The role of substantia nigra sonography in the differentiation of Parkinson's disease and multiple system atrophy

Abstract

Background: The differential diagnosis of Parkinson's disease (PD) and multiple system atrophy (MSA) remains a challenge, especially in the early stage. Here, we assessed the value of transcranial sonography (TCS) to discriminate non-tremor dominant (non-TD) PD from MSA with predominant parkinsonism (MSA-P).

Methods: Eighty-six MSA-P patients and 147 age and gender-matched non-TD PD patients who had appropriate temporal acoustic bone windows were included in this study. All the patients were followed up for at least 2 years to confirm the initial diagnosis. Patients with at least one substantia nigra (SN) echogenic size ≥18 mm² were classified as hyperechogenic, those with at least one SN echogenic size ≥25 mm² was defined as markedly hyperechogenic.

Results: The frequency of SN hyperechogenicity in non-TD PD patients was significantly higher than that in MSA-P patients (74.1% vs. 38.4%, p < 0.001). SN hyperechogenicity discriminated non-TD PD from MSA-P with sensitivity of 74.1%, specificity of 61.6%, and positive predictive value of 76.8%. If marked SN hyperechogenicity was used as the cutoff value (≥ 25 mm²), the sensitivity decreased to 46.3%, but the specificity and positive predictive value increased to 80.2 and 80.0%. Additionally, in those patients with SN hyperechogenicity, positive correlation between SN hyperechogenicity area and disease duration was found in non-TD PD rather than in MSA-P patients. In this context, among early-stage patients with disease duration ≤3 years, the sensitivity, specificity and positive predictive value of SN hyperechogenicity further declined to 69.8%, 52.2%, and 66.7%, respectively.

Conclusions: TCS could help discriminate non-TD PD from MSA-P in a certain extent, but the limitation was also obvious with relatively low specificity, especially in the early stage.

Keywords: Atypical parkinsonian disorders; Disease duration; Multiple system atrophy; Parkinson’s disease; Substantia nigra; Transcranial sonography.

Figure 1

Percentage of patients with different ultrasound findings in the two groups. a showed the percentage of cases with different sonographic findings in total patients. b showed the percentage of cases with different sonographic findings in the subgroup of patients with disease duration ≤3 years. Black: percentage of patients with a marked substantia nigra (SN) hyperechogenicity; grey: percentage of patients who exhibited a moderate increase in SN echogenicity; and white: percentage of patients with a normal SN echogenicity; non-TD PD, non-tremor dominant Parkinson’s disease; MSA-P, parkinsonian variant of multiple system atrophy.

Figure 2

Correlation of SN_L area with disease duration in patients with SN hyperechogenicity