ObjectiveTo evaluate whether the clinical benefit and relapse rates in anti‐muscle‐specific kinase (MuSK) myasthenia gravis (MG) differ depending on the protocol of rituximab followed.MethodsThis retrospective multicentre study in patients with MuSK MG compared three rituximab protocols in terms of clinical status, relapse, changes in treatment, and adverse side effects. The primary effectiveness endpoint was clinical relapse requiring a further infusion of rituximab. Survival curves were estimated using Kaplan–Meier methods and survival analyses were undertaken using Cox proportional‐hazards models.ResultsTwenty‐five patients were included: 11 treated with protocol 4 + 2 (375 mg/m2/4 weeks, then monthly for 2 months), five treated with protocol 1 + 1 (two 1 g doses 2 weeks apart), and nine treated with protocol 4 (375 mg/m2/4 weeks). Mean follow‐up was 5.0 years (SD 3.3). Relapse occurred in 18.2%, 80%, and 33.3%, and mean time to relapse was 3.5 (SD 1.5), 1.1 (SD 0.4), and 2.5 (SD 1.4) years, respectively. Based on Kaplan–Meier estimates, patients treated with protocol 4 + 2 had fewer and later relapses than patients treated with the other two protocols (log‐rank test P = 0.0001). Patients treated with protocol 1 + 1 had a higher risk of relapse than patients treated with protocol 4 + 2 (HR 112.8, 95% CI, 5.7–2250.4, P = 0.002). Patients treated with protocol 4 showed a trend to a higher risk of relapse than those treated with protocol 4 + 2 (HR 9.2, 95% CI 0.9–91.8, P = 0.059).InterpretationThis study provides class IV evidence that the 4 + 2 rituximab protocol has a lower clinical relapse rate and produces a more durable response than the 1 + 1 and 4 protocols in patients with MuSK MG.
To analyze the frequency and clinical phenotype of neurosarcoidosis (NS) in one of the largest nationwide cohorts of patients with sarcoidosis reported from southern Europe. NS was evaluated according to the Diagnostic Criteria for Central Nervous System and Peripheral Nervous System Sarcoidosis recently proposed by Stern et al. Pathologic confirmation of granulomatous disease was used to subclassify NS into definite (confirmation in neurological tissue), probable (confirmation in extraneurological tissue) and possible (no histopathological confirmation of the disease). Of the 1532 patients included in the cohort, 85 (5.5%) fulfilled the Stern criteria for NS (49 women, mean age at diagnosis of NS of 47.6 years, 91% White). These patients developed 103 neurological conditions involving the brain (38%), cranial nerves (36%), the meninges (3%), the spinal cord (10%) and the peripheral nerves (14%); no patient had concomitant central and peripheral nerve involvements. In 59 (69%) patients, neurological involvement preceded or was present at the time of diagnosis of the disease. According to the classification proposed by Stern et al., 11 (13%) were classified as a definite NS, 61 (72%) as a probable NS and the remaining 13 (15%) as a possible NS. In comparison with the systemic phenotype of patients without NS, patients with CNS involvement presented a lower frequency of thoracic involvement (82% vs 93%, q = 0.018), a higher frequency of ocular (27% vs 10%, q < 0.001) and salivary gland (15% vs 4%, q = 0.002) WASOG involvements. In contrast, patients with PNS involvement showed a higher frequency of liver involvement (36% vs 12%, p = 0.02) in comparison with patients without NS. Neurosarcoidosis was identified in 5.5% of patients. CNS involvement prevails significantly over PNS involvement, and both conditions do not overlap in any patient. The systemic phenotype associated to each involvement was clearly differentiated, and can be helpful not only in the early identification of neurological involvement, but also in the systemic evaluation of patients diagnosed with neurosarcoidosis.
Background and Purpose-Intrapleural fibrinolytic therapy is a technique used to treat empyemas and parapneumonic effusions. Cerebral air embolism is an unusual potentially severe complication of this technique. Summary of Case-A patient with parapneumonic pleural effusion underwent pleural lavage with streptokinase when he suddenly demonstrated focal neurological signs and seizures. The CT revealed multiple air-isodense spots in right hemisphere of the brain, suggesting cerebral air embolism. As a result of early diagnosis and emergency hyperbaric oxygenation, the patient recovered without delayed sequelae. Conclusions-Air embolism is a potentially severe complication which can occur during fibrinolytic pleural lavage, and clinicians should be aware of this risk. In this context, the onset of acute focal neurological signs or seizures should suggest the possibility of air embolism and lead to the transfer of the patient close to a hyperbaric facility within a few hours. (Stroke. 2007;38:2602-2604.)Key Words: air embolism Ⅲ fibrinolytic therapy Ⅲ hyperbaric oxygenation Ⅲ stroke P arapneumonic effusions and empyema may complicate lower respiratory tract infections. In this condition, loculation of fluid is a major problem, and therapeutic options include conventional surgical drainage, video-assisted thoracoscopic surgery and the use of intrapleural fibrinolytic agents (urokinase and streptokinase) to break down fibrin bands that may cause loculation. 1-4 Although many physicians use fibrinolytic agents, the technique of instillation has not yet been standardized. 5 A Cochrane Systematic Review 6 evaluated the benefit of adding intrapleural fibrinolytic therapy to intercostal tube drainage in the treatment of complicated parapneumonic effusions and empyema. Controlled trials conducted up to 2003 were reviewed, concluding that intrapleural fibrinolytic therapy conferred significant benefits when compared with normal saline control. However, the routine use of fibrinolytic therapy cannot be recommended because the trial numbers were too small. A recent meta-analysis raised the same conclusions. 7 Remarkably, complications attributable to therapy were not observed at all in these trials, although several case reports of cerebral air embolism have been published. 8 -10 Herein we report a new case. Case ReportsA 50-year-old male patient, with a previous history of intestinal adenocarcinoma and resection of colorectal liver metastases developed a right purulent pleural effusion (Figure, A). He was admitted to hospital for daily pleural lavages with saline associated with streptokinase and for antibiotic treatment. During the third lavage he suddenly developed a generalized tonic-clonic seizure. Neurological examination showed a drowsy patient, with a conjugated gaze deviation toward the right, lower left facial paralysis, left hemiplegia and left Babinski sign. He immediately underwent a CT scan of the brain which revealed multiple air-isodense spots in the right hemisphere suggesting cerebral air embolisms (Figure, B)....
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