title: Bilateral motor and premotor cortex hypometabolism in a case of Mills syndrome.
abstract: Mills syndrome is a rare condition characterized by slowly progressive upper motor neuron-predominant hemiparesis, belonging to the motor neuron disorder spectrum. Predominantly unilateral primary degeneration of corticospinal pathways is the supposed underlying pathophysiological mechanism. By means of (18)F-Fluorodeoxyglucose Positron Emission Tomography, we found significant (Statistical Parametric Mapping, SPM, analysis versus controls, uncorrected p < 0.005 at voxel level, p < 0.05 at cluster level, corrected for multiple comparisons) hypometabolism in motor and premotor areas of both hemispheres, mainly contralateral to the limbs weakness in a patient with a 10-year history of slowly progressive left-sided hemiparesis. No significant grey matter loss was found on voxel based morphometry (SPM). This supports the hypothesis of a slowly progressive neurodegenerative process involving primary motor and premotor cortex.
authors.affiliation: a Department of Neurosciences , Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI) , Genova , Italy.
authors.forename: Carlo
authors.initials: C
authors.lastname: Scialò
authors.affiliation: b Nuclear Medicine Department of Health Sciences (DISSAL) , University of Genova, IRCCS AOU San Martino-IST , Genova , Italy.
authors.forename: Silvia
authors.initials: S
authors.lastname: Morbelli
authors.affiliation: a Department of Neurosciences , Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI) , Genova , Italy.
authors.forename: Nicola
authors.initials: N
authors.lastname: Girtler
authors.affiliation: c Department of Neurosciences , Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI), University of Genova and UOC Medical Genetics, IRCCS AOU San Martino-IST , Genova , Italy.
authors.forename: Paola
authors.initials: P
authors.lastname: Mandich
authors.affiliation: a Department of Neurosciences , Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI) , Genova , Italy.
authors.forename: Giovanni Luigi
authors.initials: GL
authors.lastname: Mancardi
authors.affiliation: a Department of Neurosciences , Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI) , Genova , Italy.
authors.forename: Claudia
authors.initials: C
authors.lastname: Caponnetto
authors.affiliation: a Department of Neurosciences , Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI) , Genova , Italy.
authors.forename: Flavio
authors.initials: F
authors.lastname: Nobili
mesh_terms: D019788:Fluorodeoxyglucose F18 / Q000378:metabolism; D005820:Genetic Testing; D006801:Humans; D008297:Male; D008659:Metabolic Diseases / Q000209:etiology*; D008875:Middle Aged; D009044:Motor Cortex / Q000000981:diagnostic imaging / Q000473:pathology*; D016472:Motor Neuron Disease / Q000150:complications* / Q000235:genetics / Q000473:pathology*; D049268:Positron-Emission Tomography
chemical_list: D019788:Fluorodeoxyglucose F18
keywords: ALS; Amyotrophic Lateral Sclerosis; FDG PET; Mills Syndrome; Motor Neuron Disease; PLS; Primary Lateral Sclerosis