Intravenous immunoglobulin for prophylaxis of acute exacerbation in Myasthenia Gravis.

Intravenous immunoglobulin (IVIg) treatment for acute exacerbations of Myasthenia Gravis (MG) was shown in several open-label studies. There are only two studies demonstrating the efficiency of regular intermittent IVIg therapy on MG patients who are not in their acute attack periods. Thirteen patients who had displayed an inadequate clinical response to immunosuppressive treatments, or who were not appropriate for immunosuppressive treatment due to the age factor and thus were given regular IVIg therapy, were retrospectively investigated. Moreover, the pre- and post-treatment attack frequencies were also evaluated. The mean number of attacks was 0.0960 attacks/year before IVIg therapy, and 0.0056 attacks/year after IVIg therapy (p = 0.002). The number and severity of the attacks were decreased in all patients. Eight patients (62 %) had used steroids; among them, steroid was completely stopped in two patients following the regular IVIg therapy, and the dose was decreased by 50 % in the other six patients. The requirement for pyridostigmine did not decrease in four patients, whereas this need decreased by 20-50 % in nine patients. IVIg can produce repeated beneficial effects in patients with MG and may be useful as an adjunct in the management of MG. IVIg has minimal adverse effects and ability to reduce corticosteroid dose. These results suggest that intravenous immunoglobulin maintenance therapy is a valid modality in patients with resistant treatment MG.

Therapeutic plasma exchange in treatment of neuroimmunologic disorders: review of 92 cases.

Therapeutic plasma exchange (TPE) is a procedure that reduces amount of circulating antibodies in patients through filtration for the treatment of neurologic diseases in which autoimmunity plays a major role. We reviewed the medical records of 92 neurologic patients who had been consecutively treated by TPE between June 2000 and April 2011 at Ankara University School of Medicine, Neurology Department and The Apaheresis Center. Neurological indications included myastehia gravis (MG, 16 patients), Guillain-Barre syndrome (GBS, 37 patients) and miscellaneous diseases (39 patients). The median TPE session number was 5 with a range of 1-8; total number of TPE procedures in all cases was 454. All MG patients improved with TPE during their hospitalization time. Regarding GBS, nearly 67% of the patients improved early, during their hospitalization time, either. In our series, 25% of GBS cases died because of dysautonomia. TPE was not effective in the treatment of the patients with Lambert-Eaton myasthenic syndrome, paraneoplastic polineuropathy, toxic polineuropathy, mononeuropathy multiplex in the case series. During the TPE procedures, 4 patients had hypotension and total number of the procedures was 21 in those patients. One patient had urticaria in only one session of total 5 TPE procedures. Two patients had septicemia; the first one had 3 and the second had 5 TPE procedures; both septic cases died. In conclusion, TPE is an effective treatment in neurologic diseases that autoimmunity plays an important role in the pathogenesis.

Therapeutic plasma exchange in the treatment of neuroimmunologic disorders: review of 50 cases.

Therapeutic plasma exchange (TPE) has been used for the treatment of neurologic diseases in which autoimmunity plays a major role. We reviewed the medical records of our patients who had consecutively been treated by TPE between January 1998 and June 2000. Neurological indications included myasthenia gravis (30 patients), multiple sclerosis attack (6 patients with remitting-relapsing course and 3 patients with secondary progressive course), Guillain-Barrè syndrome (6 patients), paraproteinemic neuropathy (2 patients), and chronic inflammatory demyelinating neuropathy (CIDP), transverse myelitis due to systemic lupus erythematosus, acute disseminated encephalomyelitis in one patient each. Continuous flow cell separators were used for TPE. TPE was generally given every other day for all of the patients and one plasma volume was exchanged for each cycle. Although the patients with secondary progressive multiple sclerosis (3 patients) and paraproteinemic neuropathy (2 patients) did not show any improvement after TPE, other patients' targeted neurological deficits were improved by TPE. During the TPE procedures, no patient had any morbidity or mortality, and the complications were mild and manageable such as hypotension, hypocalcemia and mild anemia; three patients had septicemia due to the venous catheter used for TPE. TPE is an effective treatment in neurologic diseases in which autoimmunity plays an important role in pathogenesis, and it is safe when performed in experienced centers.

Predictors of clinical outcome following extended thymectomy in myasthenia gravis.

OBJECTIVE: Thymectomy remains as the optimal treatment of choice in patients with myasthenia gravis (MG), however, the selection criteria for surgery remains controversial. METHODS: We examined the data charts of patients with MG underwent extended thymectomy. We investigated the possible correlations between the clinicopathologic features and clinical outcomes, and analyzed the data to clarify the effect of prognostic factors on clinical outcome. RESULTS: A total of 61 patients with a mean age of 35.8 +/- 12.2 years (range, 13-66 years) were analyzed. The overall improvement/remission and clinical worsening rates were 81.9 and 18.1%, respectively. Ossermann stage (P = 0.011) and presence of mediastinal ectopic thymic tissue (P = 0.007) showed a significant correlation with the clinical outcome. Multivariate analysis confirmed Ossermann stage (P = 0.0158), and presence of mediastinal ectopic thymic tissue (P = 0.0100) as independent predictors on clinical outcome. CONCLUSION: Ossermann stage and the presence of mediastinal ectopic thymic tissue are potential predictors on clinical outcome in patients with MG undergoing extended thymectomy.