Long-term use of a neural prosthesis in progressive paralysis.

Brain-computer interfaces (BCIs) enable communication with others and allow machines or computers to be controlled in the absence of motor activity. Clinical studies evaluating neural prostheses in amyotrophic lateral sclerosis (ALS) patients have been performed; however, to date, no study has reported that ALS patients who progressed from locked-in syndrome (LIS), which has very limited voluntary movement, to a completely locked-in state (CLIS), characterized by complete loss of voluntary movements, were able to continue controlling neural prostheses. To clarify this, we used a BCI system to evaluate three late-stage ALS patients over 27 months. We employed steady-state visual evoked brain potentials elicited by flickering green and blue light-emitting diodes to control the BCI system. All participants reliably controlled the system throughout the entire period (median accuracy: 83.3%). One patient who progressed to CLIS was able to continue operating the system with high accuracy. Furthermore, this patient successfully used the system to respond to yes/no questions. Thus, this CLIS patient was able to operate a neuroprosthetic device, suggesting that the BCI system confers advantages for patients with severe paralysis, including those exhibiting complete loss of muscle movement.

A case of musical anhedonia due to right putaminal hemorrhage: a disconnection syndrome between the auditory cortex and insula.

A 63-year-old, right-handed professional chorus conductor developed right putaminal hemorrhage, and became unable to experience emotion while listening to music. Two years later, neurological examination revealed slight left hemiparesis. Neuromusicological assessments revealed impaired judgment of "musical sense," and the inability to discriminate the sound of chords in pure intervals from those in equal temperament. Brain MRI and tractography identified the old hemorrhagic lesion in the right putamen and impaired fiber connectivity between the right insula and superior temporal lobe. These findings suggest that musical anhedonia might be caused by a disconnection between the insula and auditory cortex.

A region-based two-step P300-based brain-computer interface for patients with amyotrophic lateral sclerosis.

OBJECTIVE: The P300-based brain-computer interface (BCI) is designed to help patients with motor disabilities to control their environment, and it has been used successfully in patients with amyotrophic lateral sclerosis (ALS). However, some ALS patients were unable to use the visual P300-BCI with the conventional row/column presentation. In this study, we evaluated the effect of a newly developed region-based two-step P300 speller, which has a larger flashing area than the conventional visual array. METHODS: Seven ALS patients and seven age- and sex-matched able-bodied control subjects were required to input hiragana characters using our P300 BCI system. We prepared two types of input procedures, the conventional row/column (RC) speller and the two-step speller, and evaluated their online performance. RESULTS: The mean online accuracy of the ALS patients was 24% for the RC condition and 55% for the two-step condition. The accuracy of the control subjects was 71% and 83% for the RC and two-step condition, respectively. Accuracy in ALS patients was significantly lower than that in the control subjects, and the new visual stimuli significantly increased accuracy of ALS patients. Using the new speller, two ALS patients showed an initial accuracy sufficient for practical use (>70%). The other two ALS patients, who performed better in the first trial using the new speller, continued to experience the BCI system, and their mean accuracy increased to 92%. CONCLUSIONS: The two-step procedure for the visual P300 BCI system provided significantly increased accuracy for ALS patients compared with a conventional RC speller. SIGNIFICANCE: The new region-based two-step P300 speller was effective in ALS patients, and the system may be beneficial to expand their range of activities.

Reduction rate of body mass index predicts prognosis for survival in amyotrophic lateral sclerosis: a multicenter study in Japan.

Malnutrition in the early stage has been reported as an independent predictor of survival in amyotrophic lateral sclerosis (ALS). We analyzed retrospectively the effect of variation of body mass index (BMI) on survival in ALS patients. In total, 77 consecutive ALS patients were enrolled from nine hospitals in Japan. Reduction rate of BMI was calculated from BMI before the disease onset and at the time of the first visit to each hospital. We analyzed the correlation between BMI reduction rate and total disease duration. Results showed that the median BMI reduction rate was 2.5 per year (interquartile range 1.3-3.8). The BMI reduction rate was significantly correlated with survival length (p <0.0001). There was also a significant difference in survival between ALS patients with a BMI reduction rate ≥ and < 2.5 (Kaplan-Meier survival analysis and the log-rank test, p < 0.0001; hazard ratio by the Cox model, 2.9816). In conclusion, faster reduction of BMI at the initial stage before the first visit to hospital predicts shorter survival length also in Japanese ALS patients.

[Study and development of a new automatic suction system (SS) of intratracheal sputum in order to improve QOL of many patients with tracheostomy mechanical ventilation (TMV) and their family members by this new autonomic SS].

In Japan, many patients equipped with TMV are under medical treatment at home after 1990. These patients can't put out sputa in trachea, so that these patient's family members must suck these patient's intratracheal sputa all days. Mr Yamamoto and Mr Tokunaga, main researchers of this study, began the study on the automatic SS of itratracheal sputa from 1999. In first stage, They developed the intermittent SS in detaining the suction tube within tracheal cannula, monitering the intratracheal pressure, but this system takes the ventilation away from the patient. Hypoventilation caused by this SS may cause the serious accident in patient. Therefore, we remodel the SS from intermittent SS to rollerpomp-type SS continuing to suck the itratracheal sputa with low volume from 2004, and thereafter we made up the SS of piston pomp type-SS finally at 2007. We developed the tracheal cannula with double suction holes of inner and lower hole in the lower part of its cannula together with the suction machine. We think that the practical use of this automatic SS will bring these patients with TMV and their family members great benefits. We desire that the practical use of this SS will be realized as soon as possible.

[Network to support patients with ALS in the Hyogo prefecture].

The author reports network to support patients with amyotrophic lateral sclerosis in the Hyogo prefecture. Three types of network are working, which are the network in the multidisciplinary team in the hospital, the medico-welfare network in the area and the network intra-hospitals in the prefecture. We have to establish effective network with a passion, a mission and an action.