Abstract

BackgroundCamptocormia is common in patients with multiple system atrophy (MSA). The current study was aimed at assessing the frequency of camptocormia and its related factors in MSA patients with different disease durations. Also, the impact of camptocormia on disability was evaluated.MethodsA total of 716 patients were enrolled in the study. They were classified into three groups based on disease duration (≤ 3, 3–5, ≥ 5 years). Specific scales were used to evaluate the motor and non-motor symptoms. Disease severity was assessed using the Unified Multiple System Atrophy Rating Scale (UMSARS). The binary logistic regression model was used to explore the factors related to camptocormia. To analyze the impact of camptocormia on disability in patients with disease duration less than 5 years, propensity score matching (PSM) and stratified Cox regression analysis were used.ResultsIn the current study, we found that the frequency of camptocormia was 8.9, 19.7 and 19.2% when the disease duration was ≤3, 3–5, ≥ 5 years, respectively. In the disease duration ≤3 years group, we found that MSA-parkinsonian subtype (MSA-P) (OR = 2.043, P = 0.043), higher total UMSARS score (OR = 1.063, P < 0.001), older age of onset (OR = 1.047, P = 0.042), and lower score on the frontal assessment battery (FAB) (OR = 0.899, P = 0.046) were associated with camptocormia. Only greater disease severity was associated with camptocormia in the group of patients with disease duration 3–5 years (OR = 1.494, P = 0.025) and in the group of patients with disease duration ≥5 years (OR = 1.076, P = 0.005). There was no significant impact of camptocormia on disability in patients with a disease duration of < 5 years (HR = 0.687, P = 0.463).ConclusionThe frequency of camptocormia increased with prolonged disease duration. Disease severity was related to camptocormia at different stages of the disease. The MSA-P subtype, older age of onset, and lower FAB score were associated with camptocormia in the early stage of the disease.

Highlights

  • Camptocormia is an axial postural deformity characterized by abnormal thoracolumbar spinal flexion that occurs while standing or walking and abates or disappearsZhang et al BMC Neurology (2021) 21:181 was proposed as a supporting feature in the diagnosis of multiple system atrophy (MSA) [4]

  • In the present study, we aimed to explore the frequency of camptocormia, the potential factors related to camptocormia, and whether camptocormia is a predictor of disability in a large cohort of MSA patients with different disease durations wherein the motor and non-motor symptoms were comprehensively assessed by specific scales

  • Patients with camptocormia had a higher proportion of MSAparkinsonian subtype (MSA-P) subtype, older age, older age of onset, longer disease duration, higher proportion of symptom of onset with parkinsonism, lower score of frontal assessment battery (FAB), higher score of Hamilton Depression Rating Scale (HDRS), and higher levodopa equivalent daily dose (LEDD) than those without camptocormia

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Summary

Introduction

Camptocormia is an axial postural deformity characterized by abnormal thoracolumbar spinal flexion that occurs while standing or walking and abates or disappearsZhang et al BMC Neurology (2021) 21:181 was proposed as a supporting feature (red flag) in the diagnosis of MSA [4]. Considerably less attention has been accorded to the frequency or related factors of camptocormia in MSA. Ashour and Jankovic found camptocormia in 26.3% of the 19 patients with MSA. Koellensperger et al reported that the frequency of camptocormia was 32.1% in 74 patients with MSA-P [4]. We had found that the frequency of camptocormia was 15.6% in Chinese patients with MSA [10]. The varied frequency of camptocormia that has been reported across separate studies might be attributed to the difference in sample size and the heterogeneity in the enrolled patients with MSA. The current study was aimed at assessing the frequency of camptocormia and its related factors in MSA patients with different disease durations.

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