Muscle-specific tyrosine kinase-antibody-positive myasthenic crisis with detailed electrophysiologic studies.

A 69-year-old male who presented in a coma due to sudden respiratory arrest was transferred to our hospital. After endotracheal intubation with manual ventilation, he became alert and his neurologic findings were within the normal range, except for palsy of the respiratory muscles. Biochemical analyses of the blood and brain computed tomography failed to indicate the cause of the respiratory arrest. An edrophonium test did not improve the respiratory arrest. An urgent electromyogram at the dorsal interossei, biceps, and sternocleidomastoideus muscle and a repetitive nerve stimulation test at the trapezius and deltoid muscle were also negative on the first hospital day. However, on the 16th day in the hospital, a repetitive nerve stimulation test at the levator labii superioris alaeque nasi showed a waning phenomenon. This result indicated a diagnosis of myasthenia gravis. Anti.muscle-specific tyrosine kinase antibody was found to be positive. After treatment with plasmapheresis and prednisolone, he regained normal respiratory function. Anti.muscle-specific tyrosine kinase (MuSK)-antibodypositive myasthenia gravis (MG) (MuSK-MG) tends to be associated with a lower incidence of a positive edrophonium test, a lower incidence of a positive electrophysiologic study excluding the face, and a higher incidence of respiratory failure in comparison to anti.acetylcholine receptors (AchR)-antibody-positive MG (AchR-MG). Respiratory failure is curable with treatment. Accordingly, in addition to obtaining a precise diagnosis, an emergency physician should recommend an electrophysiologic study including the face to make a differential diagnosis for respiratory arrest when biochemical and radiologic studies fail to indicate the cause of the respiratory arrest

Post-intervention Status in Patients With Refractory Myasthenia Gravis Treated With Eculizumab During REGAIN and Its Open-Label Extension

OBJECTIVE: To evaluate whether eculizumab helps patients with anti-acetylcholine receptor-positive (AChR+) refractory generalized myasthenia gravis (gMG) achieve the Myasthenia Gravis Foundation of America (MGFA) post-intervention status of minimal manifestations (MM), we assessed patients' status throughout REGAIN (Safety and Efficacy of Eculizumab in AChR+ Refractory Generalized Myasthenia Gravis) and its open-label extension. METHODS: Patients who completed the REGAIN randomized controlled trial and continued into the open-label extension were included in this tertiary endpoint analysis. Patients were assessed for the MGFA post-intervention status of improved, unchanged, worse, MM, and pharmacologic remission at defined time points during REGAIN and through week 130 of the open-label study. RESULTS: A total of 117 patients completed REGAIN and continued into the open-label study (eculizumab/eculizumab: 56; placebo/eculizumab: 61). At week 26 of REGAIN, more eculizumab-treated patients than placebo-treated patients achieved a status of improved (60.7% vs 41.7%) or MM (25.0% vs 13.3%; common OR: 2.3; 95% CI: 1.1-4.5). After 130 weeks of eculizumab treatment, 88.0% of patients achieved improved status and 57.3% of patients achieved MM status. The safety profile of eculizumab was consistent with its known profile and no new safety signals were detected. CONCLUSION: Eculizumab led to rapid and sustained achievement of MM in patients with AChR+ refractory gMG. These findings support the use of eculizumab in this previously difficult-to-treat patient population. CLINICALTRIALSGOV IDENTIFIER: REGAIN, NCT01997229; REGAIN open-label extension, NCT02301624. CLASSIFICATION OF EVIDENCE: This study provides Class II evidence that, after 26 weeks of eculizumab treatment, 25.0% of adults with AChR+ refractory gMG achieved MM, compared with 13.3% who received placebo

Minimal Symptom Expression' in Patients With Acetylcholine Receptor Antibody-Positive Refractory Generalized Myasthenia Gravis Treated With Eculizumab

The efficacy and tolerability of eculizumab were assessed in REGAIN, a 26-week, phase 3, randomized, double-blind, placebo-controlled study in anti-acetylcholine receptor antibody-positive (AChR+) refractory generalized myasthenia gravis (gMG), and its open-label extension

Problems in communication and their solutions between severe aphasics and their wives at home

本研究の目的は,家庭での重度失語症者と妻のコミュニケーションでどのような困難が生じ,どのように解決されるかを明らかにすることである.重度失語症者3名とその妻の昼食時の会話をビデオに記録し,すべて文章化した.その中から15分間を分析対象とし,話題の変化で区切り,その区分ごとにコミュニケーション困難の有無と原因,困難解決の有無と解決理由を調べた.その結果,3組の対象は,喚語困難,錯語,聴覚的理解障害等により,全話題の半分またはそれ以上にコミュニケーション困難を生じていたが,その内の約7割は解決されていた.解決行動には「言語的補完」「内容の明確化」「相手への行動の促し」「自己の身体の使用」「道具の使用」「共有情報の使用」等があり,どの行動を使用するかは対象の組,または患者・妻のいずれであるかにより異なっていた.全ての組で1話題をめぐる発話数が,困難の有無にかかわらず健常者よりも多くなっていた.The purpose of the present study was to clarify what kinds of problems arose in communication between severe aphasics and their wives at home and how and to what extent they were solved. Three pairs consisting of a severe aphasic and his wife were asked to freely talk to one another during their lunch time at home, while they were videotaped. All the conversation was transcribed. 15 minutes from these typical communication scenes were selected and subjected to study. They were divided into "cuts" according to topics. Regarding each "cut", it was determined 1)whether a communication problem arose or not, 2)what caused the problem, if it occured, 3)whether the problem was solved or not, and 4)how it was solved, if this were the case. The results showed that each pair of subjects had the communication problems in half or more of the topics about which they communicated, because of word finding difficulty, paraphasia or impaired auditory comprehension and other problems. However, it was found that approximately 70% of the problems were solved by themselves. Their means of solving the problems were classified into six categories, namely "Verbal complementation", "Content clarification", "Encouraging of verbal expression", "Using their bodies", "Using of tools", "Guessing through previously shared information". Each pair, each patient and each wife, differed in what kind of solving procedure he or she used. Each pair showed a greater frequency of conversation turn for each topic than normally expected

Clinical safety and efficacy of implantation of octacalcium phosphate collagen composites in tooth extraction sockets and cyst holes

It was demonstrated that octacalcium phosphate collagen composite achieved notable bone regeneration in bone defects in preclinical studies. On the basis of the research results, an investigator-initiated exploratory clinical trial was conducted after approval from a local Institutional Review Board. This clinical study was performed as a single-arm non-randomized intervention study. Octacalcium phosphate collagen composite was implanted into a total of 10 cases of alveolar bone defects after tooth extractions and cystectomy. Safety assessment was performed in terms of the clinical course and several consecutive laboratory examinations, and sequential radiographs were used for efficacy assessment. All participants uneventfully completed the clinical trial without major problems in their general condition. Postoperative wound swelling was observed, as also commonly seen in tooth extraction or cystectomy. Although no serious liver dysfunction, renal dysfunction, electrolyte imbalance, or abnormal urinalysis results were recognized, the number of white blood cells and C-reactive protein level temporarily increased after the operation. An increase in radiopacity in the octacalcium phosphate collagen composite–implanted site was observed in all cases. Finally, the border between the original bone and the octacalcium phosphate collagen composite–implanted site became indistinguishable. These results suggest that octacalcium phosphate collagen composite could be utilized safely in clinical situations in the future

Efficacy of Octacalcium Phosphate Collagen Composite for Titanium Dental Implants in Dogs

Background: Previous studies showed that octacalcium (OCP) collagen composite (OCP/Col) can be used to repair human jaw bone defects without any associated abnormalities. The present study investigated whether OCP/Col could be applied to dental implant treatment using a dog tooth extraction socket model. Methods: The premolars of dogs were extracted; each extraction socket was extended, and titanium dental implants were placed in each socket. OCP/Col was inserted in the space around a titanium dental implant. Autologous bone was used to fill the other sockets, while the untreated socket (i.e., no bone substitute material) served as a control. Three months after the operation, these specimens were analyzed for the osseointegration of each bone substitute material with the surface of the titanium dental implant. Results: In histomorphometric analyses, the peri-implant bone areas (BA%) and bone-implant contact (BIC%) were measured. There was no difference in BA% or BIC% between OCP/Col and autologous bone. Conclusion: These results suggested that OCP/Col could be used for implant treatment as a bone substitute