PURPOSE: Myasthenia gravis (MG) is a lifelong autoimmune disorder that affects neuromuscular transmission. The long-term treatment plan should include immunotherapy. We investigated the long-term safety and efficacy of tacrolimus for the treatment of MG in real-world clinical practice. MATERIALS AND METHODS: We retrospectively reviewed 160 MG patients treated with tacrolimus from 2005 to 2015. Myasthenia Gravis Foundation of America (MGFA) clinical classification, MGFA post-intervention status, myasthenic functional score, and dose of oral prednisolone were investigated. RESULTS: Adverse events occurred in 68 patients (42.5%), most of which were minor and well-managed. Clinical severity scales improved after administration of tacrolimus, compared to the baseline. Compared to 6 months before administration of tacrolimus, prednisolone dose significantly decreased at 12 months after treatment (2.85±0.92 mg/day, p=0.002), 18 months after treatment (3.36±0.99 mg/day, p=0.001), and 24 months after treatment (3.71±0.93 mg/day, p<0.001). CONCLUSION: Tacrolimus may be effective in reducing the severity of MG and may permit a reduction in the steroid dose prescribed to the patients. Adverse events due to tacrolimus treatment were not serious.
Americas ; Classification ; Humans ; Immunotherapy ; Myasthenia Gravis ; Prednisolone ; Retrospective Studies ; Tacrolimus ; Weights and Measures

BACKGROUND AND PURPOSE: Acetylcholinesterase inhibitors (AChEIs) are widely used to treat myasthenia gravis (MG). Although AChEIs are usually tolerated well, some MG patients suffer from side effects. Furthermore, a small proportion of MG patients show cholinergic hypersensitivity and cannot tolerate AChEIs. Because repetitive compound muscle action potentials (R-CMAPs) are an electrophysiologic feature of cholinergic neuromuscular hyperactivity, we investigated the clinical characteristics of MG patients with R-CMAPs to identify their clinical usefulness in therapeutic decision-making. METHODS: We retrospectively reviewed the clinical records and electrodiagnostic findings of MG patients who underwent electrodiagnostic studies and diagnostic neostigmine testing (NT). RESULTS: Among 71 MG patients, 9 could not tolerate oral pyridostigmine bromide (PB) and 17 experienced side effects of PB. R-CMAPs developed in 24 patients after NT. The highest daily dose of PB was lower in the patients with R-CMAPs (240 mg/day vs. 480 mg/day, p<0.001). The frequencies of PB intolerance and side effects were higher in the patients with R-CMAPs than in those without R-CMAPs [37.5% vs. 0% (p<0.001) and 45.8% vs. 12.8% (p=0.002), respectively]. The MG Foundation of America postintervention status did not differ significantly between MG patients with and without R-CMAPs, and the response to immunotherapy was also good in both groups. CONCLUSIONS: Side effects of and intolerance to AChEIs are more common in MG patients with R-CMAPs than in those without R-CMAPs. AChEIs should be used carefully in MG patients with R-CMAPs. The presence of R-CMAPs after NT may be a good indicator of the risks of PB side effects and intolerance.
Acetylcholinesterase* ; Action Potentials* ; Americas ; Cholinesterase Inhibitors* ; Humans ; Hypersensitivity ; Immunotherapy ; Myasthenia Gravis* ; Neostigmine ; Pyridostigmine Bromide ; Retrospective Studies

BACKGROUND AND PURPOSE: Various magnetic resonance (MR) measurements have been proposed to aid in differentiating between progressive supranuclear palsy (PSP) and idiopathic Parkinson's disease (IPD); however, these methods have not been compared directly. The aim of this study was to determine which measurement method exhibits the highest power to differentiate between PSP and IPD. METHODS: Brain MR images from 82 IPD and 29 PSP patients were analyzed retrospectively. T1-weighted 3D volumetric axial images, or sagittal images reconstructed from those axial images were examined. MR measurements included the length from the interpeduncular fossa to the center of the cerebral aqueduct at the mid-mammillary-body level, adjusted according to the anterior commissure-posterior commissure length (MB(Tegm)), the ratio of the midbrain area to the pons area (M/P ratio) as measured by both Oba's method (Oba M/P) and Cosottini's method (Cosottini M/P), and a modified MR parkinsonism index (mMRPI). RESULTS: Receiver operating characteristic (ROC) analysis indicated that the areas under the ROC curves (AUCs) exceeded 0.70, with a high intrarater reliability for all MR measurement methods. ROC analyses of four MR measurements yielded AUCs of 0.69-0.76. At the cutoff value with the highest Youden index, mMRPI had the highest sensitivity, while Oba M/P offered the highest specificity. A comparison of the ROC analyses revealed that MB(Tegm) was superior to mMRPI in differentiating PSP from IPD (p=0.049). There was no difference in discriminating power among Oba M/P, Cosottini M/P, and MB(Tegm). CONCLUSIONS: Simple measurements of MB(Tegm) on axial MR images at the mid-mammillary-body level are comparable to measurements of the M/P ratio with regard to their ability to discriminate PSP from IPD.
Area Under Curve ; Brain ; Cerebral Aqueduct ; Diagnosis, Differential* ; Humans ; Magnetic Resonance Imaging ; Mesencephalon* ; Neuroimaging ; Parkinson Disease* ; Parkinsonian Disorders ; Pons ; Retrospective Studies ; ROC Curve ; Sensitivity and Specificity ; Supranuclear Palsy, Progressive*

BACKGROUND: The purpose of this study is to evaluate the disease-free survival (DFS) and overall survival (OS) of patients with stage IIB osteosarcoma at a single institution for 20 years and to compare the results according to the chemotherapy protocols. METHODS: From Jan 1988 to Nov 2008, 167 patients with osteosarcoma were treated at our hospital and among them, 117 patients (67 males and 50 females) with stage IIB osteosarcoma were evaluable. Their mean age was 22.6 years (range, 8 months to 71 years). Seventy-eight cases underwent the modified T10 (M-T10) protocol (group 1), 23 cases underwent the T20 protocol (group 2) and 16 cases underwent the T12 protocol (group 3). The DFS and OS were calculated and compared according to the chemotherapy protocols. RESULTS: At a mean follow-up of 78.9 months, 63 patients were continuously disease-free (63/117), 6 patients were alive after having metastatic lesions, 7 patients died of other cause and 41 patients died of their disease. The 5- and 10-year OS rates were 60.2% and 44.8%, respectively and the 5- and 10-year DFS rates were 53.5% and 41.4%, respectively. There was no significant difference of the OS and DFS between the chemotherapy protocols (p = 0.692, p = 0.113). CONCLUSIONS: At present, we achieved success rates close to the internationally accepted DFS and OS. We were able to achieve the higher survival rates using the M-T10 protocol over the 20 years. However, there was no significant difference of results between the chemotherapy protocols. We think the M-T10 protocol will achieve more favorable results in the near future.
Adolescent ; Adult ; Aged ; Antineoplastic Combined Chemotherapy Protocols/administration & dosage/*therapeutic use ; Bleomycin/administration & dosage ; Bone Neoplasms/*drug therapy/*mortality/surgery ; Chemotherapy, Adjuvant ; Child ; Child, Preschool ; Cyclophosphamide/administration & dosage ; Dactinomycin/administration & dosage ; Disease-Free Survival ; Doxorubicin/administration & dosage ; Female ; Follow-Up Studies ; Humans ; Infant ; Kaplan-Meier Estimate ; Leucovorin/administration & dosage ; Male ; Methotrexate/administration & dosage ; Middle Aged ; Neoadjuvant Therapy ; Osteosarcoma/*drug therapy/*mortality/surgery ; Survival Rate ; Vincristine/administration & dosage ; Young Adult

PURPOSE: To evaluate the clinical and radiological results after cementless total hip arthroplasty in dysplastic hips. MATERIALS AND METHODS: This study included 36 cases that underwent total hip arthroplasty using a cementless prosthesis in our hospital from July 1990 to May 2008. The average time of of follow-up was 103.7 months. In total, 27 cases about 24 patients were women, and 8 cases about 7 patients were men. The average age at the time of operation was 55.7 years(29-80 years). In total, 26 cases were Crowe type I and 8 cases were Crowe type II. One case was Crowe type IV. Acetabular cup angles and the contact ratio between the acetabular cup and the host bone were measured, and the grafting bone was observed for absorption in the follow-up radiographs. Revision of components was defined as a failure. RESULTS: Harris hip score was improved from 55.1 points pre-operatively to 86.1 points in the last follow-up. Revision was done in 12 cases(33.3%). Altogether, 10 cases revised the acetabular cup for osteolysis and polyethylene wear and 2 cases exchanged a polyethylene liner for only liner wear. CONCLUSION: Total hip arthroplasty in the dysplastic hip using a cementless acetabular cup, not a hydroxyapatite-coated acetabular cup, showed good clinical and radiological results when the contact ratio between acetabular cup and bone was above 70%.
Absorption ; Arthroplasty ; Crows ; Female ; Follow-Up Studies ; Hip ; Humans ; Male ; Osteolysis ; Polyethylene ; Prostheses and Implants ; Survival Rate ; Transplants

PURPOSE: We reviewed the radiological outcomes and survival rate of the total hip arthroplasty (THA) with AML(R) (Anatomic Medullary Locking, DePuy, Warsaw, IN, USA) hip prosthesis on long-term follow-up. MATERIALS AND METHODS: From May 1988 to December 1990, 93 hip arthroplasties were performed on 77 patients in our hospital. In this study, 30 patients, of whom 41 hips underwent the procedure, were alive and able to be included. Follow-up was average of 21.4 years. The mean patient age was 45 years (35-60 years) at the time of operation. Of the hip procedures included in our study, the reasons for THA were osteonecrosis of the femoral head in 25 hips, rheumatoid arthritis in 3 and acetabular dysplasia in 2. We analyzed the wear rate of the polyethylene, osteolysis of the femur and acetabulum and stress shielding of the femur on the follow-up radiographs. In addition, we investigated the survival rate of the prosthesis and causes of revision in the last follow-up. RESULTS: The polyethylene wear rate of the surviving acetabular cup was 0.15 mm/yr. Acetabular osteolysis was detected in 33 hips and was mostly in zone 2 and 3. Femoral osteolysis was showed in 32 hips in zone 1 and 7. Stress shielding over grade 3 was found in 5 of 21 femoral stems in over 13.5 mm in diameter. The grade of stress shielding did not progress with follow-up. Of the 33 hips, 26 (63.4%) cups were revised for polyethylene wear and osteolysis. There were 6 (21%) femoral stems revised for osteolysis. CONCLUSION: The cause of a high revision rate of the prosthesis was polyethylene wear and osteolysis. We predict that THA using AML(R) prosthesis with wear-resistant bearing surfaces could increase the survival rate on long-term follow-up over 20 years.
Acetabulum ; Arthritis, Rheumatoid ; Arthroplasty ; Femur ; Follow-Up Studies ; Head ; Hip ; Hip Prosthesis ; Humans ; Osteolysis ; Osteonecrosis ; Polyethylene ; Prostheses and Implants ; Survival Rate ; Tacrine ; Ursidae

PURPOSE: To evaluate the clinical and radiological results, as well as the survival rate, associated with total hip arthroplasty using a hydroxyapatite (HA)-coated anatomical femoral stem at a follow-up of > or =12 years. MATERIALS AND METHODS: From April 1992 to May 1997, 86 patients (102 hips) underwent total hip arthroplasty with a HA-coated ABG I (Anatomical Benoist Giraud; Howmedica) hip prosthesis. The average age at the time of surgery was 53.4 years and the mean duration of follow-up was 17.1 years (range, 12.1-21.0 years). The Harris hip score (HHS) and radiographic assessments of thigh pain were used to evaluate the clinical results. We observed osteointegration, cortical hypertrophy, reactive line, calcar resorption and osteolysis around the femoral stems. The survival rate of the femoral stems was evaluated by using the span of time to a revision operation for any reasons was defined as the end point. RESULTS: The mean HHS was 50.5 preoperatively and 84.2 at the time of last follow-up. Osteolysis only around the HA-coated proximal portion of the femoral stem was observed in 72 hips, cortical hypertrophy all around the distal portion of the femoral stem was observed in 38 hips, and calcar resorption was observed in 44 hips. A reactive line was observed in 13 hips, but was unrelated to component loosening. Stem revision operations were performed in 24 (23%) hips due to osteolysis (14 hips), fracture (5 hips) and infection (5 hips). The femoral stem survival rate was 75% over the mean duration of follow-up. CONCLUSION: Total hip arthroplasty using a HA-coated anatomical femoral stem showed necessitated a high rate of revision operations due to osteolysis around the femoral stem in this long term follow-up study.
Arthroplasty, Replacement, Hip* ; Durapatite* ; Follow-Up Studies ; Hip ; Hip Prosthesis ; Humans ; Hypertrophy ; Osteolysis ; Survival Rate ; Thigh

Desmoid tumor is a subtype of fibromatosis arising from deep fascial or soft tissue structure. It is characterized by locally aggressive behavior with a tendency to local recurrence, but is generally accepted the lack of ability for distant metastasis. Although excision is the best initial therapy, surgery is not always amenable in cases of lesions lying in difficult anatomical area. Two female patients with recurrent desmoid tumor in abdomen and pelvis after excision were treated with tamoxifen, goserelin, and sulindac. This therapy led to a progressive decrease of tumor size within 13 months in one patient. However, in the other patient, this combined therapy failed to reduce the size of the tumor. Despite the success of combined therapy with hormone and nonsteroidal anti-inflammatory drug is anecdotal, this treatment may improve the survival and reduce the recurrence in certain sub-group of desmoid tumor.
Abdomen* ; Deception ; Female ; Fibroma ; Fibromatosis, Aggressive* ; Goserelin* ; Humans ; Neoplasm Metastasis ; Pelvis ; Recurrence ; Sulindac* ; Tamoxifen*

PURPOSE: To evaluate the clinical and radiological results of total hip arthroplasty (THA) with a hydroxyapatite (HA)-coated ABG(R) (AnatomicalBenoist Giraud, Howmedica, Europe) hip prosthesis. MATERIALS AND METHODS: From March 1992 to December 1996, we performed 204 total hip arthroplasty with HA-coated hip prosthesis. This study included 142 hips. The causes of THA were osteonecrosis of the femoral head (122 hips), osteoarthritis (16 hips) and infectionsequalae (4 hips). Clinical results were evaluated using Harris hip score. We observed osteointegration, cortical hypertrophy, reactiveline around femoral stem, subsidence, polyethylene wear, osteolysis and radiolucent line around acetabular cup on the follow-up radiographs. RESULTS: Harris hip score was an average of 84.5 points at last follow-up. Only one femoral stem was revised for loosening. Reactive lines around uncoated portion of femoral stem was observed in 70 hips. The cortical hypertrophy was observed at zone 2, 3, 5 and 6 in 46hips. Forty-five acetabular cups (31.7%) were revised. The causes of cup revision were polyethylene liner wear and osteolysis (24 hips), loosening (19 hips) and recurrent dislocation (2 hips). CONCLUSION: We found that the HA-coated ABG(R) femoral stem had high survival rate at minimum 5 years follow-up after THA, but acetabularcup had high revision rate for loosening and osteolysis.
Acetabulum ; Arthroplasty, Replacement, Hip* ; Dislocations ; Durapatite ; Follow-Up Studies* ; Head ; Hip Prosthesis* ; Hip* ; Hypertrophy ; Osteoarthritis ; Osteolysis ; Osteonecrosis ; Polyethylene ; Survival Rate

HMG-CoA reductase inhibitor is widely used for the treatment of dyslipidemia, and for the prevention of stroke and cardiovascular disease. However, it is necessary to consider the adverse effects associated with this drug. Myopathy is a well-known side effect of statin therapy, but myotonic potentials in cases with statin myopathy have thus far been reported only rarely. We report four cases of myopathy with myotonic potentials on electromyography after multidrug administration. All of the patients recovered shortly after statin withdrawal.
Cardiovascular Diseases ; Dyslipidemias ; Electromyography ; Humans ; Hydroxymethylglutaryl-CoA Reductase Inhibitors ; Muscular Diseases* ; Oxidoreductases* ; Stroke