A case of pulmonary tuberculosis accompanied with immune thrombocytopenic purpura

A 30-year-old Japanese woman with chest pain and nodules in the left upper lung field was diagnosed as having pulmonary tuberculosis by sputum examination. Purpura on her legs had lasted for 3 months and her platelet count was 1.9 × 10⁴/mm³ on admission. She was also diagnosed as having immune thrombocytopenic purpura because of elevation of serum PA-IgG and proliferation of megakaryocytes in the bone marrow. Anti-tubercular therapy and steroid therapy were concurrently performed, resulted in recovery of the platelet count. Steroid therapy was gradually tapered off and then withdrawn, thereafter anti-tubercular therapy was finished. She has been relapse-free.Cases of pulmonary tuberculosis accompanied with immune thrombocytopenic purpura are rare. The pathogenesis in the present case was suggested to have occurred through an immunological mechanism.     

Cutaneous vasculitis induced by TNF inhibitors: a report of three cases

We describe 3 rheumatoid arthritis (RA) patients with anti-tumor necrosis factor (TNF) therapy-induced cutaneous vasculitis. Two cases were induced by infliximab and the other, in whom cutaneous vasculitis was found early at the start of therapy, was induced by etanercept. Skin biopsy was obtained in 2 patients, with histology-proven leukocytoclastic vasculitis. One patient spontaneously improved after cessation of the TNF inhibitor. Two patients required oral corticosteroid, the efficacy of which was observed to be excellent and rapid.     

A case of Möbius syndrome presenting with symptoms of severe infantile form of congenital muscular disorder

M?bius syndrome is a rare disorder characterized by congenital bilateral facial nerve palsy. Abducent palsy or other cranial nerve palsy, facial malformations, limb malformations, and skeletal malformations are common features associated with this syndrome. We report a 9-month-old infant in whom congenital muscular disorder was previously suspected because of facial muscle involvement (mask-like face), respiratory and swallowing disturbances, and hypotonia since birth. After an improvement in the respiratory infection, she showed slightly exaggerated deep tendon reflexes and an improvement in muscle tone. The occurrence of combined facial nerve palsy, glossopharyngeal nerve palsy, vagus nerve palsy, and hypoglossal nerve palsy strongly suggested that she had M?bius syndrome. Finally, the absence of the roots of bilateral facial nerves on an MRI confirmed that the disorder was M?bius syndrome. We propose that a thin slice MRI should be obtained to observe the cranial nerves around the brain stem if patients show symptoms of congenital myopathy or congenital myotonic dystrophy as well as facial nerve and other cranial nerve paralyses.     

Recurrent fever related to dantrolene sodium in a girl with sequelae of acute encephalopathy

A fever of unknown origin developed in a patient with sequelae of acute encephalopathy who had received dantrolene for severe spasticity. A chronic subdural hematoma was found on MRI, and initially it was suspected that the patient had an intracranial infection. However, close investigation ruled out the chronic subdural hematoma as the source of infection. The patient's fever continued in spite of administration of antibiotics and antimycotics. We suspected that the fever was drug-induced and discontinued the use of dantrolene. As a result, the patient's fever promptly went down. After discontinuation of dantrolene the patient experienced increased muscle tone, vomiting and sleep disturbances. Dantrolene was readministered with the consent of the patient's family, but the fever returned. When dantrolene was once again discontinued, the fever immediately went down. We concluded that the fever of the patient was induced by dantrolene.     

A study of the factors inducing the development of childhood-onset myasthenia gravis using CDR3 spectratyping analysis of the TCR repertoire

Myasthenia gravis (MG) is an autoimmune disease. AChR-specific autologous helper T (Th) cells are essential to the pathogenesis of MG. Factors correlated with the development of childhood-onset MG are unknown. In longitudinal studies, we found TCR Vbeta 2/5.1/6/7 usage in the development or relapse phases, but not in the remission phase. We also found that TCR Vbeta 8/9/13.1/15/18/20 usage persisted. The polyclonally expanded TCR Vbeta 2/5.1/6/7 by CDR3 spectratyping was found to be associated with the development of disease. These data suggest that in patients with childhood-onset MG, stimuli such as superantigens induced by a preceding infection, which cause development of the polyclonal pattern in TCR Vbeta families, play an important role in the development of the disease.