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.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/progression-of-nigro-striatal-denervation-in-cerebellar-multiple-system-atrophy-a-prospective-study/