Is IVIg therapy warranted in progressive lower motor neuron syndromes without conduction block?
نویسندگان
چکیده
منابع مشابه
Differentiating lower motor neuron syndromes
Lower motor neuron (LMN) syndromes typically present with muscle wasting and weakness and may arise from pathology affecting the distal motor nerve up to the level of the anterior horn cell. A variety of hereditary causes are recognised, including spinal muscular atrophy, distal hereditary motor neuropathy and LMN variants of familial motor neuron disease. Recent genetic advances have resulted ...
متن کاملUse of human intravenous immunoglobulin in lower motor neuron syndromes.
OBJECTIVE To determine whether patients with the clinical phenotype of multifocal motor neuropathy but without the electrophysiological criteria for conduction block would respond to intravenous immunoglobulin (IVIg). METHODS Ten patients were selected with a slowly progressive, asymmetric, lower motor neuron disorder, and were treated prospectively with IVIg at a dose of 2g/kg over 5 days. A...
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Conduction in the central motor pathways of the brain and spinal cord was studied in 12 patients with motor neuron disease. Six healthy volunteers served as controls. Transcutaneous electrical stimulation of the cortex, cervical cord, thoracic cord and conus medullaris was used to determine motor latencies to the biceps brachii, thenar eminence and tibialis anterior muscles. Prominent, and ofte...
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Motor neuron disease is still one of the most dramatic neurological conditions; it reflects a range of which one end encompasses patients with the bulbar form of amyotrophic lateral sclerosis who have dysarthria and dysphagia and have a dramatically shortened life expectancy. For patients with probable or definite motor neuron disease according to established criteria, the recent introduction o...
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Here we illustrate a 74-year-old patient presenting with clinical signs of slowly progressive motor neuron disease. After electromyography demonstrated myopathic changes, a skeletal muscle biopsy was performed showing histological signs of granulomatous myositis. In extensive diagnostic workup underlying primary granulomas forming diseases were excluded and corticoid therapy was initiated, but ...
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ژورنال
عنوان ژورنال: Neurology
سال: 2013
ISSN: 0028-3878,1526-632X
DOI: 10.1212/01.wnl.0000437301.28441.7e