Midbrain atrophy in patients with presymptomatic progressive supranuclear palsy-Richardson's syndrome

Ahn, Jong Hyeon; Kim, Min-Kyeong; Kim, Ji Sun; Youn, Jinyoung; Jang, Wooyoung; Oh, Eungseok; Lee, Phil Hyu; Koh, Seong Beom; Ahn, Tae-Beom; Cho, Jin Whan

doi:10.1016/j.parkreldis.2019.07.009

Cited by 13 publications

(18 citation statements)

References 38 publications

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“…After the removal of two duplicates, the screening of the titles and abstracts of the 519 remaining articles was performed, and the following 485 articles were excluded: 46 reviews, 37 case reports, 172 conference abstracts, three editorial/chapter/note, 216 articles that were not in the field of interest, one article with a partially overlapping cohort and 10 articles for which the reconstruction of 2 × 2 tables was not possible. A total of 34 full-text articles were further assessed for eligibility, and the following 20 articles were excluded: 12 articles that did not differentiate PSP from PD [ 12 , 13 , 14 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 ], six articles that used the MRPI but differentiated between PSP and non-PSP [ 47 , 48 , 49 , 50 , 51 , 52 ], one article for which the reconstruction of a 2 × 2 table was not possible [ 53 ], and one article that was a meta-analysis [ 24 ]. Finally, 14 original articles involving 484 PSP patients and 1243 PD patients were included in our meta-analysis [ 15 , 16 , 17 , 18 , 19 , 20 , 21 , 23 , 54 , 55 , 56 , 57 , 58 , 59 ].…”

Section: Resultsmentioning

confidence: 99%

Diagnostic Performance of the Magnetic Resonance Parkinsonism Index in Differentiating Progressive Supranuclear Palsy from Parkinson’s Disease: An Updated Systematic Review and Meta-Analysis

et al. 2021

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Progressive supranuclear palsy (PSP) and Parkinson’s disease (PD) are difficult to differentiate especially in the early stages. We aimed to investigate the diagnostic performance of the magnetic resonance parkinsonism index (MRPI) in differentiating PSP from PD. A systematic literature search of PubMed-MEDLINE and EMBASE was performed to identify original articles evaluating the diagnostic performance of the MRPI in differentiating PSP from PD published up to 20 February 2021. The pooled sensitivity, specificity, and 95% CI were calculated using the bivariate random-effects model. The area under the curve (AUC) was calculated using a hierarchical summary receiver operating characteristic (HSROC) model. Meta-regression was performed to explain the effects of heterogeneity. A total of 14 original articles involving 484 PSP patients and 1243 PD patients were included. In all studies, T1-weighted images were used to calculate the MRPI. Among the 14 studies, nine studies used 3D T1-weighted images. The pooled sensitivity and specificity for the diagnostic performance of the MRPI in differentiating PSP from PD were 96% (95% CI, 87–99%) and 98% (95% CI, 91–100%), respectively. The area under the HSROC curve was 0.99 (95% CI, 0.98–1.00). Heterogeneity was present (sensitivity: I2 = 97.29%; specificity: I2 = 98.82%). Meta-regression showed the association of the magnet field strength with heterogeneity. Studies using 3 T MRI showed significantly higher sensitivity (100%) and specificity (100%) than those of studies using 1.5 T MRI (sensitivity of 98% and specificity of 97%) (p < 0.01). Thus, the MRPI could accurately differentiate PSP from PD and support the implementation of appropriate management strategies for patients with PSP.

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Section: Resultsmentioning

confidence: 99%

Diagnostic Performance of the Magnetic Resonance Parkinsonism Index in Differentiating Progressive Supranuclear Palsy from Parkinson’s Disease: An Updated Systematic Review and Meta-Analysis

et al. 2021

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“…With a mean DD of 63.1 months we investigated patients in rather progressed disease stages. This is particularly important considering that midbrain atrophy could also serve as PSP-specific preclinical marker in very early disease stages [28,29]. Thus, it remains to be studied, whether the relΔ t _MTPR-as indicator of midbrain atrophy rate-contributes better to diagnostic accuracy, earlier in the disease, i.e., when the overall MTPR has not yet reached PSP-specific values.…”

Section: Discussionmentioning

confidence: 99%

Investigating the 1-year decline in midbrain-to-pons ratio in the differential diagnosis of PSP and IPD

et al. 2020

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Background A reliable measure of PSP-specific midbrain atrophy, the midbrain-to-pons ratio (MTPR) has been reported to support the differential diagnosis of progressive supranuclear palsy (PSP) from idiopathic Parkinson’s disease (IPD). Since longitudinal analyses are lacking so far, the present study aimed to evaluate the diagnostic value of the relative change of MTPR (relΔt_MTPR) over a 1-year period in patients with PSP, IPD, and healthy controls (HC). Methods Midsagittal individual MRIs of patients with PSP (n = 15), IPD (n = 15), and healthy controls (HC; n = 15) were assessed and the MTPR at baseline and after 1 year were defined. The diagnostic accuracy of the MTPR and its relative change were evaluated using ROC curve analyses. Results PSP-patients had a significantly lower MTPR at baseline (M = 0.45 ± 0.06), compared to both non-PSP groups (F (2, 41) = 62.82, p < 0.001), with an overall predictive accuracy of 95.6% for an MTPR ≤ 0.54. PSP-patients also presented a significantly stronger 1-year decline in MTPR compared to IPD (p < 0.001). Though predictive accuracy of relΔt_MTPR for PSP (M = − 4.74% ± 4.48) from IPD (M = + 1.29 ± 3.77) was good (76.6%), ROC analysis did not reveal a significant improvement of diagnostic accuracy by combining the MTPR and relΔt_MTPR (p = 0.670). Still, specificity for PSP increased, though not significantly (p = 0.500). Conclusion The present results indicate that the relΔt_MTPR is a potentially useful tool to support the differential diagnosis of PSP from IPD. For its relative 1-year change, still, more evaluation is needed.

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“…IgLON5 antibody encephalitis), orbital and myogenic diseases, metabolic disorders (e.g. Wernicke encephalopathy, Wilsonʼs disease), genetic lysosomal storage diseases such as Niemann-Pick type C [8,16] and prion diseases [17]; the course in these cases is insidious [19]. On the other hand, onset is acute/subacute in cases of midbrain stroke or acute inflammatory diseases (pathogen-caused diseases, e.g.…”

Section: Syndromes and Diseases Of The Midbrainmentioning

confidence: 99%

“…in multiple sclerosis). Characteristic of PSP-RR [19], which often starts in the midbrain, is gaze palsy with vertical slow downward saccades combined with infrequent blinking and increased occurrence of square wave jerks; clinically, however, a gaze palsy upward and downward is frequent. Small saccades can show normal speed in PSP [20].…”

Section: Syndromes and Diseases Of The Midbrainmentioning

confidence: 99%

Central Ocular Motor Disorders: Clinical and Topographic Anatomical Diagnosis, Syndromes and Underlying Diseases

Strupp

Straumann

Helmchen

2021

Klin Monbl Augenheilkd

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The key to the diagnosis of ocular motor disorders is a systematic clinical examination of the different types of eye movements, including eye position, spontaneous nystagmus, range of eye movements, smooth pursuit, saccades, gaze-holding function, vergence, optokinetic nystagmus, as well as testing of the function of the vestibulo-ocular reflex (VOR) and visual fixation suppression of the VOR. This is like a window which allows you to look into the brain stem and cerebellum even if imaging is normal. Relevant anatomical structures are the midbrain, pons, medulla, cerebellum and rarely the cortex. There is a simple clinical rule: vertical and torsional eye movements are generated in the midbrain, horizontal eye movements in the pons. For example, isolated dysfunction of vertical eye movements is due to a midbrain lesion affecting the rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF), with impaired vertical saccades only or vertical gaze-evoked nystagmus due to dysfunction of the Interstitial nucleus of Cajal (INC). Lesions of the lateral medulla oblongata (Wallenberg syndrome) lead to typical findings: ocular tilt reaction, central fixation nystagmus and dysmetric saccades. The cerebellum is relevant for almost all types of eye movements; typical pathological findings are saccadic smooth pursuit, gaze-evoked nystagmus or dysmetric saccades. The time course of the development of symptoms and signs is important for the diagnosis of underlying diseases: acute: most likely stroke; subacute: inflammatory diseases, metabolic diseases like thiamine deficiencies; chronic progressive: inherited diseases like Niemann-Pick type C with typically initially vertical and then horizontal saccade palsy or degenerative diseases like progressive supranuclear palsy. Treatment depends on the underlying disease. In this article, we deal with central ocular motor disorders. In a second article, we focus on clinically relevant types of nystagmus such as downbeat, upbeat, fixation pendular, gaze-evoked, infantile or periodic alternating nystagmus. Therefore, these types of nystagmus will not be described here in detail.

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Midbrain atrophy in patients with presymptomatic progressive supranuclear palsy-Richardson's syndrome

Cited by 13 publications

References 38 publications

Diagnostic Performance of the Magnetic Resonance Parkinsonism Index in Differentiating Progressive Supranuclear Palsy from Parkinson’s Disease: An Updated Systematic Review and Meta-Analysis

Diagnostic Performance of the Magnetic Resonance Parkinsonism Index in Differentiating Progressive Supranuclear Palsy from Parkinson’s Disease: An Updated Systematic Review and Meta-Analysis

Investigating the 1-year decline in midbrain-to-pons ratio in the differential diagnosis of PSP and IPD

Central Ocular Motor Disorders: Clinical and Topographic Anatomical Diagnosis, Syndromes and Underlying Diseases

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