Brain and nerve = Shinkei kenkyū no shinpo
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Intravascular large B-cell lymphoma (IVLBCL) is an important cause of fever of unknown origin (FUO) and multiple organ failure (MOF). Earlier, most IVLBCL cases were diagnosed only postmortem; however, now, it is possible to diagnose and treat these cases antemortem. Although hematogeneous dissemination of malignant tumor cells except lymphoma is beyond the scope of present treatment regimens, IVLBCL (hematogeneous dissemination of lymphoma) can be treated by chemotherapy so correct diagnosis is important. ⋯ Delayed administration of rituximab and reduced dose of chemotherapy on the first course may be initially indicated in elderly, poor performance status or cases with high tumor burden. High-dose chemotherapy with autologous hematopoietic stem cell rescue should be considered, if possible. Aggressive combination therapy with high dose methotrexate is a recent idea because of central nervous system involvement, or relapse is common and there is poor prognosis.
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We report a case of malignant catatonia initially diagnosed as neuroleptic malignant syndrome (NMS) that responded successfully to diazepam administration. A 29-year-old man with mental retardation was admitted to our hospital because of high fever, muscle pain, and consciousness disturbance. Fifteen days before admission, he had developed muscle pain and weakness in his legs. ⋯ The clinical presentation of malignant catatonia is similar to that of NMS. Indeed, some authors have described NMS as a variant of malignant catatonia. If treatment is refractory in cases of NMS, malignant catatonia may be suspected, and changing treatment to diazepam administration may be useful.
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Review
[Treatment strategy for central nervous system involvement in intravascular large B-cell lymphoma].
Intravascular large B-cell lymphoma (IVLBCL) is a distinct disease entity of non-Hodgkin lymphoma which is characterized in the current WHO classification by the selective growth of tumour cells in the lumina of small vessels of various organs. This rare disease entity has a high incidence of central nervous system (CNS) involvement at diagnosis. Although several retrospective analyses indicate that the treatment of this lymphoma has improved in the post-rituximab era, risk of CNS recurrence at 3 years still reaches ~25%. ⋯ Considering that almost all IVLBCL patients have similar risk factors, CNS prophylaxis could also be effective, and thereby give rise to better clinical outcomes, in the treatment of IVLBCL, especially for patients without CNS involvement at initial diagnosis. Intensity regimens for CNS and systemic chemotherapy could be useful for patients with CNS involvement at initial diagnosis. This review describes the current understanding of clinical outcome and CNS involvement in IVLBCL, and discusses the optimal treatment strategy and future perspectives for CNS involvement of this rare lymphoma.
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Intravascular malignant lymphomatosis (IML) is a relatively rare type of malignant lymphoma that is mostly caused by B-cell type neoplastic lymphocytes and rarely by T-cell and NL-cell type cells. B-cell type IML is currently considered to consist of 2 types: a conventional European type and an Asian variant that was originally reported from Japan. In IML, the tumor cells primarily grow within the blood vessel lumina but may cause minimal extravascular infiltration around the involved vessels in some patients. ⋯ Although the antemortem diagnosis of IML has been difficult, the results of recent studies involving random skin biopsy are promising for such a diagnosis. Since the recent addition of rituximab to CHOP therapy has proven to markedly improve the prognosis of IML. We should therefore try to avoid overlooking this treatable disease.
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To investigate the various clinical courses of patients with amyotrophic lateral sclerosis (ALS), we developed a telephone survey system for determining the activities of daily living (ADL) status of patients with ALS. In this system, every 3 months, clinical research coordinators (CRCs) conducted a telephone survey using the flow charts of the revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R; Japanese version). To confirm the reliability of the results of the telephone survey, we compared the scores of 37 ALS patients obtained in the telephone surveys (telephone scores) to the scores obtained during clinical examinations by neurologists (neurologists' scores). ⋯ In the JaCALS, prospective clinical information was obtained using this telephone survey system. Of the 284 ALS patients who were registered at JaCALS over a year ago, 93% were followed up and provided ALSFRS-R scores and information relevant to prognosis. We have established an efficient and reliable telephone survey system for studying the longitudinal clinical courses of patients with ALS.