title: Is there a paraneoplastic ALS?
abstract: Our objective was to examine the strength of evidence in support of the paraneoplastic syndrome (PNS) as one cause of ALS and, if the association appears more likely than chance, determine which features of ALS imply concurrent malignancy. We reviewed the literature on concurrent ALS and neoplasia assessing the strength of evidence for the association. Most accounts of ALS and neoplasm are case reports or small uncontrolled series. In order of strength of evidence, three clinical situations that support a paraneoplastic aetiology for ALS are: 1) laboratory evidence of well-characterized onconeuronal antibodies, most often anti-Hu, anti-Yo or anti-Ri; 2) co-occurrence of ALS and a neoplasm known to cause PNS, usually lymphoma or cancer of the breast; and 3) combined ALS and a neoplasm not classically associated with PNS, without detectable onconeuronal antibodies. Clinical features that warrant evaluation of neoplasm include upper motor neuron disease in elderly females, rapid progression, non-motor signs, and young onset. In conclusion, most examples of ALS and neoplasm do not constitute a classically established PNS. Rare instances of elevated onconeuronal antibody titres or typical neoplasm, implies that, albeit rare, the PNS is one of a multitude of causes of ALS.
authors.affiliation: ALS Centre, CHU Tours.
authors.forename: Philippe
authors.initials: P
authors.lastname: Corcia
authors.forename: Paul H
authors.initials: PH
authors.lastname: Gordon
authors.forename: Jean-Philippe
authors.initials: JP
authors.lastname: Camdessanche
mesh_terms: D000328:Adult; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000209:etiology*; D000906:Antibodies / Q000378:metabolism; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000503:physiopathology; D009419:Nerve Tissue Proteins / Q000276:immunology / Q000378:metabolism; D010257:Paraneoplastic Syndromes / Q000150:complications* / Q000175:diagnosis
chemical_list: D000906:Antibodies; C095184:CDR2 protein, human; D009419:Nerve Tissue Proteins; C088463:ribonuclease inhibitor, brain
keywords: Motor neuron diseases; paraneoplastic; review