Parkinson-like features in ALS with predominant upper motor neuron involvement

Carla D'Ascenzo, Diego Cecchin, Luca Santelli, Arianna Palmieri, Alessandra Gaiani, Giorgia Querin, Valentina Cima, Marco Volpe, Luca Bello, Franco Bui, Annachiara Cagnin, Corrado Angelini, Elena Pegoraro, Gianni Sorar

Research output: Contribution to journalArticle

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 123I-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 123I-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.

Original languageEnglish
Pages (from-to)137-143
Number of pages7
JournalAmyotrophic Lateral Sclerosis
Volume13
Issue number1
DOIs
Publication statusPublished - Jan 2012

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Keywords

  • Amyotrophic lateral sclerosis
  • DatScan
  • Nigrostriatal system
  • Upper motor neuron

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

Cite this

D'Ascenzo, C., Cecchin, D., Santelli, L., Palmieri, A., Gaiani, A., Querin, G., Cima, V., Volpe, M., Bello, L., Bui, F., Cagnin, A., Angelini, C., Pegoraro, E., & Sorar, G. (2012). Parkinson-like features in ALS with predominant upper motor neuron involvement. Amyotrophic Lateral Sclerosis, 13(1), 137-143. https://doi.org/10.3109/17482968.2011.603732