Objective: To assess frequency, clinical characteristics and functional neuroimaging correlates of parkinsonian signs in a population-based series of new-diagnosed amyotrophic lateral sclerosis (ALS) patients.

Background: Parkinsonian signs in ALS patients have been reported in few papers, and their pathophysiological correlates are largely unknown.

Methods: ALS patients resident in Piemonte, Italy, diagnosed during 2013 were eligible to participate in the study. All patients were evaluated by two neurologists expert in movement disorders to detect the presence of parkinsonian signs (ALS-P) at diagnosis, 1 and 2 years. Patients underwent genetic tests and neuropsychological examination, and severity of parkinsonian symptoms was rated by MDS-UPDRS. DaTSCAN® SPECT was analysed visually and semiquantitavely and FDG-PET was compared between patients with or without parkinsonian signs by means of SPM8.

Results: Out of a total of 114 eligible ALS patients, 101 (64 men and 37 women; mean age at onset of 65.1 ± 11.7 years) were recruited. Twenty-eight patients (27.7%) were classified as ALS-P at the first visit. MDS-UPDRS scores were higher in ALS-P than ALS patients, in particular ALS-P patients showed more serious bradykinesia, rigidity and tremor (p<0,05). ALS-P patients were more frequently male (relative risk 1.36, c.i. 1.04-1.80; p=0.05). Age and site of onset, cognitive impairment, ALSFRS-R and MRC scores, genetics and survival were similar in ALS and ALS-P patients. Both visual and semi-quantitative analysis of DaTSCAN® revealed a normal scan in all but two ALS-P patients. On FDG-PET, ALS-P patients showed lower FDG uptake in left cerebellum and a relatively more preserved metabolism in right insula and in some right frontal regions compared to ALS patients.

Conclusions: In this population-based study more than one fourth of ALS patients showed parkinsonian signs. In ALS-P patients no significant nigrostriatal involvement could be demonstrated. Instead, relative hypometabolism in the left cerebellar hemisphere together with more preserved right prefrontal metabolism were found. These data suggest the involvement of brain circuitries other than classical nigrostriatal ones in ALS patients with parkinsonian signs.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/extrapyramidal-involvement-in-amyotrophic-lateral-sclerosis-results-of-a-prospective-population-based-study/