The Profile and Evolution of Neuropsychiatric Symptoms in Multiple System Atrophy: Self- and Caregiver Report
Abstract
Objective:
Methods:
Results:
Conclusions:
Methods
Participants
Baseline cohort (N=47) | 1-Year follow-up cohort (N=25) | |||||
---|---|---|---|---|---|---|
Characteristic | Mean | SD | Range | Mean | SD | Range |
Age (years) | 57.7 | 7.3 | 40–72 | 58.4 | 7.5 | 40–72 |
Education (years) | 11.1 | 3.2 | 4–16 | 11.4 | 3.4 | 4–16 |
Duration of the disease (years) | 3.3 | 1.7 | 1–8 | 3.5 | 1.8 | 1–8 |
Age at onset of the disease (years) | 54.4 | 7.5 | 35–69 | 54.8 | 7.9 | 35–69 |
Hoehn and Yahr stage (median) | 3 | 2–5 | 3 | 2–5 | ||
UMSARS | ||||||
Part I | 23.1 | 8.6 | 9–39 | 22.8 | 8.5 | 9–38 |
Part II | 24.8 | 7.4 | 10–43 | 24.9 | 7.8 | 10–43 |
Total (parts I and II) | 47.9 | 15.2 | 20–75 | 47.8 | 15.5 | 20–75 |
Part IV | 3.3 | 0.8 | 2–5 | 3.2 | 0.8 | 2–5 |
SCOPA-AUT total | 24.7 | 8.3 | 8–46 | 22.7 | 8.0 | 8–46 |
MMSE | 27.3 | 2.3 | 19–30 | 27.7 | 2.1 | 23–30 |
FAB total | 14.5 | 2.5 | 9–18 | 14.8 | 2.1 | 9–18 |
DRS | ||||||
Total | 127.5 | 8.4 | 107–141 | 131.1 | 5.6 | 117–141 |
Attention | 33. | 2.6 | 25–36 | 34.2 | 2.5 | 25–36 |
Initiation/Perseveration | 32.2 | 2.2 | 28–37 | 33.3 | 2.0 | 29–37 |
Construction | 4.9 | 1.0 | 3–6 | 5.3 | 0.8 | 3–6 |
Conceptualization | 34.3 | 2.9 | 27–39 | 35.1 | 2.3 | 30–39 |
Memory | 22.7 | 1.7 | 18–25 | 23.2 | 1.3 | 20–25 |
HAM-A | 11.3 | 6.1 | 2–24 | 11.0 | 6.3 | 2–24 |
HAM-D | 15.6 | 8.6 | 1–36 | 16.1 | 9.0 | 1–33 |
BDI | 18.0 | 9.4 | 2–38 | 17.3 | 8.4 | 2–31 |
AS | 16.7 | 8.5 | 4–40 | 19.0 | 9.1 | 5–40 |
Statistical Analysis
Results
Neuropsychiatric Inventory Scores
All patients | Patients with symptoms present (nonzero score) | Patients with scores ≥4 | ||||||
---|---|---|---|---|---|---|---|---|
Symptom | Mean | SD | Mean | SD | N | % | N | % |
Delusions | 0.19 | 1.31 | — | 1 | 2 | 1 | 2 | |
Hallucinations | 0.31 | 0.75 | 1.50 | 0.97 | 10 | 21 | 1 | 2 |
Agitation | 0.51 | 1.15 | 2.00 | 1.53 | 12 | 25 | 2 | 4 |
Depression | 4.92 | 3.91 | 5.37 | 3.77 | 43 | 92 | 28 | 60 |
Anxiety | 3.13 | 2.68 | 4.08 | 2.32 | 36 | 76 | 25 | 53 |
Euphoria | 0.02 | 0.15 | — | 1 | 2 | 0 | 0 | |
Apathy | 3.04 | 3.14 | 3.87 | 3.06 | 37 | 78 | 20 | 43 |
Disinhibition | 0.55 | 2.22 | 6.50 | 4.93 | 4 | 9 | 3 | 6 |
Irritability | 0.57 | 1.92 | 2.52 | 2.18 | 21 | 45 | 8 | 17 |
Aberrant motor behavior | 0.11 | 0.38 | 1.25 | 0.50 | 4 | 9 | 0 | 0 |
Neuropsychiatric Inventory | ||||||||
Sleep | 4.21 | 3.34 | 4.95 | 3.07 | 40 | 85 | 31 | 66 |
Appetite/eating | 1.38 | 2.46 | 4.33 | 2.50 | 15 | 32 | 12 | 26 |
Total | 19.28 | 14.39 | 20.59 | 13.92 | 44 | 94 | — | — |
Distress total score | 8.45 | 5.47 | 9.02 | 5.17 | 44 | 94 | — | — |
Severity and Frequency of NPSs in Terms of Age, Disease Duration, Motor Severity, and Frontal and Global Cognition Status
Age (years) | Disease duration (years) | UMSARS total score | FAB total score | DRS total score | ||||||
---|---|---|---|---|---|---|---|---|---|---|
NPI domain | <60 | ≥60 | ≤3 | >3 | <40 | ≥40 | ≤14 | >14 | ≤125 | >125 |
Delusions | ||||||||||
Mean | 0.00 | 0.60 | 0.00 | 0.43 | 0.00 | 0.27 | 0.50 | 0.00 | 0.60 | 0.00 |
SD | 0.00 | 2.32 | 0.00 | 1.96 | 0.00 | 1.57 | 2.12 | 0.00 | 2.32 | 0.00 |
Patients with scores 0–3 | 32 | 14 | 26 | 20 | 14 | 32 | 17 | 29 | 14 | 31 |
Patients with scores ≥4 | 0 | 1 | 0 | 1 | 0 | 1 | 1 | 0 | 1 | 0 |
Hallucinations | ||||||||||
Mean | 0.34 | 0.27 | 0.27 | 0.38 | 0.29 | 0.33 | 0.44 | 0.24 | 0.60 | 0.19 |
SD | 0.83 | 0.59 | 0.83 | 0.67 | 1.07 | 0.60 | 1.04 | 0.51 | 1.12 | 0.48 |
Patients with scores 0–3 | 31 | 14 | 25 | 20 | 13 | 32 | 17 | 28 | 14 | 30 |
Patients with scores ≥4 | 1 | 1 | 1 | 1 | 1 | 1 | 1 | 1 | 1 | 1 |
Agitation | ||||||||||
Mean | 0.31 | 0.93 | 0.31 | 0.76 | 0.00 | 0.73 | 0.72 | 0.38 | 0.47 | 0.55 |
SD | 0.64 | 1.79 | 0.68 | 1.55 | 0.00 | 1.33 | 1.67 | 0.67 | 1.06 | 1.23 |
Patients with scores 0–3 | 32 | 13 | 26 | 19 | 14 | 31 | 16 | 29 | 14 | 30 |
Patients with scores ≥4 | 0 | 2 | 0 | 2 | 0 | 2 | 2 | 0 | 1 | 1 |
Depression | ||||||||||
Mean | 4.75 | 5.27 | 4.35 | 5.62 | 3.07 | 5.70 | 5.06 | 4.83 | 5.27 | 4.90 |
SD | 3.65 | 4.54 | 3.51 | 4.34 | 3.63 | 3.81 | 4.63 | 3.48 | 4.73 | 3.49 |
Patients with scores 0–3 | 13 | 6 | 12 | 7 | 9 | 10 | 9 | 10 | 6 | 12 |
Patients with scores ≥4 | 19 | 9 | 14 | 14 | 5 | 23 | 9 | 19 | 9 | 19 |
Anxiety | ||||||||||
Mean | 3.38 | 2.60 | 3.77 | 2.33 | 3.00 | 3.18 | 2.56 | 3.48 | 1.87 | 3.77 |
SD | 2.86 | 2.23 | 2.92 | 2.15 | 3.16 | 2.49 | 2.60 | 2.71 | 1.85 | 2.85 |
Patients with scores 0–3 | 15 | 7 | 11 | 11 | 8 | 14 | 11 | 11 | 9 | 12 |
Patients with scores ≥4 | 17 | 8 | 15 | 10 | 6 | 19 | 7 | 18 | 6 | 19 |
Euphoria | ||||||||||
Mean | 0.00 | 0.07 | 0.04 | 0.00 | 0.00 | 0.03 | 0.00 | 0.03 | 0.07 | 0.00 |
SD | 0.00 | 0.26 | 0.10 | 0.00 | 0.00 | 0.17 | 0.00 | 0.19 | 0.26 | 0.00 |
Patients with scores 0–3 | 32 | 15 | 26 | 21 | 14 | 33 | 18 | 29 | 15 | 31 |
Patients with scores ≥4 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
Apathy | ||||||||||
Mean | 3.03 | 3.07 | 2.19 | 4.10 | 1.36 | 3.76 | 3.11 | 3.00 | 3.40 | 2.97 |
SD | 3.06 | 0.26 | 2.50 | 3.58 | 1.55 | 3.38 | 2.97 | 3.30 | 3.81 | 2.82 |
Patients with scores 0–3 | 19 | 8 | 18 | 9 | 11 | 16 | 9 | 18 | 8 | 18 |
Patients with scores ≥4 | 13 | 7 | 8 | 12 | 3 | 17 | 9 | 11 | 7 | 13 |
Disinhibition | ||||||||||
Mean | 0.00 | 1.73 | 0.50 | 0.62 | 0.00 | 0.79 | 0.72 | 0.45 | 0.33 | 0.68 |
SD | 0.00 | 3.75 | 2.35 | 2.11 | 0.00 | 2.63 | 2.27 | 2.23 | 1.05 | 2.65 |
Patients with scores 0–3 | 32 | 12 | 25 | 19 | 14 | 30 | 16 | 28 | 14 | 29 |
Patients with scores ≥4 | 0 | 3 | 1 | 2 | 0 | 3 | 2 | 1 | 1 | 2 |
Irritability | ||||||||||
Mean | 1.00 | 1.40 | 1.27 | 0.95 | 1.07 | 1.15 | 1.22 | 1.07 | 0.87 | 1.26 |
SD | 1.41 | 2.75 | 2.13 | 1.66 | 1.39 | 2.12 | 1.90 | 1.96 | 1.77 | 2.03 |
Patients with scores 0–3 | 27 | 13 | 21 | 19 | 12 | 28 | 15 | 25 | 14 | 25 |
Patients with scores ≥4 | 5 | 2 | 5 | 2 | 2 | 5 | 3 | 4 | 1 | 6 |
Aberrant motor behavior | ||||||||||
Mean | 0.13 | 0.07 | 0.08 | 0.27 | 0.00 | 0.15 | 0.11 | 0.10 | 0.20 | 0.06 |
SD | 0.42 | 0.26 | 0.14 | 0.48 | 0.00 | 0.04 | 0.32 | 0.41 | 0.56 | 0.25 |
Patients with scores 0–3 | 32 | 15 | 26 | 21 | 14 | 33 | 18 | 29 | 15 | 31 |
Patients with scores ≥4 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
NPI sleep | ||||||||||
Mean | 4.50 | 3.60 | 4.23 | 4.19 | 3.71 | 4.42 | 4.94 | 3.76 | 3.53 | 4.65 |
SD | 2.92 | 4.17 | 3.62 | 3.06 | 3.17 | 3.44 | 4.43 | 2.43 | 3.50 | 3.26 |
Patients with scores 0–3 | 8 | 8 | 11 | 5 | 6 | 10 | 6 | 10 | 6 | 9 |
Patients with scores ≥4 | 24 | 7 | 15 | 16 | 8 | 23 | 12 | 19 | 9 | 22 |
NPI appetite/eating | ||||||||||
Mean | 1.44 | 1.27 | 0.88 | 2.00 | 1.14 | 1.48 | 1.50 | 1.31 | 1.00 | 1.61 |
SD | 2.65 | 2.09 | 2.66 | 2.10 | 3.30 | 2.06 | 3.19 | 1.95 | 1.65 | 2.80 |
Patients with scores 0–3 | 24 | 11 | 23 | 12 | 12 | 23 | 14 | 21 | 12 | 22 |
Patients with scores ≥4 | 8 | 4 | 3 | 9 | 2 | 10 | 4 | 8 | 3 | 9 |
NPI total score | ||||||||||
Mean | 12.81 | 15.93 | 12.69 | 15.19 | 8.79 | 15.94 | 14.50 | 13.38 | 13.47 | 14.32 |
SD | 7.94 | 16.28 | 9.67 | 12.95 | 6.66 | 12.10 | 13.85 | 9.43 | 13.44 | 10.18 |
Mild (0–20) | 24 | 11 | 20 | 15 | 13 | 22 | 11 | 24 | 10 | 24 |
Moderate (20–40) | 8 | 4 | 6 | 6 | 1 | 11 | 7 | 5 | 5 | 7 |
NPI distress total | ||||||||||
Mean | 8.28 | 8.80 | 7.77 | 9.29 | 5.36 | 9.76 | 8.33 | 8.52 | 8.27 | 8.74 |
SD | 4.69 | 7.03 | 4.76 | 6.26 | 3.41 | 5.69 | 6.14 | 5.13 | 6.14 | 5.18 |
Disease-Related Factors Associated With NPI Total Score
Multivariate regression analysis | |||
---|---|---|---|
Variables | β | SE | p |
UMSARS, part IV | 6.644 | 3.169 | 0.044 |
MMSE | –2.140 | 0.744 | 0.010 |
Measuring Depression, Apathy, and Anxiety by NPI Compared With Psychiatric Tools Based on Patient Assessment
Evolution of Clinical Variables Over 1-Year Follow-Up
Baseline | Follow-up visit | |||||
---|---|---|---|---|---|---|
Variable | Mean | SD | Mean | SD | Effect size | 95% CI |
Hoehn and Yahr stage | 3.3 | 1.0 | 3.8 | 0.8 | 0.82** | 0.41, 1.23 |
UMSARS | ||||||
Part I | 22.8 | 8.5 | 28.5 | 9.5 | 1.07** | 0.67, 1.49 |
Part II | 24.9 | 7.8 | 28.8 | 7.6 | 0.97** | 0.56, 1.38 |
Part IV | 3.2 | 0.8 | 3.8 | 0.7 | 1.00** | 0.60, 1.40 |
SCOPA-AUT total | 22.7 | 8.0 | 31.7 | 9.2 | 1.60 | 0.94, 2.26 |
MMSE | 27.7 | 2.1 | 27.4 | 1.6 | –0.20 | –0.62, –0.22 |
FAB total | 14.8 | 2.1 | 15.5 | 3.2 | –0.12 | –0.54, 0.30 |
DRS | ||||||
Total | 131.1 | 5.6 | 126.9 | 8.0 | –0.70** | –1.14, –0.27 |
Attention | 34.2 | 2.6 | 33.3 | 2.4 | –0.57** | –1.01, –0.13 |
Initiation/Perseveration | 33.2 | 2.3 | 32.3 | 2.8 | –0.31 | –0.73, 0.12 |
Construction | 4.1 | 1.6 | 2.6 | 1.8 | –0.49* | –0.91, –0.07 |
Conceptualization | 35.0 | 2.4 | 34.0 | 2.8 | –0.32 | –0.75, 0.11 |
Memory | 23.8 | 1.4 | 22.3 | 2.4 | –0.49* | –0.91, –0.06 |
NPI total score | 18.9 | 11.5 | 19.5 | 13.9 | 0.06 | –0.52, 0.26 |
HAM-A | 11.0 | 6.3 | 12.0 | 4.3 | 0.21 | –0.20, 0.62 |
HAM-D | 16.1 | 9.0 | 17.1 | 7.8 | 0.17 | –0.24, 0.58 |
BDI | 17.3 | 8.4 | 18.7 | 8.3 | 0.28 | –0.13, 0.69 |
AS | 18.9 | 9.2 | 18.8 | 9.6 | –0.01 | –0.43, 0.40 |
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