures. The analyses revealed that food form was significantly associated with age (adjusted odds ratio [OR], 0.98), BI (OR, 1.04), number of teeth present (OR, 1.03), presence of dysphagia (OR, 0.44), and use of dentures (OR, 2.82). CONCLUSIONS: Denture use was associated with food forms among Japanese LTCF residents. This indicates that the use of dentures is related to the maintenance of food forms, even in the elderly who participate in few activities of daily living, have reduced cognitive function, and require long-term care.

Dysphagia in Duchenne muscular dystrophy versus myotonic dystrophy type 1.

INTRODUCTION: In this study we aimed to demonstrate the distinctive features of dysphagia in patients with myotonic dystrophy type 1 (DM1) and Duchenne muscular dystrophy (DMD). METHODS: Diet-type score (DTS) and maximum tongue pressure (MTP) were measured in 20 DM1 and 24 DMD patients; all patients were also examined by videofluoroscopy (VF). We used VF to measure the range of hyoid bone excursion (RHBE) during pharyngeal transit time and the area of pharyngeal residue (APR) after the first swallow. RESULTS: RHBE and APR values for DM1 patients were significantly greater than those for DMD patients. DTS and MTP did not differ between patients. A significant correlation was observed between DTS, MTP, and RHBE in DMD patients, but not in DM1 patients. CONCLUSIONS: Our findings reveal the differences in the distinctive features of dysphagia in DM1 and DMD. Adjustments in the diet of DMD patients in accordance with swallowing ability could be useful.

Relationship between motor dysfunction and chewing movement in patients with Parkinson's disease: A transversal study.

Objective: To assess the impact of chewing movement in patients with Parkinson's disease (PD), we examined the relation between chewing movement and motor dysfunction in association with PD progression. Methods: Thirty patients with PD (mean age, 68.9 ± 9.0 years; mean Hoehn and Yahr stage, 3.0 ± 0.7) were recruited. The PD condition was assessed in each patient by using the score of Movement Disorder Society Unified PD Rating Scale (MDS-UPDRS) part III score, body mass index (BMI), serum albumin (Alb), and tongue pressure, number of chews, mealtime, and chewing speed were collected. The patients were divided into two groups (mild and moderate PD groups) based on an MDS-UPDRS part III cut-off value of 32. Results: The chewing speed positively correlated with tongue pressure (rho = 0.69, p < 0.01) in the mild group, and with BMI (rho = 0.54, p = 0.03), serum Alb (rho = 0.63, p = 0.02), and number of chews (rho = 0.69, p < 0.01) in the moderate group. The MDS-UPDRS part III scores for all participants correlated negatively with chewing speed (rho = -0.48, p < 0.01), serum Alb (rho = -0.49, p < 0.01), and positively with mealtime (rho = 0.43, p = 0.01). Tongue pressure and serum Alb were identified to be as factors affecting the chewing speed (ß= 0.560, p < 0.01; ß= 0.457, p < 0.01, respectively). Conclusions: These results indicated that the progression of motor dysfunction in patients with PD is likely to affect chewing speed and the nutritional status decline may be linked to the impairment of chewing movement in these patients.

Cardiac sympathetic denervation could be associated with dysphagia in Parkinson's disease.

Background: Dysphagia is an important non-motor symptom that is closely associated with quality of living and mortality in Parkinson's disease (PD). However, the pathophysiology of dysphagia in PD remains inconclusive. We tried to confirm whether the occurrence of dysphagia could be related to sympathetic degeneration using cardiac 123I-metaiodobenzylguanidine (MIBG) scintigraphy. Methods: We prospectively recruited 27 PD patients and classified them into two groups (PD with dysphagia vs. PD without dysphagia) by Swallowing Disturbance Questionnaire (SDQ) score and compared the clinical characteristics, videofluoroscopic swallowing study (VFSS) findings and parameters from cardiac MIBG scintigraphy. Results: The mean early and late H/M ratios were significantly lower in the PD with dysphagia group than those in the PD without dysphagia group (1.39 ± 0.21 vs. 1.86 ± 0.21, p < 0.01; 1.26 ± 0.18 vs. 1.82 ± 0.29, p < 0.01). In the correlation analysis, both the early and late H/M ratios were negatively correlated with the SDQ score and total VDS score (r = -0.65, p < 0.01; r = -0.53, p < 0.01; r = -0.65, p < 0.01, r = -0.58, p < 0.01). Conclusion: We confirmed that cardiac sympathetic denervation might be associated with the presence and severity of dysphagia. This finding indicates that dysphagia in PD could be associated with a nondopaminergic mechanism.

Observational Variables for Considering a Switch from a Normal to a Dysphagia Diet among Older Adults Requiring Long-Term Care: A One-Year Multicenter Longitudinal Study.

This one-year multicenter longitudinal study aimed to assess whether older adult residents of long-term care facilities should switch from a normal to a dysphagia diet. Using the results of our previous cross-sectional study as baseline, older adults were subdivided into those who maintained a normal diet and those who switched to a dysphagia diet. The explanatory variables were age, sex, body mass index (BMI), Barthel Index, clinical dementia rating (CDR), and 13 simple and 5 objective oral assessments (remaining teeth, functional teeth, oral diadochokinesis, modified water swallowing test, and repetitive saliva swallowing test), which were used in binomial logistic regression analysis. Between-group comparison showed a significantly different BMI, Barthel Index, and CDR. Significant differences were also observed in simple assessments for language, drooling, tongue movement, perioral muscle function, and rinsing and in objective assessments. In multi-level analysis, switching from a normal to a dysphagia diet was significantly associated with simple assessments of tongue movement, perioral muscle function, and rinsing and with the objective assessment of the number of functional teeth. The results suggest that simple assessments can be performed regularly to screen for early signs of discrepancies between food form and eating/swallowing functions, which could lead to the provision of more appropriate food forms.

Impaired food transportation in Parkinson's disease related to lingual bradykinesia.

This study aimed to analyze quantitatively videofluoroscopic (VF) images of patients with Parkinson's disease (PD), to evaluate if the predicted factors of the oral phase of swallowing deteriorated with PD progression, and to demonstrate a relationship between the abnormal movements of the tongue and food transportation. Thirty PD patients were recruited and divided into mild/moderate (Hoehn & Yahr stages II and III) and advanced (stages IV and V) groups. They underwent measurement of tongue strength and VF using 5 ml of barium gelatin jelly as a test food. We measured the speed of bolus movement and the range of tongue and mandible movements during oropharyngeal transit time. The maximum tongue pressure of the mild/moderate group was significantly larger than that of advanced group (p = 0.047). The oropharyngeal transit time of the mild/moderate group was significantly shorter than that of the advanced group (p = 0.045). There was a significant negative correlation between the speed of tongue movement and the oropharyngeal transit time (p = 0.003, R = -0.527). Prolonged mealtimes and the ejection of insufficiently masticated food from the oral cavity into oropharynx were associated with PD progression. These results indicate the importance of the oral phase of swallowing in PD patients.

Impact of Progression of Parkinson's Disease on Swallowing Ability and Oral Environment.

This study investigated the impact of the severity and treatment of Parkinson's disease (PD) on the swallowing ability and oral environment of patients. Swallowing dysfunction increases the aspiration risk and may lead to poor oral health among patients with PD. We investigated the influences of PD progression and drug treatment on the swallowing ability and oral environment using simple noninvasive screening measurements. We recruited 87 patients with PD (mean age, 71.9 ± 8.0 years; mean Hoehn and Yahr score, 2.9 ± 0.9). The PD condition was assessed in each patient using the unified Parkinson's disease rating scale (UPDRS) part III, diet type and oropharyngeal function using the swallowing disturbances questionnaire (SDQ), maximum bite force (MBF), tongue pressure (TP), and oral bacterial count (OBC). Levodopa equivalent daily dose (LEDD) was also calculated for 56 participants. Based on an SDQ score of ≥11, 29.5% of patients were dysphagic, but almost all were still on a regular diet. The SDQ score was positively correlated with disease duration (rho = 0.228, p=0.047) and UPDRS part III score (rho = 0.307, p=0.007) but was negatively correlated with OBC (rho = -0.289, p=0.012). OBC was significantly higher among patients with an SDQ score of <11 (nondysphagic) (p=0.01), and the SDQ score was lower in patients with higher OBC requiring professional oral care (p=0.03). However, OBC was also negatively correlated with LEDD (rho = -0.411, p=0.004). These results indicated low self-awareness of dysphagia among the participants and an association between dysphagia and PD progression. Moreover, the oral environment could have deteriorated with swallowing dysfunction. Patients and clinicians should be aware that higher LEDD can increase xerostomia and associated deficits in oral health.

Case Report: GPi DBS for Non-parkinsonian Midline Tremor: A Normative Connectomic Comparison to a Failed Thalamic DBS.

Introduction: The clinical efficacy of deep brain stimulation (DBS) for midline tremor has been heterogenous. Here, we present an atypical case with facial and palatal tremor treated with DBS. We aimed to show the difference between the fibers affected by stimulation of the two targets [globus pallidus interna (GPi) and ventral intermediate (Vim) thalamic nucleus] using a normative connectome analysis. Case Report: A 76-year-old woman with a 4-year history of severe facial and palatal tremor due to craniofacial dystonia. Following a failed bilateral Vim DBS, explantation of preexisting leads and implantation of bilateral GPi leads resulted in the resolution of tremor symptoms following a failed bilateral Vim DBS. We performed a normative connectome analysis using the volume of tissue activated (VTA) as a region of interest. The results revealed that the fiber tracts associated with VTA of GPi DBS had connections with the facial area of the motor cortex while the Vim DBS did not. Conclusion: This case study suggests the possibility that GPi DBS may be considered for midline tremor, and that the normative connectome analysis may possibly offer clues as to the structures underpinning a positive response. We may refine targets for some of the more difficult to control symptoms such as the midline tremor in this case.

Association between Weight Loss and Food Form in Older Individuals Residing in Long-Term Care Facilities: 1-Year Multicenter Longitudinal Study.

Changing the food form for older adults requiring nursing care from a regular to dysphagia diet is thought to impact their nutritional status. We assessed the association between changes in food form and weight loss over 1 year in older adults. Older adults residing in long-term care facilities in Japan (n = 455) who participated in the baseline (2018) and follow-up (2019) surveys were divided into two groups (regular diet, n = 284; dysphagia diet, n = 171). The regular diet group was further divided into the weight loss (n = 80; weight loss ≥5% over 1 year) and weight maintenance (n = 204; weight loss <5%) groups. After 1 year, the Barthel Index significantly decreased, and the proportion of participants who switched from a regular diet to a dysphagia diet significantly increased in the weight loss group than in the weight maintenance group. Multivariate logistic regression analysis found that Barthel index variation (odds ratio (OR): 0.97, 95% confidence interval (CI): 0.94‒0.99), change from a regular diet to a dysphagia diet (OR: 4.41, 95% CI: 1.87‒10.41), and body weight at baseline (OR = 1.06, 95% CI: 1.01‒1.11) were significantly associated with weight loss. Our results suggest that maintaining the food form inhibits weight loss and improves health outcomes in older adults.

Management of Dysphagia in Patients with Parkinson's Disease and Related Disorders.

Various methods of rehabilitation for dysphagia have been suggested through the experience of treating stroke patients. Although most of these patients recover their swallowing function in a short period, dysphagia in Parkinson's disease (PD) and Parkinson-related disorder (PRD) degenerates with disease progression. Muscle rigidity and bradykinesia are recognized as causes of swallowing dysfunction, and it is difficult to easily apply the strategies for stroke to the rehabilitation of dysphagia in PD patients. Disease severity, weight loss, drooling, and dementia are important clinical predictors. Silent aspiration is a pathognomonic sign that may lead to aspiration pneumonia. Severe PD patients need routine video fluoroscopy or video endoscopy to adjust their food and liquid consistency. Patients with PRD experience rapid progression of swallowing dysfunction. Nutrition combined with nasogastric tube feeding or percutaneous endoscopic gastrostomy feeding should be considered owing to the increased risk of aspiration and difficulty administrating oral nutrition.

Impact of Residual Drug in the Pharynx on the Delayed-On Phenomenon in Parkinson's Disease Patients.

BACKGROUND AND OBJECTIVE: The delayed-on phenomenon (DOP) related to levodopa treatment frequently disturbs quality of life in advanced-stage Parkinson's disease (PD) patients. The objective of this study was to explore the impact of swallowing dysfunction on the development of DOP. METHODS: Swallowing function was investigated by endoscopic evaluation in 11 PD patients with the DOP and 9 PD patients without the DOP during the on phase. Residual drug in the pharynx after taking the drug in tablet, capsule, and powder forms was also observed. RESULTS: Residual drug was seen in the pharynx in six cases (30.0%). Pooling of saliva, delayed swallowing reflex, and residual drug were more frequent in the DOP group than in the group without the DOP (P < 0.05). The odds ratios for residual drug in the pharynx, pooling of saliva, and delayed swallowing reflex for the DOP were 42.7 (95% confidence interval, 1.89-962.9), 14.0 (95% confidence interval, 1.25-156.6), and 15.8 (95% confidence interval, 1.75-141.4), respectively. CONCLUSIONS: These results suggest that swallowing dysfunction leading to residual antiparkinsonian drug in the pharynx has substantial impacts on the DOP in PD patients.

Relationship between tongue pressure and functional oral intake scale diet type in patients with neurological and neuromuscular disorders.

INTRODUCTION: Patients with neurological and neuromuscular disorders (NNMD) frequently experience swallowing disorders that increase aspiration pneumonia risk and therefore require specialized diets or tube feeding. Diet type level usually is assessed by video fluoroscopic swallowing study (VFSS). To identify a simpler assessment method, we examined the association between diet type (based on the Functional Oral Intake Scale [FOIS]) diet type and maximum tongue pressure (MTP). METHODS: From 2011-2020, FOIS diet type level and MTP were assessed in a sample of 927 patients. Of these patients, 186 had Parkinson's disease (PD), 69 had Parkinson-related disease (PRD), 61 had multiple system atrophy (MSA), 42 had spinocerebellar degeneration (SCD), 147 had amyotrophic lateral sclerosis (ALS), 180 had myotonic dystrophy type 1 (DM1), and 242 had Duchenne muscular dystrophy (DMD). VFSS was conducted while patients swallowed water and foods containing barium. MTP measurements were collected the same day. Participants' diet type level was adjusted based on the VFSS, with some participants requiring multiple examinations. Relationships between diet type level and MTP were tested using univariate and Spearman rank correlation analyses. RESULTS: Mean MTP for the entire NNMD group (25.5 ± 13.1 kPa) was lower than that of healthy elderly individuals, as determined in previous reports. The highest MTP was found in the MSA group (32.2 ± 15.7 kPa) and the lowest in the DM1 group (19.1 ± 9.0 kPa). Diet type level was highest in the MSA group (5.8 ± 1.4) and lowest in the DMD group (5.2 ± 1.7). A significant correlation was observed between diet type level and MTP (R = 0.384, p < 0.001). The optimum MTP cutoff values-detected using ROC curves to predict a requirement to change to a dysphagia diet-was highest in the DMD group (29.0 kPa) and lowest in the ALS group (12.3 kPa). CONCLUSIONS: The decision to change NNMD patients to a dysphagia diet can be made based on MTP. Modifying a patient's oral diet (FOIS level ≤ 5) should be considered for those with a MTP of 10-25 kPa, with the cutoff value varying by disease.

Masticatory dysfunction in patients with myotonic dystrophy (type 1): a 5-year follow-up.

Eight patients with myotonic dystrophy type 1, DM1 (age range 45-63 years) were followed for a period of 5 years. Two sets of data analyzing masticatory function and activities of daily living (ADL) were obtained at baseline and at 5 years. The results from both time points were compared. The number of food items considered easy to chew, maximum bite force, occlusal contact area, number of teeth, and ADLs were significantly reduced (p < 0.05) at the second assessment when compared to the first. During the intervening 5 years, six of the eight patients adopted a soft diet. These results suggest deterioration in ADLs and masticatory function of patients with DM1. Continuous evaluation of the feeding ability and the type of diet are necessary for patients with DM1.

Characteristics of tongue and pharyngeal pressure in patients with neuromuscular diseases.

BACKGROUND: Tongue and pharyngeal pressure is an essential factor associated with the swallowing function; however, little is known about the difference in tongue and pharyngeal pressure between neuromuscular diseases. This study aimed to characterize tongue and pharyngeal pressure in myotonic dystrophy type 1 (DM1), Duchenne muscular dystrophy (DMD), and amyotrophic lateral sclerosis (ALS) patients. METHODS: This study recruited 17 DMD patients, 32 DM1 patients, and 26 ALS patients. They underwent separate measurements of tongue and pharyngeal pressure under videofluoroscopy, swallowing 5 mL of barium water. We measured the largest change in pharyngeal pressure in the hypopharynx and the upper esophageal sphincter (UES) over several swallows. RESULTS: The mean tongue pressure (TP) was greatest in the DMD group than in the other groups (p<0.01). There was a significant difference in pressure changes in the hypopharynx and UES between the DM1 group and other groups (p<0.01). Significant correlations were observed between pressure change in the UES and the patient's age in the DMD group (R=-0.500, p=0.045) and between pressure change in the hypopharynx and TP in the DM1 group (R=0.421, p=0.016). There was a significant correlation between pressure change in the hypopharynx and disease severity in the ALS group (R=0.435, p=0.030). CONCLUSION: Patients with DMD, DM1, and ALS have weakness in the muscles involved in swallowing; however, the results of this study suggested that each disorder has a distinctive profile of impairment in the swallowing function.

Dysphagia in Perry Syndrome: Pharyngeal Pressure in Two Cases.

BACKGROUND: To investigate the impact of dysphagia in Perry syndrome (PS), an autosomal dominant parkinsonism caused by mutation of DCTN1, which is associated with hypoventilation, depression, and weight loss. CASE PRESENTATION: We used tongue pressure measurements and manofluorography to investigate swallowing function in 2 patients with PS. Case 1, a 60-year-old male showing parkinsonism, and case 2, a 49-year-old male admitted with pneumonia, were diagnosed as having PS based on the DCTN1 gene analysis. Case 1 showed a pharyngeal retention of the bolus on videofluorography (VF) and a few swallows were required for its passage into the esophagus. However, tongue pressure and manometry were within the normal range. This patient could eat a normal diet under supervision. Case 2 required artificial ventilation and tube feeding on admission. The VF image showed a slow transfer of the bolus, delayed swallow reflex, and pharyngeal retention of the bolus that required several swallows for its passage into the esophagus. The tongue pressure was within the normal range, but manometry showed a significant decrease in pressure at the hypopharynx and upper esophageal sphincter. The oral intake of the patients was limited to 2 cups of jelly per day. CONCLUSIONS: The investigation of swallowing dysfunction of 2 cases of PS showed that maintaining pharyngeal pressure within the normal range was very important for oral feeding success and prognosis.

[Isolated Severe Dysphagia Associated with Medial Medullary Infarction: A Case Report and Review of the Literature].

A 59-year-old man developed a sudden onset of vertigo and nausea, and after a few hours, he could not swallow at all. On admission, neurological examination revealed severe dysphagia associated with other transient and mild neurological deficits, including left facial paresis, and hypesthesia in the right side of his body. MRI with diffusion weighted imaging showed a hyperintense signal lesion at the left rostral medial region of the medulla, prompting the diagnosis of an acute medial medullary infarction. His facial paresis and hypesthesia disappeared within 2 weeks. Despite lacking motor paralysis in the tongue, soft palate, and pharyngeal muscle, the patient had severe and prolonged dysphagia. It is speculated that the lesion in this patient is located at the central pattern generator (CPG), which is thought to be the human swallowing center. (Received April 1, 2016; Accepted June 1, 2016; Published October 1, 2016).