Objective: Recent studies suggest that non-motor symptoms (NMS) including urinary disorders, erectile dysfunction in men, orthostatic hypotension, sleep disruption, constipation, depression and anxiety are common in cerebellar ataxia. However, there are few studies comparing the three groups. In this study, we will investigate: 1) whether the clinical manifestation between SCA, MSA and ILOCA are different 2) whether the NMS and QoL between SCA, MSA and ILOCA are different.

Background: Cerebellar ataxias represent a heterogeneous group of neurodegenerative disorders and can be categorized into two groups: hereditary or sporadic. Sporadic cerebellar ataxia may be the result of symptomatic cause, such as structural lesion, intoxication, endocrine disorders or idiopathic causes, such as multiple system atrophy (MSA) and idiopathic late-onset cerebellar ataxia(ILOCA). Hereditary ataxias have different modes of inheritance with mutations in scores of genes. Spinocerebellar ataxia (SCA) represents the most common autosomal dominant cerebellar ataxia. They share similar manifestations, including ataxia, pyramidal and extrapyramidal signs.

Methods: This prospective study recruited 23 patients with cerebellar ataxia. All patients were evaluated with SARA, UPDRS III, MoCA, MMSE, Non-motor symptoms scaled(NMSS), 36-Item Short Form Health Survey(SF-36), Beck Depression Inventory-II (BDI-II)

Results: The study population included 8 patients with spinocerebellar ataxia (SCA) types 1, 2, 3 and 6 (age, 49.38 ± 9.63), 11 patients with MSA (age 65.45 ± 6.96), and 4 patients with ILOCA (age 56.50 ± 9.88). Age of onset was earliest in the SCA when compared with the MSA and ILOCA patients (42.50 ±13.65 vs 62.55±70.4 vs 53.50±11.67 years). Because of the disease-specific demographic differences between SCA, MSA and ILOCA, as expected, age and disease onset age were different between the groups (P < .01). There were no significant differences in the disease duration, educational years, SARA, UPDRS part III, MMSE, SF-39, total NMSS and depression between the three group. However, there was a significant difference in sexual dysfunction and pain domain of NMSS in SCA, MSA and ILOCA patients (P < .05). Post hoc analyses revealed that MSA were more severe in the sexual dysfunction and pain domain compared with the SCA and ILOCA. There are significant differences between the three groups in the SF-36 scores of the physical health dimension(P=0.05). Post hoc analyses revealed that MSA had significantly worse physical health dimension compared with ILOCA(p<0.05).

Conclusions: In the current study , we investigate the clinical features ,NMS and QoL difference in SCA, MSA and ILOCA. We found that NMS are more severe in MSA, especially in sexual dysfunction and pain symptoms and QoL in MSA had worse than SCA and ILOCA.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/non-motor-symptoms-in-spinocerebellar-ataxia-multiple-system-atrophy-and-idiopathic-late-onset-cerebellar-ataxia/