Isolated vomiting due to a unilateral dorsal vagal complex lesion.
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Adult↗
Biomedical subjects
Naoki Kawaguchi
Publications and source records attributed to Naoki Kawaguchi.
High-dose intravenous immunoglobulin for the treatment of MuSK antibody-positive seronegative myasthenia gravis.
We treated two patients with anti-muscle specific tyrosine kinase (MuSK)-antibody positive seronegative myasthenia gravis (MG) with high-dose intravenous gammaglobulin (IVIg) and evaluated their clinical courses. Both patients were Japanese women, MuSK-positive seronegative MG, and were unresponsive to conventional treatments, including thymectomy, steroids, and tacrolimus. The patients required frequent hospitalization for plasmapheresis. In case 1, a 45-year-old woman, it was difficult to obtain blood access for plasmapheresis. High-dose IVIg, 400 mg/kg per day for 5 days, was administered in cases 1 and 2. In both cases, clinical improvement was observed 3 days after the start of IVIg therapy and lasted for 2 to 3 months. We propose that IVIg therapy is an effective treatment for MuSK-positive seronegative MG, when conventional treatments have failed.
Adult↗
Does perioperative high-dose prednisolone have clinical benefits for generalized myasthenia gravis?
OBJECTIVE: The purpose of this study was to clarify the clinical benefits of perioperative administration of high-dose prednisolone (PSL) combined with extended thymectomy on the long-term outcomes of 116 consecutive patients with generalized myasthenia gravis (MG). METHODS: A retrospective review was conducted on 116 patients diagnosed with generalized MG who received alternate-day oral administration of high-dose PSL (100 mg/alternate days) and had undergone transsternal extended thymectomy. Incidences of postoperative myasthenic crisis, adverse effects of steroid, long-term outcomes, such as complete stable remission (CSR), pharmacologic remission (PR) or improvement (Imp), and disease recurrence after CSR were evaluated. RESULTS: Six patients (5.2%) experienced post-thymectomy myasthenic crisis. Crude cumulative CSR and PR + CSR rates were 44.8 and 62.7%, respectively. Life table analysis showed that 41.8, 52.8 and 63.4% of the patients were in CSR at 3, 5 and 10 years, respectively. Multivariate analysis revealed that age and pretreatment classification according to the Myasthenia Gravis Foundation of America (MGFA) criteria tended to be independent predictors of CSR. There were 6.9% with compressive vertebral fracture, 13.8% with cataract, and 5.2% with steroid-induced diabetes. Life table analysis revealed that recurrence rates after CSR were 36.8 and 46.0% at 3 and 5 years, respectively. Patients with thymoma had a significantly higher rate of recurrence than those without thymoma (p = 0.001). CONCLUSIONS: Alternate-day administration of high-dose prednisolone reduced the risk of post-thymectomy myasthenic crisis. Presence of thymoma was a risk factor for MG recurrence after CSR.
Adult↗
[Myasthenia gravis].
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Activities of Daily Living↗
Treatment and outcome of myasthenia gravis: retrospective multi-center analysis of 470 Japanese patients, 1999-2000.
To clarify the current status of treatments and outcomes of patients with myasthenia gravis (MG) in Japan, a total of 470 patients (164 men and 306 women; mean age 41 years) were recruited from 19 Japanese tertiary medical centers in 1999-2000. Thymectomy was performed in 319 (68%) of the patients. Patients who received thymectomy were younger (p = 0.01) and had more severe disabilities (p < 0.01) than patients without thymectomy. Irrespective of receiving thymectomy, most of the patients were administered corticosteroids (64%), other immunosuppressive agents (10%), or cholinesterase inhibitors (86%). Of 395 patients followed up for more than 12 months after treatment (mean 8.0 years), 30% (34% of thymectomized and 21% of non-thymectomized patients) were in remission (no symptoms with/without medication), 34% had only ocular symptoms, and the remaining 35% still had weakness of bulbar or limb muscles at the end of follow-up. The prognosis of MG in Japan was generally favorable, but despite the frequent use of thymectomy and immunosuppressive treatments, approximately one-third of patients still had generalized weakness. More effective or intensive treatments are required to improve the prognosis.
Adolescent↗
Low-dose tacrolimus treatment in thymectomised and steroid-dependent myasthenia gravis.
OBJECTIVES: The effects of tacrolimus, a new immunosuppressive drug, which inhibits calcineurin pathway and also might enhance corticosteroid (CS) receptor-mediated gene expressions, on clinical outcome and biochemical data were evaluated in thymectomised and steroid-dependent myasthenia gravis (MG) patients. PATIENTS AND METHODS: We administrated low-dose tacrolimus (3 mg/day orally) to 17 steroid-dependent thymectomised MG patients. They were followed for 4 to 58 months, average 19.2 months. The MG activities of daily living (MGADL) scores and the dosage of prednisolone (PSL) were assessed at baseline and 4 months later. RESULTS: The average MGADL scores improved from 6.8 to 5.0 (p < 0.01) at 4 months; to 3.5 at the last visit (p < 0.01) as well as the average dosage of PSL reducing from 31.6 to 24.1 mg/alternate day (p < 0.01) at 4 months; 14.6 mg at the last visit (p < 0.01). CONCLUSIONS: The additional low dose tacrolimus therapy for steroid-dependent thymectomised MG is effective in improving symptoms as well as allowing the tapering of CSs.
Activities of Daily Living↗