Massimo Filippi1,
Federica Agosta2, Elisa Canu2, Nilo Riva3,
Alberto Inuggi1, Adriano Chi4, Stefano Messina5,
Sandro Iannaccone6, Andrea Calvo4, Vincenzo Silani5,
Paola Valsasina2, Andrea Falini7, Giancarlo Comi3
1Neuroimaging
Research Unit, Institute of Experimental Neurology, Division of Neuroscience,
San Raffaele Scientific Institute, Vita-Salute San Raffaele University,
Milan, MI, Italy; 2Neuroimaging Research Unit , Institute of
Experimental Neurology, Division of Neuroscience, San Raffaele Scientific
Institute, Vita-Salute San Raffaele University, Milan, MI, Italy; 3Department
of Neurology, Institute of Experimental Neurology, San Raffaele Scientific
Institute, Vita-Salute San Raffaele University, Milan, MI, Italy; 4Department
of Neuroscience, University of Torino, Turin, TO, Italy; 5Department
of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico
Italiano, Milan, MI, Italy; 6Departement of Clinical Neuroscience,
San Raffaele Scientific Institute, Vita-Salute San Raffaele University,
Milan, MI, Italy; 7Department of Neuroradiology and CERMAC, San
Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan,
MI, Italy
We assessed the functional connectivity within motor and extra-motor brain networks in patients with primary lateral sclerosis (PLS) compared with healthy controls. We observed that functional abnormalities within large-scale neuronal networks occur in patients with PLS, involving not only the sensorimotor network but also the fronto-parietal and the executive networks. The pattern of increased functional connectivity in PLS patients correlated with the damage to the long white matter tracts and with cognitive deficits. This pattern of functional alterations in PLS supports a pathogenic loss of local inhibitory circuitry, rather than only compensatory recruitment.