Quantitative and Morphological Assessment of Computed Tomography-depicted Gynecomastia in Spinal and Bulbar Muscular Atrophy.

RATIONALE AND OBJECTIVES: To evaluate the prevalence, size, and characteristics of gynecomastia on thoracic computed tomography (CT) in patients with spinal and bulbar muscular atrophy (SBMA) or amyotrophic lateral sclerosis (ALS), compared to those of patients with myasthenia gravis (as controls). MATERIALS AND METHODS: A total of 189 male patients (SBMA [n = 15]; ALS [n = 76]; control [n = 98]) who underwent thoracic computed tomography were included. The size of breast glandular tissue diameters, and characteristic of CT-depicted gynecomastia were compared. RESULTS: On multivariate logistic regression analysis, mean breast glandular tissue diameter (adjusted odds ratio [aOR] 1.13, 95% confidence interval [CI] 1.08-1.19), maximum breast glandular tissue diameter (aOR 1.14, 95% CI 1.08-1.20), prevalence of CT-depicted gynecomastia (aOR 21.71, 95% CI 5.39-87.38), dendritic or diffuse pattern of gynecomastia (aOR 35.30, 95% CI 8.02-155.40), and bilateral gynecomastia (aOR 41.96, 95% CI 10.20-172.69) were positively associated with SBMA, but not ALS. On receiver operating characteristic (ROC) analysis, the area under the curve of the mean breast tissue diameter for predicting SBMA was 0.92 with the optimal cutoff value of 16.5 mm. The ROC analysis showed that a maximum breast tissue diameter of 18.6 mm can also effectively distinguish SBMA from controls. CONCLUSION: These findings suggest that the evaluation of breast glandular tissue on thoracic CT could be a screening examination to distinguish SBMA patients and assist in its differential diagnosis.

Efficacy and safety of tacrolimus as long-term monotherapy for myasthenia gravis.

The objective was to evaluate the long-term efficacy and safety of tacrolimus monotherapy in myasthenia gravis (MG) patients. Immunosuppressive drug-naïve MG patients were administered tacrolimus, followed by thymectomy in some of the cases according to the clinical guideline for MG. Additional aggressive immunosuppressive therapies were allowed if the patients without thymectomy did not achieve minimal manifestation (MM) or better status after 3 weeks of tacrolimus administration or in the thymectomized patients by 1-2 weeks after the operation (i.e., 1st evaluation). Of all 14 patients included in this study, 8 of them (57%) achieved MM or better status at the 1st evaluation, and the remaining 6 (43%), who had failed to gain MM or better status at the 1st evaluation, also achieved MM or better status with 1 course of aggressive immunosuppressive therapy. The quantitative MG (QMG) scores, MG-Activities of Daily Living (ADL) scales, and anti-acetylcholine receptor (AchR) antibody levels were significantly decreased at 6 months and maintained thereafter. At the end of the follow-up period (41-70 months), all patients were in MM or better status. None of the patients experienced severe adverse effects. Our small preliminary study indicates that long-term tacrolimus monotherapy is possibly effective and safe for MG patients.

[A rare case of inclusion body myositis associated with anti-PM/Scl-75 antibodies].

A 71-year-old man presented with progressive muscle weakness of the four limbs in November 2014. His symptoms had started from the left leg in 2008, resulting in frequent falls. In 2011, he became unable to stand up without a handrail due to weakness of the both legs. Physical examination showed almost symmetric muscle weakness of the arms and legs; MMT4. The CK level was slightly elevated of 304 IU/l. The patient was diagnosed as having inclusion body myositis based on the muscle biopsy findings showing many fibers with rimmed vacuoles in addition to mononuclear cell infiltrating into the endomysium, surrounding and sometimes invading into non-necrotic muscle fibers. Anti-PM/Scl-75 antibodies were positive. Muscle strength improved after intravenous immunoglobulin therapy, although the effect was only temporary. This rare case suggests the autoimmunological etiology in inclusion body myositis.

Natalizumab is Effective for the Treatment of Relapsing-remitting Tumefactive Multiple Sclerosis.

We herein report the case of a 57-year-old woman presenting with a biopsy-proven tumefactive demyelinating lesion as her first clinical event. Subsequently, she displayed a relapsing-remitting course with recurrence of large demyelinating lesions exceeding 2 cm in diameter rather than the small ovoid lesions characteristic of multiple sclerosis. Administration of interferon beta did not suppress the disease activity. Finally, treatment with natalizumab, which is a humanized monoclonal antibody against the cell-adhesion molecule α4-integrin, was initiated, resulting in clinical and radiological stabilization. Our experience here suggests that natalizumab may be an effective therapeutic option for relapsing-remitting tumefactive multiple sclerosis with high disease activity.