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We studied 169 patients with motor neuron disease.Seventeen showed abnormal amplitude reduction ofthe compound muscle action potential.Ten had focal loss of both amplitude and area across a specific segment (conduction block).Eight of the 10 had slowing of conduction across that segment.Nine were ment and had prominent hand involvement.Sixe had probable of definite upper motor neuron signs.Five of the 10 showed immunologic abnormalities(elevated GM1 antibody titers or paraproteinemia),and eight had had symptoms for more than 4 years.Seven of the 17 patients showed loss of amplitude without corresponding loss of area and focal slowing of conduction(temporal dispersion).Five of the seven were men,five had prominent hand involvement,and ive had definite or probably upper motor neuron signs.Two had immunologic abnormalities,and only one had had symptoms for longer than 4 years.Among 152 patients with no abnormality of conduction,64%were men,hands were dominately involved in 34%,uppor motor neuron signs were definite or probable in 72%,and 3%had immunolgic abnormalities.None had symptoms for more than 4 years.Beacause there were so many exceptions,we could not define a unique syndrome by criteria invovling conduction block,GM1 antibodies,or lack of upper motor neuron signs.The clinical syndrome associated with multifocal conduction block seemed uniform,however,and patients with conduction block had slower progression if there were no upper motor neuron signs. |
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amyotrophic lateral sclerosis,differential diagnosis
conduction block
fasciculation
gammaglobulin therapy,intravenous
GM1 ganglioside antibodies
muscle atrophy,progressive
nerve conduction studies
nerve conduction studies,motor
neuropathy
neuropathy,motor
neuropathy,motor,multifocal
neuropathy,multifocal
neuropathy,peripheral,treatment
paraproteinemia
treatment of neurologic disorder
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