Impact of expiratory strength training in amyotrophic lateral sclerosis.

INTRODUCTION: We evaluated the feasibility and impact of expiratory muscle strength training (EMST) on respiratory and bulbar function in persons with amyotrophic lateral sclerosis (ALS). METHODS: Twenty-five ALS patients participated in this delayed intervention open-label clinical trial. Following a lead-in period, patients completed a 5-week EMST protocol. Outcome measures included: maximum expiratory pressure (MEP); physiologic measures of swallow and cough; and penetration-aspiration scale (PAS) scores. RESULTS: Of participants who entered the active phase of the study (n = 15), EMST was well tolerated and led to significant increases in MEPs and maximum hyoid displacement during swallowing post-EMST (P < 0.05). No significant differences were observed for PAS scores or cough spirometry measures. CONCLUSIONS: EMST was feasible and well tolerated in this small cohort of ALS patients and led to improvements in expiratory force-generating pressures and swallow kinematics. Further investigation is warranted to confirm these preliminary findings. Muscle Nerve 54: 48-53, 2016.

Cough assistance device for patients with glottis dysfunction and/or tracheostomy.

OBJECTIVE: To estimate the efficiency of a cough assistance device, the Cough Aid, in patients with weak respiratory muscles with bulbar palsy and/or tracheostomy. The Cough Aid is a device that has been developed to substitute for glottis function. DESIGN: Before-after trial. SUBJECTS/PATIENTS: A total of 74 patients with bulbar palsy and/or tracheostomy, as well as respiratory muscle weakness, were recruited. METHODS: Forced vital capacity, unassisted cough peak flow, lung insufflation capacity, and assisted cough peak flow were measured via tracheostomy or oronasal interface. Lung insufflation capacity and assisted cough peak flow were measured using the Cough Aid. RESULTS AND CONCLUSION: In all 74 subjects, lung insufflation capacities measured with the device were significantly higher than forced vital capacities (p < 0.01). Assisted cough peak flows measured using the device were also significantly higher than unassisted cough peak flows (p < 0.01). The Cough Aid is effective in helping to increase cough flow and assisting in the air stacking exercise by substituting the function of the glottis in patients who have glottis dysfunction or a tracheostomy tube.

Rehabilitation for postpolio sequelae.

BACKGROUND: Postpolio sequelae (PPS) are new, late manifestations that occur many years after the initial poliomyelitis infection. Recurrence of symptoms and fear of reactivation of the polio virus is particularly distressing to polio survivors. OBJECTIVE: This article outlines the diagnosis, pathophysiology, and management of PPS disabilities using a case vignette. DISCUSSION: Clinical features of PPS include fatigue, joint and muscle pain, new muscular weakness and bulbar symptoms. Diagnosis can be complicated particularly in nonparalytic cases of poliomyelitis. Disabilities in PPS may not be obvious to the observer but significantly affect the quality of life of the PPS patient. Previous rehabilitation intervention focussed on physical effort and determination to overcome disability at all costs. The treatment in PPS is now modified, and aggressive physical measures that may exacerbate muscle weakness are avoided. Most disabilities in PPS can be well managed with rehabilitation interventions that address limitations in patient activities of daily living, mobility and cardiopulmonary fitness.

The effect of a mechanical glottis on peak expiratory flow rate and time to peak flow during a peak expiratory flow manoeuvre: a study in normal subjects and patients with motor neurone disease.

We have evaluated a mechanical glottis in healthy volunteers and in patients with bulbar motor neurone disease. In healthy volunteers, the mechanical glottis increased peak flow rate and decreased the time to peak flow during forced expiration, but cough produced even higher flow rates and shorter times to peak flow. In patients, the mechanical glottis increased peak flow rate and decreased the time to peak flow. The mechanical glottis also produced higher peak flow rates when compared to the cough manoeuvres, and the time to peak flow was also significantly shorter with the mechanical glottis. We have shown that the use of a mechanical glottis tends to convert the airflow profile of a peak expiratory flow manoeuvre into that of a cough in both healthy volunteers and patients with motor neurone disease. Its potential role as an aid to clearance of airway secretions in patients with impaired laryngeal function remains to be seen.

Percutaneous endoscopic gastrostomy in the adult patient--a local case report.

Patients who require a gastrostomy tube placement have traditionally been subjected to various open surgical methods requiring laparotomy. Since it was first described Percutaneous Endoscopic Gastrostomy (PEG) has rapidly become the preferred method for gastrostomy tube placement. We present a case report to illustrate the simplicity and elegance of the technique in a patient with bulbar palsy.

Rehabilitation in progressive supranuclear palsy: case report.

Progressive supranuclear palsy (PSP) is a distinct clinicopathologic entity characterized by supranuclear ophthalmoplegia, pseudobulbar palsy, axial dystonia in extension, and subcortical dementia. Although relatively rare, PSP is disabling, thus rehabilitation techniques and management are indicated in nearly every case. This report describes the neurologic presentation, rehabilitation management, and outcome of treatment of a patient with PSP during a 12-month period. The patient required thorough neuromuscular, neuropsychological, speech, swallowing, vision, and social service evaluations prior to the implementation of a rehabilitation program. Therapeutic rehabilitation techniques focused on limb coordination activities, tilt board balancing, ambulation activities, and activities to improve route finding and visual scanning ability. Prism lenses were introduced to compensate for deficits in vertical eye movements. Treatment improved the patient's functional status. Later, as the patient's neurologic status deteriorated, it became necessary to educate the family and caretakers in the ongoing rehabilitation management of the patient.