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Progression of Nigrostriatal Denervation in Cerebellar Multiple System Atrophy: A Prospective Study
Thomas Wirth1, Izzie Jacques Namer2, Ben Monga2
1From the Neurology Department (T.W., A.V.I., O.G., S.M., T.B., L.R., C.T., M.A.), Strasbourg University Hospital, Strasbourg, France; Strasbourg Federation of Translational Medicine (FMTS) (T.W., C.T., M.A.), Strasbourg University, Strasbourg, France; INSERM-U964 (T.W., C.T., M.A.), CNRS-UMR7104, University of Strasbourg, Illkirch-Graffenstaden, France; Unit of Functional Neurosurgery (T.W.), National Hospital for Neurology and Neurosurgery, University College London Hospital, London, United-Kingdom; MNMS Platform (I.J.N., C.B.), University Hospitals of Strasbourg, Strasbourg, France; ICube (I.J.N., C.B., S.K.), University of Strasbourg / CNRS UMR 7357, Strasbourg, France; Department of Nuclear Medicine and Molecular Imaging (I.J.N., C.B.), ICANS, Strasbourg, France; Faculty of Medicine and School of Public Health (B.M.), Lumbumbashi University, Lubumbashi, Democratic Republic of Congo; Neurology Department (M.R.), Nancy Regional University Hospital, Nancy, France; Lorraine University (M.R.), Inserm U1256, NGERE, Nancy, France; and Imaging Department 2 (S.K.), Strasbourg University Hospital, Strasbourg, France. thomas.wirth@chru-strasbourg.fr.
Objectives:
Nigrostriatal dopaminergic denervation (NSDD) remains poorly characterized in cerebellar multiple system atrophy (MSA-C). We aimed to study NSDD progression in MSA-C and evaluate the capacity for [123I]-FP-CIT-SPECT and parkinsonism to differentiate MSA-C from idiopathic late-onset cerebellar ataxia (ILOCA).
Methods:
We included 85 patients successively referred for sporadic late-onset cerebellar ataxia (SLOCA). Every 6 months, SARA, UPDRS-III, and SDFS scores were measured, and MSA-C diagnostic criteria were searched for. Striatal/occipital dopaminergic binding ratio was evaluated every year with [123I]-FP-CIT-scintigraphy.
Results:
After a mean follow-up of 33.8 months, 33 patients had probable MSA-C, 8 possible MSA-C, and 44 ILOCA. SARA and UPDRS-III scores worsened faster in the probable MSA-C group (p < 0.01) compared with the ILOCA group. The baseline striatal/occipital ratio was lower (2.3 vs 2.97; p < 0.01) and more decreasing among patients with probable MSA-C (p < 0.01). Weighting dysautonomia and parkinsonism and/or NSDD as additional and principal criterion, respectively, in the possible MSA-C diagnostic criteria slightly improved their specificity (81.6% vs 76.9%) and sensitivity (77.8% vs 72.2%) to predict a final diagnosis of probable MSA-C.
Discussion:
Rapid symptom worsening and NSDD existence and progression predict MSA-C among patients with SLOCA. Parkinsonism, NSDD, and dysautonomia should be considered equivalent for possible MSA-C diagnosis.
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