title: Natural history of upper motor neuron-dominant ALS.
abstract: A new amyotrophic lateral sclerosis (ALS) category named 'UMN-dominant ALS' and defined as 'due predominantly to UMN signs but with minor electromyogram (EMG) denervation or LMN signs on examination' has been proposed. The clinical and laboratory features of 20 patients with UMN-dominant ALS are described here, their disease course is analysed longitudinally according to their disability progression, and all these parameters are compared with those of typical ALS patients. Ten women and 10 men diagnosed with UMN-dominant ALS were evaluated. Their mean age at disease onset was 58.6 years. At the most recent evaluation, after a mean disease duration of 7.7 years, all patients progressed to a tetrapyramidal syndrome with pseudobulbar features of varying degree. No patient had respiratory problems. Cognitive impairment was observed in eight patients. The differences in disease progression between the UMN-dominant ALS and typical ALS patients proved significant (p <0.02) both with regard to the total ALSFRS-R score at six months and to each single region subscore at 12 months. Our findings suggest that there is both a different pattern of disability and longer survival in UMN-dominant ALS compared to classic ALS patients.
authors.affiliation: Department of Neurosciences, University of Padova, Italy. gianni.soraru@unipd.it
authors.forename: Gianni
authors.initials: G
authors.lastname: Sorarù
authors.forename: Mario
authors.initials: M
authors.lastname: Ermani
authors.forename: Giancarlo
authors.initials: G
authors.lastname: Logroscino
authors.forename: Arianna
authors.initials: A
authors.lastname: Palmieri
authors.forename: Carla
authors.initials: C
authors.lastname: D' Ascenzo
authors.forename: Valeria
authors.initials: V
authors.lastname: Orsetti
authors.forename: Marco
authors.initials: M
authors.lastname: Volpe
authors.forename: Valentina
authors.initials: V
authors.lastname: Cima
authors.forename: Gabriella
authors.initials: G
authors.lastname: Zara
authors.forename: Elena
authors.initials: E
authors.lastname: Pegoraro
authors.forename: Corrado
authors.initials: C
authors.lastname: Angelini
mesh_terms: D000328:Adult; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology*; D018450:Disease Progression; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology*; D009046:Motor Neurons / Q000502:physiology*