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Progression of nigro-striatal denervation in cerebellar Multiple System Atrophy: a prospective study

T. Wirth, I. Namer, B. Monga, C. Bund, A. Iosif, O. Gebus, S. Montaut, T. Bogdan, L. Robelin, M. Renaud, S. Kremer, C. Tranchant, M. Anheim (Strasbourg, France)

Meeting: MDS Virtual Congress 2021

Abstract Number: 575

Keywords: Ataxia: Etiology and Pathogenesis, Multiple system atrophy(MSA): Pathophysiology, Nigrostriatal dopaminergic synapse deficiency

Category: Parkinsonism, Atypical: MSA

Objective: To study the progression of nigro-striatal denervation and evaluate the capacity for [123I]-FP-CIT-SPECT and parkinsonian signs to differentiate MSA-C from idiopathic late-onset cerebellar ataxia (ILOCA).

Background: Natural history of nigro-striatal dopaminergic denervation remains poorly characterized in cerebellar multiple system atrophy (MSA-C).

Method: We included all patients referred to our centre for sporadic cerebellar ataxia occurring after 40 years of age (SLOCA). Every six months, exhaustive investigation was performed, 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. Patients finally diagnosed with probable MSA-C were compared to ILOCA. Patients with another diagnosis were excluded.

Results: Eighty-five patients were included between January 2013 and April 2017. After a mean follow-up of 33.8 months, 33 patients had probable MSA-C, 8 possible MSA-C and 44 ILOCA. SARA, SDFS and UPDRS-III scores worsened faster in the probable MSA-C group (p<0.01). Striatal/occipital ratio was lower at baseline (2.3 vs 3; p<0.01) and more decreasing in the probable MSA-C group (p<0.01).

Conclusion: The rapid worsening of parkinsonian and cerebellar signs with the early existence and progression of striatal dopaminergic denervation predict the diagnosis of MSA-C among SLOCA patients.

To cite this abstract in AMA style:

T. Wirth, I. Namer, B. Monga, C. Bund, A. Iosif, O. Gebus, S. Montaut, T. Bogdan, L. Robelin, M. Renaud, S. Kremer, C. Tranchant, M. Anheim. Progression of nigro-striatal denervation in cerebellar Multiple System Atrophy: a prospective study [abstract]. Mov Disord. 2021; 36 (suppl 1). https://www.mdsabstracts.org/abstract/progression-of-nigro-striatal-denervation-in-cerebellar-multiple-system-atrophy-a-prospective-study/. Accessed May 21, 2025.
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