George Umemoto

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 5xa0ml 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 (pxa0=xa00.047). The oropharyngeal transit time of the mild/moderate group was significantly shorter than that of the advanced group (pxa0=xa00.045). There was a significant negative correlation between the speed of tongue movement and the oropharyngeal transit time (pxa0=xa00.003, Rxa0=xa0−0.527). 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.

Dysphagia in duchenne muscular dystrophy versus myotonic dystrophy type 1

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).

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.

Treatment outcome of the two-part semi-rigid oral appliance in obstructive sleep apnea

Abstract Aim The aim of this study was to assess the effectiveness of the two-part semi-rigid oral appliance, Silensor ® (Erkodent, Tuttlingen, Germany) which prevents the mandible from retracting during mouth opening. Materials and methods Ten patients with mild or moderate obstructive sleep apnea (2 males and 8 females; mean age=62.5±10.0 years) were recruited and lateral cephalometric radiographs were taken. The patients underwent polysomnography before and after 3 months of receiving treatment with the Silensor ® . The relationship between the improvement in the polysomnographic variables after the therapy and the cephalometric features was analyzed. Results A significant difference was observed in the apnea–hypopnea index after 3 months of Silensor ® therapy (1st (baseline), 17.1±5.5; 2nd (therapy of Silensor ® ), 11.0±7.2, p =0.011). Furthermore there was a significant positive correlation between the improvement in the degree of slow wave sleep (%) and the mandibular plane angle ( R =0.662, p =0.037), as well as between the improvement in degree of slow wave sleep (%) and the lower face height ( R =0.845, p =0.002). A significant negative correlation was observed between the improvement in degree of sleep efficiency (%) and the soft palate area ( R =−0.809, p =0.005). Conclusion These results suggested that keeping the nasopharyngeal airway space during mouth opening improves apnea–hypopnea index of some patients with mild or moderate obstructive sleep apnea and quality of sleep in obstructive sleep apnea patients with a long lower face height and a small soft palate.

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.

Dysphagia in Two Cases of Fibrodysplasia Ossificans Progressiva

Fibrodysplasia Ossificans Progressiva (FOP) is an autosomal dominant genetic disorder caused by a heterozygous mutation in the ACVR1 gene. It is characterized by progressive heterotopic ossification (HO) and developmental skeletal defects. In this study, we assessed the swallowing function in two patients with FOP presenting the limited mandibular opening. The computerized tomography scans of case 1, a 56-year-old female, and case 2, a 66-year-old male, demonstrated HO of the medial pterygoid muscle between the mandibular ramus and lateral pterygoid plate. With limited mandibular movements, they usually ate a puree diet through their teeth with assistance. In case 2, HO of the suprahyoid and infrahyoid muscles between the mentum and collarbone, including the hyoid bone, was observed. On the fiberoptic endoscopic evaluation of swallowing and modified barium swallow, some foods were found to be retained in their pharynx even after repeated swallowing with the limited laryngeal elevation. These findings suggest that dysphagia in patients with FOP may be caused not only by the limited mandibular opening but also by the limited laryngeal elevation. However, further studies are required to generalize dysphagia in patients with FOP based on these findings.

Hospital-based study on emergency admission of patients with Parkinson's disease

Objective Parkinsons disease (PD) is a progressive neurodegenerative disease. As the severity of disease worsens, patients have lower tolerance for treatments and occasionally need to be hospitalized. This study focuses on characteristics of patients with PD who were emergently admitted to our hospital and evaluates their prognosis during hospitalization. Methods Hospital-based study on emergency admission was conducted in a consecutive series of patients with PD between April 2009 and March 2015. Results A total of 164 admissions involving 136 patients with PD with available medical records were identified. Among these, 40 admissions involving 38 patients were emergency admissions. The most common cause of hospitalization was aspiration pneumonia (n = 17) followed by parkinsonism hyperpyrexia syndrome (n = 6), cerebrovascular disease (n = 2), dehydration (n = 2), and others (n = 13). The mean Hoehn and Yahr stage at admission and discharge were 3.5 and 4.2, respectively, with significant differences between time points (p < 0.001). All patients except one presented with either postural instability gait difficulty phenotype (PIGD) or mixed phenotype with PIGD and tremor. All 17 patients with aspiration pneumonia had various combinations of three components: abnormalities seen on videofluoroscopy swallowing study, cognitive impairment, and history of psychiatric symptoms. Conclusion Aspiration pneumonia was the most common reason for emergency admission in patients afflicted with PD for more than five years. Abnormalities seen in videofluoroscopy, PIGD and mixed phenotypes, cognitive impairment, and history of psychiatric symptoms could potentially be predictors for aspiration pneumonia in patients with PD.

A case of sudden deterioration in Parkinson disease

An 84-year-old woman with a 9-year history of Parkinson disease (PD) had been treated with levodopa. Hoehn & Yahr stage was 3 and her daily activity was almost independent until her swallowing suddenly worsened and her parkinsonism deteriorated. Fiber optic endoscopy revealed a tablet stuck in the pharynx despite multiple attempts to swallow (figure; video at Neurology.org). With IV levodopa, her swallowing function and parkinsonism steadily recovered.

Rapid Improvement of Blood Glucose Level after Prosthetic Mandibular Advancement in a Patient with Diabetes Mellitus and Obstructive Sleep Apnea

Introduction: Obstructive Sleep Apnea Syndrome (OSAS) is often associated with impaired glucose metabolism. However, the effects of Prosthetic Mandibular Advancement (PMA) on blood glucose levels and insulin resistance remain unclear. Therefore, we assessed the immediate effect of PMA on glycemic control measured using a Continuous Glucose Monitoring System (CGMS) in a patient with Type 2 Diabetes Mellitus (T2DM) and OSAS. Case presentation: A 77-year-old Japanese woman with T2DM was diagnosed with OSAS with a Respiratory Disturbance Index (RDI) of 39.3. Because the patient did not accept Continuous Positive Airway Pressure (CPAP) therapy, she wore a PMA that advanced the mandible 7 mm forward. Overnight sleep apnea monitoring and CGM were performed before and after wearing the PMA. PMA induced a marked reduction in RDI from 39.3 to 12.8, an increase in the minimum hemoglobin saturation from 78.0% to 87.0%, and a decrease in the number of episodes of oxygen desaturation of >4% below baseline in during the bedtime from 31.3 /h to 12.1 /h. The mean glucose level markedly improved with PMA from 126.1 to 100.5 mg/dL. Conclusion: The patient with showed improvement in RDI and glucose levels after wearing the PMA overnight. To our knowledge, this is the first case of a patient with OSAS and T2DM showing a beneficial effect of PMA on rapid glycemic control. CGMS may greatly help to promote compliance with the treatment of OSAS in patients with T2DM.

G.P.7

To assess the evolution of dysphagia in patients with oculopharyngeal muscular dystrophy (OPMD). OPMD is an autosomal dominant form of late-onset muscular dystrophy. Ptosis and dysphagia are the most common presenting symptoms. In some reports, patients with OPMD who are homozygotes for two (GCN) expansions in the PABPN1 encoding gene have cognitive impairment and reduced life span. But about dysphagia, few things are known. For about 5xa0years, we followed one patient with (GCN)12 homozygotes and another patient with heterozygotes patient, (GCN)17. In addition to clinical evaluation, psychological tests, brain imaging studies and examination by videofluoroscopy (VF) were performed. OPMD started about age 50 years, with bilateral ptosis and dysphagia; external ophthalmoparesis and dysphonia followed within a few years, as well as weakness in four limb muscles. Maximum tongue pressure were lower than in other reports, 9.7 and 10.6xa0kPa. In VF, laryngeal penetration by water and large amounts of pharyngeal residue by solids were seen. Both patients had recurrent aspirations and lost weight. Second patient required feeding gastrostomies. Both patients showed mild cognitive decline, no depression nor psychotic episodes. Dysphagia in oculopharyngeal muscular dystrophy may also progresses faster in homozygote compared with heterozygote patients. It is important to be careful for dysphagia, as it may lead to reduce life expectancy.