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Respiratory Function and Functional Decline in Spinocerebellar Ataxia Type 2

N. Mello, M. Zonta, H. Teive, A. Meira, B. Zeigelboim (Curitiba, Brazil)

Meeting: 2019 International Congress

Abstract Number: 272

Keywords: Spinocerebellar ataxia

Session Information

Date: Monday, September 23, 2019

Session Title: Ataxia

Session Time: 1:45pm-3:15pm

Location: Les Muses, Level 3

Objective: To evaluate the relationship between the values of Peak Expiratory Flow rate (PEFr), Maximum Inspiratory Pressure (MIP), the presence of respiratory complaints with disease time, balance scores, functional independence and severity of ataxia in individuals with SCA2.

Background: Spinocerebellar ataxia type 2 (SCA2) is characterized by cerebellar ataxia, accompanied by dysarthria, tremor, hyporeflexia, peripheral neuropathy, fasciculations of the facial muscles and limbs1.Subclinical respiratory dysfunction has been observed in individuals with SCA2. There are no studies relating respiratory dysfunction to functional decline in SCA2.

Method: Cross-sectional study considering age, duration of illness, Berg Balance Scale (BBS) and independence for basic activities (FIMs) and instrumental (IADLs) (Lawton) of daily life, ataxia severity scores (SARA), values of PEFr, MIP and respiratory complaints.

Results: Thirty-six individuals with SCA2 were evaluated, with a mean of 42.5 (± 12.4) years of age, of 7.6 (± 8.2) years of disease time and 9.9 (± 10.3) points in SARA. The lowest PEFr values correlated poorly and negatively with the duration of the disease duration (p = 0.021; r=-0,384). The lowest values of PEFr and MIP were related to the greater impairment of balance (p = 0.019, p = 0.045), greater dependence for ADLs (p = 0.006, p = 0.050) and IADLs (p = 0.003, p = 0.001). The highest severity of ataxia (p = 0.006, p = 0.017). Respiratory complaints were observed in 12 (33.3%) individuals, who were not related to disease time, balance, independence, the severity of ataxia or changes in PEFr and MIP values.

Conclusion: Respiratory dysfunction, even asymptomatic, is related to the impairment of balance, independence, and severity of ataxia in individuals with SCA2.

References: 1.Teive, HA; Ashizawa,T .Primary and secondary ataxias. Curr Opin Neurol 2015; 28 (4): 413-422 2. SriranjinI, SJ; Pal,PK; Krishna,N; Sathyaprabha,TN. Subclinical pulmonary disfunction in spinocerebellar ataxias 1,2,3. Acta Neurol Scand 2010:122:323-328.

To cite this abstract in AMA style:

N. Mello, M. Zonta, H. Teive, A. Meira, B. Zeigelboim. Respiratory Function and Functional Decline in Spinocerebellar Ataxia Type 2 [abstract]. Mov Disord. 2019; 34 (suppl 2). https://www.mdsabstracts.org/abstract/respiratory-function-and-functional-decline-in-spinocerebellar-ataxia-type-2/. Accessed May 25, 2025.
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