Language Disorder in Progressive Supranuclear Palsy and Corticobasal Syndrome: Neural Correlates and Detection by the MLSE Screening Tool

Background: Progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) affect speech and language as well as motor functions. Clinical and neuropathological data indicate a close relationship between these two disorders and the non-fluent variant of primary progressive aphasia (nfvPPA). We use the recently developed Mini Linguistic State Examination tool (MLSE) to study speech and language disorders in patients with PSP, CBS, and nfvPPA, in combination with structural magnetic resonance imaging (MRI).Methods: Fifty-one patients (PSP N = 13, CBS N = 19, nfvPPA N = 19) and 30 age-matched controls completed the MLSE, the short form of the Boston Diagnostic Aphasia Examination (BDAE), and the Addenbrooke’s Cognitive Examination III. Thirty-eight patients and all controls underwent structural MRI at 3 Tesla, with T1 and T2-weighted images processed by surface-based and subcortical segmentation within FreeSurfer 6.0.0 to extract cortical thickness and subcortical volumes. Morphometric differences were compared between groups and correlated with the severity of speech and language impairment.Results: CBS and PSP patients showed impaired MLSE performance, compared to controls, with a similar language profile to nfvPPA, albeit less severe. All patient groups showed reduced cortical thickness in bilateral frontal regions and striatal volume. PSP and nfvPPA patients also showed reduced superior temporal cortical thickness, with additional thalamic and amygdalo-hippocampal volume reductions in nfvPPA. Multivariate analysis of brain-wide cortical thickness and subcortical volumes with MLSE domain scores revealed associations between performance on multiple speech and language domains with atrophy of left-lateralised fronto-temporal cortex, amygdala, hippocampus, putamen, and caudate.Conclusions: The effect of PSP and CBS on speech and language overlaps with nfvPPA. These three disorders cause a common anatomical pattern of atrophy in the left frontotemporal language network and striatum. The MLSE is a short clinical screening tool that can identify the language disorder of PSP and CBS, facilitating clinical management and patient access to future clinical trials.

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Language disorder in progressive supranuclear palsy and corticobasal syndrome: neural correlates and detection by the MLSE screening tool

10.1101/2021.03.04.21252852 ◽

2021 ◽

Author(s):

Katie A. Peterson ◽

P. Simon Jones ◽

Nikil Patel ◽

Kamen A. Tsvetanov ◽

Ruth Ingram ◽

...

Keyword(s):

Progressive Supranuclear Palsy ◽

Cortical Thickness ◽

Screening Tool ◽

Language Disorder ◽

Short Form ◽

Temporal Cortex ◽

Primary Progressive Aphasia ◽

Corticobasal Syndrome ◽

Speech And Language ◽

Subcortical Volumes

AbstractBackgroundProgressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) affect speech and language as well as motor functions. Clinical and neuropathological data indicate a close relationship between these two disorders and the non-fluent variant of primary progressive aphasia (nfvPPA). We use the recently developed Mini Linguistic State Examination tool (MLSE) to study speech and language disorders in patients with PSP, CBS, and nfvPPA, in combination with structural magnetic resonance imaging (MRI).MethodsFifty-one patients (PSP N = 13, CBS N = 19, nfvPPA N = 19) and 30 age-matched controls completed the MLSE, the short form of the Boston Diagnostic Aphasia Examination (BDAE), and the Addenbrooke’s Cognitive Examination III. Thirty-eight patients and all controls underwent structural MRI at 3 Tesla, with T1 and T2-weighted images processed by surface-based and subcortical segmentation within FreeSurfer 6.0.0 to extract cortical thickness and subcortical volumes. Morphometric differences were compared between groups and correlated with severity of speech and language impairment.ResultsCBS and PSP patients showed impaired MLSE performance, compared to controls, with a similar language profile to nfvPPA, albeit less severe. All patient groups showed reduced cortical thickness in bilateral frontal regions and striatal volume. PSP and nfvPPA patients also showed reduced superior temporal cortical thickness, with additional thalamic and amygdalo-hippocampal volume reductions in nfvPPA. Multivariate analysis of brain-wide cortical thickness and subcortical volumes with MLSE domain scores revealed associations between performance on multiple speech and language domains with atrophy of left-lateralised fronto-temporal cortex, amygdala, hippocampus, putamen and caudate.ConclusionsThe effect of PSP and CBS on speech and language overlaps with nfvPPA. These three disorders cause a common anatomical pattern of atrophy in the left frontotemporal language network and striatum. The MLSE is a short clinical screening tool that can identify the language disorder of PSP and CBS, facilitating clinical management and patient access to future clinical trials.

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Cortical thickness, cortical surface area and subcortical volumes in schizophrenia and bipolar disorder patients with cannabis use

10.26226/morressier.5971be84d462b80290b52671 ◽

2017 ◽

Author(s):

Cecilie Bhandari Hartberg

Keyword(s):

Bipolar Disorder ◽

Surface Area ◽

Cortical Thickness ◽

Cannabis Use ◽

Cortical Surface ◽

Cortical Surface Area ◽

Subcortical Volumes

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Infratentorial pathology in frontotemporal dementia: cerebellar grey and white matter alterations in FTD phenotypes

Journal of Neurology ◽

10.1007/s00415-021-10575-w ◽

2021 ◽

Author(s):

Mary Clare McKenna ◽

Rangariroyashe H. Chipika ◽

Stacey Li Hi Shing ◽

Foteini Christidi ◽

Jasmin Lope ◽

...

Keyword(s):

White Matter ◽

Fractional Anisotropy ◽

Cortical Thickness ◽

Cerebellar Atrophy ◽

Primary Progressive Aphasia ◽

Radial Diffusivity ◽

Axial Diffusivity ◽

Progressive Aphasia ◽

Primary Progressive ◽

Crus I

AbstractThe contribution of cerebellar pathology to cognitive and behavioural manifestations is increasingly recognised, but the cerebellar profiles of FTD phenotypes are relatively poorly characterised. A prospective, single-centre imaging study has been undertaken with a high-resolution structural and diffusion tensor protocol to systematically evaluate cerebellar grey and white matter alterations in behavioural-variant FTD(bvFTD), non-fluent variant primary progressive aphasia(nfvPPA), semantic-variant primary progressive aphasia(svPPA), C9orf72-positive ALS-FTD(C9 + ALSFTD) and C9orf72-negative ALS-FTD(C9-ALSFTD). Cerebellar cortical thickness and complementary morphometric analyses were carried out to appraise atrophy patterns controlling for demographic variables. White matter integrity was assessed in a study-specific white matter skeleton, evaluating three diffusivity metrics: fractional anisotropy (FA), axial diffusivity (AD) and radial diffusivity (RD). Significant cortical thickness reductions were identified in: lobule VII and crus I in bvFTD; lobule VI VII, crus I and II in nfvPPA; and lobule VII, crus I and II in svPPA; lobule IV, VI, VII and Crus I and II in C9 + ALSFTD. Morphometry revealed volume reductions in lobule V in all groups; in addition to lobule VIII in C9 + ALSFTD; lobule VI, VIII and vermis in C9-ALSFTD; lobule V, VII and vermis in bvFTD; and lobule V, VI, VIII and vermis in nfvPPA. Widespread white matter alterations were demonstrated by significant fractional anisotropy, axial diffusivity and radial diffusivity changes in each FTD phenotype that were more focal in those with C9 + ALSFTD and svPPA. Our findings indicate that FTD subtypes are associated with phenotype-specific cerebellar signatures with the selective involvement of specific lobules instead of global cerebellar atrophy.

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Characteristics of progressive supranuclear palsy presenting with corticobasal syndrome: a cortical variant

Neuropathology and Applied Neurobiology ◽

10.1111/nan.12037 ◽

2014 ◽

Vol 40 (2) ◽

pp. 149-163 ◽

Cited By ~ 41

Author(s):

H. Ling ◽

R. Silva ◽

L. A. Massey ◽

R. Courtney ◽

G. Hondhamuni ◽

...

Keyword(s):

Progressive Supranuclear Palsy ◽

Corticobasal Syndrome

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Development and validation of a PSP questionnaire as screening tool for early diagnosis of progressive supranuclear palsy (PSP)

Parkinsonism & Related Disorders ◽

10.1016/j.parkreldis.2020.06.358 ◽

2020 ◽

Vol 79 ◽

pp. e98-e99

Author(s):

E. Höller ◽

G. Höglinger ◽

G. Respondek

Keyword(s):

Early Diagnosis ◽

Progressive Supranuclear Palsy ◽

Screening Tool ◽

Development And Validation

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Word comprehension in temporal cortex and Wernicke area

Neurology ◽

10.1212/wnl.0000000000006788 ◽

2018 ◽

Vol 92 (3) ◽

pp. e224-e233 ◽

Cited By ~ 6

Author(s):

M.-Marsel Mesulam ◽

Benjamin M. Rader ◽

Jaiashre Sridhar ◽

Matthew J. Nelson ◽

Jungmoon Hyun ◽

...

Keyword(s):

Temporal Lobe ◽

Sentence Comprehension ◽

Single Case ◽

Temporal Cortex ◽

Primary Progressive Aphasia ◽

Single Word ◽

Word Comprehension ◽

Cerebrovascular Lesions ◽

Core Components

ObjectiveTo explore atrophy–deficit correlations of word comprehension and repetition in temporoparietal cortices encompassing the Wernicke area, based on patients with primary progressive aphasia (PPA).MethodsCortical thickness in regions within and outside the classical Wernicke area, measured by FreeSurfer, was correlated with repetition and single word comprehension scores in 73 right-handed patients at mild to moderate stages of PPA.ResultsAtrophy in the Wernicke area was correlated with repetition (r = 0.42, p = 0.001) but not single word comprehension (r = −0.072, p = 0.553). Correlations with word comprehension were confined to more anterior parts of the temporal lobe, especially its anterior third (r = 0.60, p < 0.001). A single case with postmortem autopsy illustrated preservation of word comprehension but not repetition 6 months prior to death despite nearly 50% loss of cortical volume and severe neurofibrillary degeneration in core components of the Wernicke area.ConclusionsTemporoparietal cortices containing the Wernicke area are critical for language repetition. Contrary to the formulations of classic aphasiology, their role in word and sentence comprehension is ancillary rather than critical. Thus, the Wernicke area is not sufficient to sustain word comprehension if the anterior temporal lobe is damaged. Traditional models of the role of the Wernicke area in comprehension are based almost entirely on patients with cerebrovascular lesions. Such lesions also cause deep white matter destruction and acute network diaschisis, whereas progressive neurodegenerative diseases associated with PPA do not. Conceptualizations of the Wernicke area that appear to conflict, therefore, can be reconciled by considering the hodologic and physiologic differences of the underlying lesions.

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17q23.2q23.3 de novo duplication in association with speech and language disorder, learning difficulties, incoordination, motor skill impairment, and behavioral disturbances: a case report

BMC Medical Genetics ◽

10.1186/s12881-017-0479-3 ◽

2017 ◽

Vol 18 (1) ◽

Cited By ~ 1

Author(s):

Karen Wessel ◽

Jehan Suleiman ◽

Tamam E. Khalaf ◽

Shivendra Kishore ◽

Arndt Rolfs ◽

...

Keyword(s):

Case Report ◽

Motor Skill ◽

De Novo ◽

Language Disorder ◽

Learning Difficulties ◽

Speech And Language ◽

Behavioral Disturbances

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Hostility, Impulsivity, and Behavior Inhibition Among Women with PMDD

CNS Spectrums ◽

10.1017/s1092852912000387 ◽

2011 ◽

Vol 16 (9) ◽

pp. 205-213 ◽

Cited By ~ 5

Author(s):

Ju-Yu Yen ◽

Cheng-Chung Chen ◽

Shun-Jen Chang ◽

Chih-Hung Ko ◽

Cheng-Sheng Chen ◽

...

Keyword(s):

Functional Impairment ◽

Behavioral Inhibition ◽

Screening Tool ◽

Short Form ◽

Premenstrual Dysphoric Disorder ◽

Control Group ◽

Dysfunctional Impulsivity ◽

Behavior Inhibition System ◽

And Behavior ◽

Behavior Inhibition

AbstractIntroductionThis study aimed to compare hostility, impulsivity, and behavior inhibition between women with and without premenstrual dysphoric disorder (PMDD) during both luteal and follicular phases and to examine whether these variables contribute to irritability and daily functional impairment of PMDD.MethodsPMDD was screened via the Premenstrual Symptoms Screening Tool. A diagnosis of PMDD was confirmed by psychiatric interviewing without 2-month prospective confirmation. Sixty women in the PMDD group and 60 women in the control group completed the Chinese Version of the Buss-Durkee Hostility Inventory-Short Form, Dickman's Impulsivity Inventory, and the Behavior Inhibition System Scale during the luteal and follicular phases.ResultsWe found that the PMDD group had significantly higher levels of hostility, dysfunctional impulsivity, and behavioral inhibition than the control group in both luteal and follicular phases. The PMDD group also had more premenstrual aggravation on total hostility, hostility affect, and suppressive hostility than the control group. Higher hostility, dysfunctional impulsivity, and behavior inhibition were associated with more severe irritability and functional impairment of PMDD.DiscussionHostility, impulsivity, and behavior inhibition might contribute to irritability and functional impairment in women with PMDD.ConclusionAssessment and interventions based on these factors should be provided for women with PMDD, especially in the luteal phase.

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