1,721,034 research outputs found
Neuropatia motoria multifocale
No full textWe describe a patient with a chronic, asymmetric, limb weakness, fasciculations, relatively preserved reflexes, normal cranial nerves and no sensory abnormalities. The symptoms had been progressive over 2 years with a stepwise course, and then stabilized. Electrodiagnostic studies showed the presence of multifocal conduction blocks in motor nerves at sites not prone to compression; by contrast, the sensory conduction studies over the same nerve segments were normal, indicating selective involvement of motor fibers. Serum antibodies to the ganglioside GM1 were absent (ELISA). Treatment with prednisone failed to demonstrate any positive effect; a beneficial response was observed only with immunoglobulin therapy
Intravenous immunoglobulin therapy in amyotrophic lateral sclerosis
No full textSeven consecutive patients with amyotrophic lateral sclerosis (ALS) were treated with intravenous immunoglobulins (IVIg; 0.4 g/kg per day for 5 consecutive days followed by monthly 2-day infusions at the same daily dosage) continued with oral cyclophosphamide (1-2 mg/kg per day), for 4-13 months (mean 8.1). Response to treatment was assessed by means of the Medical Research Council (MRC) rating scale for muscle strength on 40 muscles (10 per limb), a clinical scale for bulbar function and a modified Rankin disability scale. All patients continued to deteriorate during treatment on as regards both their MRC score and either their bulbar or Rankin score or both. The progression of the disease during treatment, expressed as the monthly variation in MRC score (mean = -2.71; SD = 1.36), was no slower than that estimated before therapy (mean = -1.81; SD = 0.93). Even if the results of this small, uncontrolled study do not permit the exclusion of an effect of IVIg on the progression of ALS, they also do not provide any evidence that this expensive form of therapy consistently slows the course of the disease
Are there immunologically treatable motor neuron diseases?
No full textSeveral studies have addressed the issue of a possible immunological involvement in the pathogenesis of amyotrophic lateral sclerosis (ALS) or motor neuron disease (MND), particularly when the disease was associated with cancer, lymphoma or other monoclonal gammopathies or with the presence of serum antibodies to neural antigens. The hypothesis of the existence of immunologically treatable MND was reinforced by the occasional report of MND patients responding to immune or cytostatic therapies and by the identification among those with a purely lower motor neuron syndrome (LMNS) of a motor neuropathy, presently known as multifocal motor neuropathy (MMN), which almost invariably responded to immune therapies. These observations have led to several attempts to treat patients with MND or LMNS, either idiopathic or associated with the above mentioned conditions, with a number of immune or cytostatic therapies. The aim of this review is to verify whether the available data provide enough evidence to support the concept of dysimmune MND and to justify the use in these patients of potentially harmful immune cytostatic therapies
High-dose intravenous immunoglobulin and cyclophosphamide in amyotrophic lateral sclerosis
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