Defining per

Nilo Riva, Linda Chaabane, Marco Peviani, Daniela Ungaro, Teuta Domi, Giorgia Dina, Francesca Bianchi, Giorgia Spano, Federica Cerri, Paola Podini, Massimo Corbo, Ubaldo Del Carro, Giancarlo Comi, Caterina Bendotti, Angelo Quattrini

Research output: Contribution to journalArticlepeer-review


Growing evidence indicates that alterations within the peripheral nervous system (PNS) are involved at an early stage in the amyotrophic lateral sclerosis (ALS) pathogenetic cascade. In this study, magnetic resonance imaging (MRI), neurophysiologic analyses, and histologic analyses were used to monitor the extent of PNS damage in the hSOD-1 ALS rat model. The imaging signature of the disease was defined using in vivo MRI of the sciatic nerve. Initial abnormalities were detected in the nerves by an increase in T2 relaxation time before the onset of clinical disease; diffusion MRI showed a progressive increase in mean and radial diffusivity and reduction of fractional anisotropy at advanced stages of disease. Histologic analysis demonstrated early impairment of the blood-nerve barrier followed by acute axonal degeneration associated with endoneurial edema and macrophage response in motor nerve compartments. Progressive axonal degeneration and motor nerve fiber loss correlated with MRI and neurophysiologic changes. These functional and morphologic investigations of the PNS might be applied in following disease progression in preclinical therapeutic studies. This study establishes the PNS signature in this rat ALS model (shedding new light into pathogenesis) and provides a rationale for translating into future systematic MRI studies of PNS involvement in patients with ALS.

Original languageEnglish
Pages (from-to)658-670
Number of pages13
JournalJournal of Neuropathology and Experimental Neurology
Issue number7
Publication statusPublished - 2014


  • Animal models
  • Magnetic resonance imaging
  • Motor nerve
  • Motor neuron disease
  • Neuropathy
  • Peripheral nerve

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Clinical Neurology
  • Neurology
  • Cellular and Molecular Neuroscience


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