Comparison of Pulmonary Functions at Onset of Ventilatory Insufficiency in Patients With Amyotrophic Lateral Sclerosis, Duchenne Muscular Dystrophy, and Myotonic Muscular Dystrophy

OBJECTIVE: To evaluate pulmonary functions of patients with amyotrophic lateral sclerosis (ALS), Duchenne muscular dystrophy (DMD), and myotonic muscular dystrophy (MMD) at the onset of ventilatory insufficiency. METHODS: This retrospective study included ALS, DMD, and MMD patients with regular outpatient clinic follow-up in the Department of Rehabilitation Medicine at Gangnam Severance Hospital before the application of non-invasive positive pressure ventilation (NIPPV). The patients were enrolled from August 2001 to March 2014. If patients experienced ventilatory insufficiency, they were treated with NIPPV, and their pulmonary functions were subsequently measured. RESULTS: Ninety-four DMD patients, 41 ALS patients, and 21 MMD patients were included in the study. The mean SpO2 was lower in the MMD group than in the other two groups. The mean forced vital capacity (FVC) in the supine position was approximately low to mid 20% on average in DMD and ALS patients, whereas it was 10% higher in MMD patients. ALS patients showed a significantly lower FVC in the supine position than in the sitting position. Maximal insufflation capacity, unassisted peak cough flow, maximum inspiratory pressure (MIP), and maximum expiratory pressure (MEP) were significantly higher in MMD group than in the other groups. MEP was significantly the lowest in DMD patients, followed by in ALS, and MMD patients, in order. CONCLUSION: Disease-specific values of pulmonary function, including FVC, MEP, and MIP, can be accurately used to assess the onset of ventilatory insufficiency in patients with ALS, DMD, and MMD.

Changes in Hyolaryngeal Movement and Swallowing Function After Neuromuscular Electrical Stimulation in Patients With Dysphagia

OBJECTIVE: To investigate immediate changes in hyolaryngeal movement and swallowing function after a cycle of neuromuscular electrical stimulation (NMES) on both submental and throat regions and submental placement alone in patients with dysphagia. METHODS: Fifteen patients with dysphagia were recruited. First, videofluoroscopic swallowing study (VFSS) was performed before NMES. All patients thereafter received a cycle of NMES by 2 methods of electrode placement: 1) both submental and throat regions and 2) submental placement alone concomitant with VFSS. The Penetration-Aspiration Score (PAS) and the NIH-Swallowing Safety Scale (NIH-SSS) were measured for swallowing function. RESULTS: During swallowing, hyolaryngeal descent significantly occurred by NMES on both submental and throat regions, and anterior displacement of hyolaryngeal complex was significant on submental placement alone. NMES on submental placement alone did not change the PAS and NIH-SSS. However, NMES on both submental and throat regions significantly reduced the NIH-SSS, although it did not change the PAS. Patients with no brainstem lesion and with dysphagia duration of <3 months showed significantly improved the NIH-SSS. CONCLUSION: Immediate hyolaryngeal movement was paradoxically depressed after NMES on both submental and throat regions with significant reductions in the NIH-SSS but not the PAS, suggesting improvement in pharyngeal peristalsis and cricopharyngeal functions at the esophageal entry rather than decreased aspiration and penetration. The results also suggested that patients with dysphagia should be carefully screened when determining motor-level NMES.

Atypical Supernumerary Phantom Limb and Phantom Limb Pain in a Patient With Spinal Cord Injury: Case Report

Supernumerary phantom limb (SPL) resulting from spinal cord lesions are very rare, with only sporadic and brief descriptions in the literature. Furthermore, the reported cases of SPL typically occurred in neurologically incomplete spinal cord patients. Here, we report a rare case of SPL with phantom limb pain that occurred after traumatic spinal cord injury in a neurologically complete patient. After a traffic accident, a 43-year-old man suffered a complete spinal cord injury with a C6 neurologic level of injury. SPL and associated phantom limb pain occurred 6 days after trauma onset. The patient felt the presence of an additional pair of legs that originated at the hip joints and extended medially, at equal lengths to the paralyzed legs. The intensity of SPL and associated phantom limb pain subsequently decreased after visual-tactile stimulation treatment, in which the patient visually identified the paralyzed limbs and then gently tapped them with a wooden stick. This improvement continued over the 2 months of inpatient treatment at our hospital and the presence of the SPLs was reduced to 20% of the real paralyzed legs. This is the first comprehensive report on SPLs of the lower extremities after neurologically complete spinal cord injury.