title: Parkinson-like features in ALS with predominant upper motor neuron involvement.
abstract: Owing to the frequent observation of poverty of movements, facial hypomimia and balance impairment, amyotrophic lateral sclerosis (ALS) variant with predominance of upper motor neuron involvement (UMN-ALS) is prone to be diagnosed with Parkinsonism. A clinical assessment, including the velocity-dependent stretch response test to differentiate between pyramidal and extrapyramidal stiffness; the Unified Parkinson's Disease Rating Scale and the Berg Balance Scale to assess degree of bradykinesia and postural instability; and (123)I-FP-CIT scintigraphy evaluation to investigate the nigrostriatal circuit involvement, were carried out to characterize Parkinson-like features in UMN-ALS patients. Sixteen UMN-ALS patients were included in the study. The velocity-dependent stretch response indicated spasticity in all the muscles tested. The degree of stiffness was found to be related to bradykinesia and postural instability. Eleven patients (70%) showed a reduction in striatal (123)I-FP-CIT uptake found to be related to disease duration and patients' ages but not to scores of the functional scales. Slowness of movements and postural instability noted in our patients could be mostly attributed to spasticity. The lack of any correlation between UPDRS or BBS scores and the degree of nigrostriatal impairment on DaTSCAN seems to disprove nigrostriatal circuit involvement in these extrapyramidal-like features.
authors.affiliation: Department of Neurosciences, University of Padova, via Giustiniani 5, Padova, Italy
authors.forename: Carla
authors.initials: C
authors.lastname: D'Ascenzo
authors.forename: Diego
authors.initials: D
authors.lastname: Cecchin
authors.forename: Luca
authors.initials: L
authors.lastname: Santelli
authors.forename: Arianna
authors.initials: A
authors.lastname: Palmieri
authors.forename: Alessandra
authors.initials: A
authors.lastname: Gaiani
authors.forename: Giorgia
authors.initials: G
authors.lastname: Querin
authors.forename: Valentina
authors.initials: V
authors.lastname: Cima
authors.forename: Marco
authors.initials: M
authors.lastname: Volpe
authors.forename: Luca
authors.initials: L
authors.lastname: Bello
authors.forename: Franco
authors.initials: F
authors.lastname: Bui
authors.forename: Annachiara
authors.initials: A
authors.lastname: Cagnin
authors.forename: Corrado
authors.initials: C
authors.lastname: Angelini
authors.forename: Elena
authors.initials: E
authors.lastname: Pegoraro
authors.forename: Gianni
authors.initials: G
authors.lastname: Sorarù
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000502:physiology*; D010300:Parkinson Disease / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology*; D011877:Radionuclide Imaging; D012189:Retrospective Studies