1990
DOI: 10.1212/wnl.40.4.595
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Motor neuron disease and amyotrophic lateral sclerosis

Abstract: From 1984 to 1988, 11 of 120 patients (9%) with motor neuron disease (MND) had paraproteins detected by serum immunofixation electrophoresis (IFE), compared with 4 (3%) by cellulose acetate gels: 1 patient had progressive spinal muscular atrophy, 5 patients had amyotrophic lateral sclerosis (ALS), and 5 patients had ALS with probable upper motor neuron signs. Four of 5 patients (80%) with cerebrospinal fluid (CSF) protein content above 75 mg/dl had paraproteins, as did 6 of 30 with values above 50 mg/dl. Four … Show more

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Cited by 113 publications
(39 citation statements)
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“…However, all medical records were scrutinized by a neurol ogist before the MND diagnosis was accepted. Some disorders clinically indistinguishable from classic MND are known, namely multi focal conduction block [27] with a monoclonal paraprotein [28] and MND with antibodies against the GM1 ganglioside [29]. In the future, several other subtypes of MND might be identified and the aetiology and pathogenesis might vary for the different subtypes.…”
Section: Discussionmentioning
confidence: 99%
“…However, all medical records were scrutinized by a neurol ogist before the MND diagnosis was accepted. Some disorders clinically indistinguishable from classic MND are known, namely multi focal conduction block [27] with a monoclonal paraprotein [28] and MND with antibodies against the GM1 ganglioside [29]. In the future, several other subtypes of MND might be identified and the aetiology and pathogenesis might vary for the different subtypes.…”
Section: Discussionmentioning
confidence: 99%
“…Third, in the 13 autopsy patients ([l, 2, 4-6, 10-12) [17], excluding the 3 patients with lymphoma in that series. bFrom Table 2.…”
Section: Pathogenesismentioning
confidence: 95%
“…34,43,84,109 -111,113,151-153 In addition to typical cases of amyotrophic lateral sclerosis, patients with subacute and chronic motor nerve disease have been described. [151][152][153] There have also been reports of multifocal motor neuropathy with antibodies directed at nerve gangliosides in patients with lymphoma, 96 and autopsy-proven amyotrophic lateral sclerosis with Waldenström's macroglobulinemia and antibodies to sulfated glucuronic acid paragloboside. 110 Among patients with HL or NHL and either classic amyotrophic lateral sclerosis or progressive spinal muscular atrophy, 94,111,[151][152][153] some have paraproteinemia and abnormally increased cerebrospinal fluid protein levels with oligoclonal bands.…”
Section: Clinical Presentationsmentioning
confidence: 99%