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Francesca Caso - One of the best experts on this subject based on the ideXlab platform.

  • clinical and mri correlates of disease progression in a case of nonfluent agrammatic variant of primary progressive aphasia due to progranulin grn cys157lysfsx97 mutation
    Journal of the Neurological Sciences, 2014
    Co-Authors: Francesca Caso, Giancarlo Comi, Giuseppe Magnani, F. Agosta, S. Galantucci, E.g. Spinelli, D. Galimberti, A. Falini, Massimo Filippi
    Abstract:

    Abstract Little is known about the longitudinal changes of brain damage in patients with sporadic nonfluent/agrammatic variant of primary progressive aphasia (nfvPPA) and in progranulin (GRN) mutation carriers. This study reports the clinical and MRI longitudinal data of a patient with nfvPPA carrying GRN Cys157LysfsX97 mutation (GRN +). Voxel-based morphometry, tensor-based morphometry and diffusion tensor MRI were applied to evaluate gray matter (GM) and white matter (WM) changes over three years. The prominent clinical feature was motor speech impairment associated with only mild Agrammatism. MRI demonstrated a progressive and severe GM atrophy of inferior fronto–insular–temporo–parietal regions with focal damage to frontotemporal and frontoparietal WM connections. This is the first report of longitudinal MRI data in a nfvPPA- GRN + patient and this report offers new insights into the pathophysiology of the disease.

  • in vivo signatures of nonfluent agrammatic primary progressive aphasia caused by ftld pathology
    Neurology, 2014
    Co-Authors: Francesca Caso, Maya L Henry, Benno Gesierich, Maria Luisa Mandelli, Brianne M Bettcher, Jennifer M Ogar, Massimo Filippi, Giancarlo Comi, Giuseppe Magnani
    Abstract:

    Objective: To identify early cognitive and neuroimaging features of sporadic nonfluent/agrammatic variant of primary progressive aphasia (nfvPPA) caused by frontotemporal lobar degeneration (FTLD) subtypes. Methods: We prospectively collected clinical, neuroimaging, and neuropathologic data in 11 patients with sporadic nfvPPA with FTLD-tau (nfvPPA-tau, n = 9) or FTLD–transactive response DNA binding protein pathology of 43 kD type A (nfvPPA-TDP, n = 2). We analyzed patterns of cognitive and gray matter (GM) and white matter (WM) atrophy at presentation in the whole group and in each pathologic subtype separately. We also considered longitudinal clinical data. Results: At first evaluation, regardless of pathologic FTLD subtype, apraxia of speech (AOS) was the most common cognitive feature and atrophy involved the left posterior frontal lobe. Each pathologic subtype showed few distinctive features. At presentation, patients with nfvPPA-tau presented with mild to moderate AOS, mixed dysarthria with prominent hypokinetic features, clear Agrammatism, and atrophy in the GM of the left posterior frontal regions and in left frontal WM. While speech and language deficits were prominent early, within 3 years of symptom onset, all patients with nfvPPA-tau developed significant extrapyramidal motor signs. At presentation, patients with nfvPPA-TDP had severe AOS, dysarthria with spastic features, mild Agrammatism, and atrophy in left posterior frontal GM only. Selective mutism occurred early, when general neurologic examination only showed mild decrease in finger dexterity in the right hand. Conclusions: Clinical features in sporadic nfvPPA caused by FTLD subtypes relate to neurodegeneration of GM and WM in frontal motor speech and language networks. We propose that early WM atrophy in nfvPPA is suggestive of FTLD-tau pathology while early selective GM loss might be indicative of FTLD-TDP.

  • clinical neuroimaging and pathologic features in a group of nonfluent variant primary progressive aphasia nfvppa studied prospectively p07 172
    Neurology, 2012
    Co-Authors: Francesca Caso, Maya L Henry, Benno Gesierich, Stephen M Wilson, Nina F Dronkers, Bruce L Miller, William W Seeley, Maria Luisa Gornotempini
    Abstract:

    Objective: To examine the relationship between clinical, neuroimaging, and pathologic features in nonfluent variant primary progressive aphasia (nfvPPA). Background Pathologic diagnosis in nfvPPA has been associated predominantly with FTLD-tau pathology but TDP-43 pathology has also been reported.It has been proposed that nfvPPA cases with tau and TDP-43 show distinct clinical features that may assist in predicting pathology. Specifically,Agrammatism has been linked with TDP-43 whereas motor speech involvement has been associated with tau. Design/Methods: Here we present detailed, prospective longitudinal data for 9 nfvPPA cases. Patients were followed clinically for an average of 4.5 years until autopsy. Voxel-based morphometry(VBM)was used to assess regional gray and white matter atrophy in relation to a group of 140 healthy control subjects. Results: Autopsy revealed two patients with TDP-43 pathology (22%) and remaining cases with Tau pathology (78%), (4 cortico-basal degeneration, 2 progressive supranuclear palsy, 1 Pick9s disease). At first evaluation, both TDP-43 cases were functionally mute, but writing samples did not reveal frank Agrammatism. Tau patients all showed apraxia of speech. Mild Agrammatism was present in six tau cases and dysarthria in four.Both TDP-43 and four Tau patients developed a mild extrapyramidal syndrome whereas dramatic motor involvement was observed in three Tau cases(2 PSP and 1 CBD.VBM results for the entire group of nfvPPA patients revealed prevalent involvement of the left hemisphere, including premotor cortex, rolandic operculum, and insula.Tau patients showed greater lateralization relative to the TDP43 cases, which showed additional involvement of the right rolandic operculum. In patients with tau pathology, grey matter loss was also observed in subcortical structures, including the left thalamus and caudate.Finally, tau patients showed extended cortico-subcortical white matter damage compared to TDP-43 patients. Conclusions: NfvPPA with TDP-43 or Tau pathology have clinical and neuroimaging commonalities.However,they also show distinctive features that,if confirmed in a larger cohort,may be helpful in-vivo prediction of pathology. Supported by: R01 NS050915,NIA P50 AG03006,NIA P01 AG019724;DHS04-35516;03-75271 DHS/AD/ARCC;Larry L. Hillblom Foundation;John Douglas French Alzheimer9s Foundation; Koret Family Foundation;McBean Family Foundation. Disclosure: Dr. Caso has nothing to disclose. Dr. Henry has nothing to disclose. Dr. Gesierich has nothing to disclose. Dr. Wilson has nothing to disclose. Dr. Dronkers has nothing to disclose. Dr. Miller has received personal compensation for activities with Allon Therapeutics, Inc. and TauRx Therapeutics, Ltd. Dr. Miller has received research support from Novartis. Dr. Seeley has received personal compensation for activities with Korea Novartis. Dr. Gorno Tempini has nothing to disclose.

Massimo Filippi - One of the best experts on this subject based on the ideXlab platform.

  • clinical and mri correlates of disease progression in a case of nonfluent agrammatic variant of primary progressive aphasia due to progranulin grn cys157lysfsx97 mutation
    Journal of the Neurological Sciences, 2014
    Co-Authors: Francesca Caso, Giancarlo Comi, Giuseppe Magnani, F. Agosta, S. Galantucci, E.g. Spinelli, D. Galimberti, A. Falini, Massimo Filippi
    Abstract:

    Abstract Little is known about the longitudinal changes of brain damage in patients with sporadic nonfluent/agrammatic variant of primary progressive aphasia (nfvPPA) and in progranulin (GRN) mutation carriers. This study reports the clinical and MRI longitudinal data of a patient with nfvPPA carrying GRN Cys157LysfsX97 mutation (GRN +). Voxel-based morphometry, tensor-based morphometry and diffusion tensor MRI were applied to evaluate gray matter (GM) and white matter (WM) changes over three years. The prominent clinical feature was motor speech impairment associated with only mild Agrammatism. MRI demonstrated a progressive and severe GM atrophy of inferior fronto–insular–temporo–parietal regions with focal damage to frontotemporal and frontoparietal WM connections. This is the first report of longitudinal MRI data in a nfvPPA- GRN + patient and this report offers new insights into the pathophysiology of the disease.

  • in vivo signatures of nonfluent agrammatic primary progressive aphasia caused by ftld pathology
    Neurology, 2014
    Co-Authors: Francesca Caso, Maya L Henry, Benno Gesierich, Maria Luisa Mandelli, Brianne M Bettcher, Jennifer M Ogar, Massimo Filippi, Giancarlo Comi, Giuseppe Magnani
    Abstract:

    Objective: To identify early cognitive and neuroimaging features of sporadic nonfluent/agrammatic variant of primary progressive aphasia (nfvPPA) caused by frontotemporal lobar degeneration (FTLD) subtypes. Methods: We prospectively collected clinical, neuroimaging, and neuropathologic data in 11 patients with sporadic nfvPPA with FTLD-tau (nfvPPA-tau, n = 9) or FTLD–transactive response DNA binding protein pathology of 43 kD type A (nfvPPA-TDP, n = 2). We analyzed patterns of cognitive and gray matter (GM) and white matter (WM) atrophy at presentation in the whole group and in each pathologic subtype separately. We also considered longitudinal clinical data. Results: At first evaluation, regardless of pathologic FTLD subtype, apraxia of speech (AOS) was the most common cognitive feature and atrophy involved the left posterior frontal lobe. Each pathologic subtype showed few distinctive features. At presentation, patients with nfvPPA-tau presented with mild to moderate AOS, mixed dysarthria with prominent hypokinetic features, clear Agrammatism, and atrophy in the GM of the left posterior frontal regions and in left frontal WM. While speech and language deficits were prominent early, within 3 years of symptom onset, all patients with nfvPPA-tau developed significant extrapyramidal motor signs. At presentation, patients with nfvPPA-TDP had severe AOS, dysarthria with spastic features, mild Agrammatism, and atrophy in left posterior frontal GM only. Selective mutism occurred early, when general neurologic examination only showed mild decrease in finger dexterity in the right hand. Conclusions: Clinical features in sporadic nfvPPA caused by FTLD subtypes relate to neurodegeneration of GM and WM in frontal motor speech and language networks. We propose that early WM atrophy in nfvPPA is suggestive of FTLD-tau pathology while early selective GM loss might be indicative of FTLD-TDP.

  • Clinical and MRI correlates of disease progression in a case of nonfluent/agrammatic variant of primary progressive aphasia due to progranulin (GRN) Cys157LysfsX97 mutation
    'Elsevier BV', 2014
    Co-Authors: F. Caso, Giancarlo Comi, Giuseppe Magnani, F. Agosta, S. Galantucci, E.g. Spinelli, D. Galimberti, A. Falini, Massimo Filippi
    Abstract:

    Little is known about the longitudinal changes of brain damage in patients with sporadic nonfluent/agrammatic variant of primary progressive aphasia (nfvPPA) and in progranulin (GRN) mutation carriers. This study reports the clinical and MRI longitudinal data of a patient with nfvPPA carrying GRN Cys157LysfsX97 mutation (GRN +). Voxel-based morphometry, tensor-based morphometry and diffusion tensor MRI were applied to evaluate gray matter (GM) and white matter (WM) changes over three years. The prominent clinical feature was motor speech impairment associated with only mild Agrammatism. MRI demonstrated a progressive and severe GM atrophy of inferior fronto-insular-temporo-parietal regions with focal damage to frontotemporal and frontoparietal WM connections. This is the first report of longitudinal MRI data in a nfvPPA- GRN + patient and this report offers new insights into the pathophysiology of the disease

Giuseppe Magnani - One of the best experts on this subject based on the ideXlab platform.

  • clinical and mri correlates of disease progression in a case of nonfluent agrammatic variant of primary progressive aphasia due to progranulin grn cys157lysfsx97 mutation
    Journal of the Neurological Sciences, 2014
    Co-Authors: Francesca Caso, Giancarlo Comi, Giuseppe Magnani, F. Agosta, S. Galantucci, E.g. Spinelli, D. Galimberti, A. Falini, Massimo Filippi
    Abstract:

    Abstract Little is known about the longitudinal changes of brain damage in patients with sporadic nonfluent/agrammatic variant of primary progressive aphasia (nfvPPA) and in progranulin (GRN) mutation carriers. This study reports the clinical and MRI longitudinal data of a patient with nfvPPA carrying GRN Cys157LysfsX97 mutation (GRN +). Voxel-based morphometry, tensor-based morphometry and diffusion tensor MRI were applied to evaluate gray matter (GM) and white matter (WM) changes over three years. The prominent clinical feature was motor speech impairment associated with only mild Agrammatism. MRI demonstrated a progressive and severe GM atrophy of inferior fronto–insular–temporo–parietal regions with focal damage to frontotemporal and frontoparietal WM connections. This is the first report of longitudinal MRI data in a nfvPPA- GRN + patient and this report offers new insights into the pathophysiology of the disease.

  • in vivo signatures of nonfluent agrammatic primary progressive aphasia caused by ftld pathology
    Neurology, 2014
    Co-Authors: Francesca Caso, Maya L Henry, Benno Gesierich, Maria Luisa Mandelli, Brianne M Bettcher, Jennifer M Ogar, Massimo Filippi, Giancarlo Comi, Giuseppe Magnani
    Abstract:

    Objective: To identify early cognitive and neuroimaging features of sporadic nonfluent/agrammatic variant of primary progressive aphasia (nfvPPA) caused by frontotemporal lobar degeneration (FTLD) subtypes. Methods: We prospectively collected clinical, neuroimaging, and neuropathologic data in 11 patients with sporadic nfvPPA with FTLD-tau (nfvPPA-tau, n = 9) or FTLD–transactive response DNA binding protein pathology of 43 kD type A (nfvPPA-TDP, n = 2). We analyzed patterns of cognitive and gray matter (GM) and white matter (WM) atrophy at presentation in the whole group and in each pathologic subtype separately. We also considered longitudinal clinical data. Results: At first evaluation, regardless of pathologic FTLD subtype, apraxia of speech (AOS) was the most common cognitive feature and atrophy involved the left posterior frontal lobe. Each pathologic subtype showed few distinctive features. At presentation, patients with nfvPPA-tau presented with mild to moderate AOS, mixed dysarthria with prominent hypokinetic features, clear Agrammatism, and atrophy in the GM of the left posterior frontal regions and in left frontal WM. While speech and language deficits were prominent early, within 3 years of symptom onset, all patients with nfvPPA-tau developed significant extrapyramidal motor signs. At presentation, patients with nfvPPA-TDP had severe AOS, dysarthria with spastic features, mild Agrammatism, and atrophy in left posterior frontal GM only. Selective mutism occurred early, when general neurologic examination only showed mild decrease in finger dexterity in the right hand. Conclusions: Clinical features in sporadic nfvPPA caused by FTLD subtypes relate to neurodegeneration of GM and WM in frontal motor speech and language networks. We propose that early WM atrophy in nfvPPA is suggestive of FTLD-tau pathology while early selective GM loss might be indicative of FTLD-TDP.

  • Clinical and MRI correlates of disease progression in a case of nonfluent/agrammatic variant of primary progressive aphasia due to progranulin (GRN) Cys157LysfsX97 mutation
    'Elsevier BV', 2014
    Co-Authors: F. Caso, Giancarlo Comi, Giuseppe Magnani, F. Agosta, S. Galantucci, E.g. Spinelli, D. Galimberti, A. Falini, Massimo Filippi
    Abstract:

    Little is known about the longitudinal changes of brain damage in patients with sporadic nonfluent/agrammatic variant of primary progressive aphasia (nfvPPA) and in progranulin (GRN) mutation carriers. This study reports the clinical and MRI longitudinal data of a patient with nfvPPA carrying GRN Cys157LysfsX97 mutation (GRN +). Voxel-based morphometry, tensor-based morphometry and diffusion tensor MRI were applied to evaluate gray matter (GM) and white matter (WM) changes over three years. The prominent clinical feature was motor speech impairment associated with only mild Agrammatism. MRI demonstrated a progressive and severe GM atrophy of inferior fronto-insular-temporo-parietal regions with focal damage to frontotemporal and frontoparietal WM connections. This is the first report of longitudinal MRI data in a nfvPPA- GRN + patient and this report offers new insights into the pathophysiology of the disease

Elizabeth Rochon - One of the best experts on this subject based on the ideXlab platform.

  • lack of frank Agrammatism in the nonfluent agrammatic variant of primary progressive aphasia
    Dementia and geriatric cognitive disorders extra, 2016
    Co-Authors: Naida L. Graham, Carol Leonard, Tiffany W. Chow, Chris J.m. Scott, Alicia A. Mcneely, Mario Masellis, David F Tangwai, Sandra E Black, Elizabeth Rochon
    Abstract:

    Background/Aims: Frank Agrammatism, defined as the omission and/or substitution of grammatical morphemes with associated grammatical errors, is variably reported

  • Lack of Frank Agrammatism in the Nonfluent Agrammatic Variant of Primary Progressive Aphasia
    Karger Publishers, 2016
    Co-Authors: Naida L. Graham, Carol Leonard, David F. Tang-wai, Sandra Black, Tiffany W. Chow, Chris J.m. Scott, Alicia A. Mcneely, Mario Masellis, Elizabeth Rochon
    Abstract:

    Background/Aims: Frank Agrammatism, defined as the omission and/or substitution of grammatical morphemes with associated grammatical errors, is variably reported in patients with nonfluent variant primary progressive aphasia (nfPPA). This study addressed whether frank Agrammatism is typical in agrammatic nfPPA patients when this feature is not required for diagnosis. Method: We assessed grammatical production in 9 patients who satisfied current diagnostic criteria. Although the focus was Agrammatism, motor speech skills were also evaluated to determine whether dysfluency arose primarily from apraxia of speech (AOS), instead of, or in addition to, Agrammatism. Volumetric MRI analyses provided impartial imaging-supported diagnosis. Results: The majority of cases exhibited neither frank Agrammatism nor AOS. Conclusion: There are nfPPA patients with imaging-supported diagnosis and preserved motor speech skills who do not exhibit frank Agrammatism, and this may persist beyond the earliest stages of the illness. Because absence of frank Agrammatism is a subsidiary diagnostic feature in the logopenic variant of PPA, this result has implications for differentiation of the nonfluent and logopenic variants, and indicates that PPA patients with nonfluent speech in the absence of frank Agrammatism or AOS do not necessarily have the logopenic variant

Giancarlo Comi - One of the best experts on this subject based on the ideXlab platform.

  • clinical and mri correlates of disease progression in a case of nonfluent agrammatic variant of primary progressive aphasia due to progranulin grn cys157lysfsx97 mutation
    Journal of the Neurological Sciences, 2014
    Co-Authors: Francesca Caso, Giancarlo Comi, Giuseppe Magnani, F. Agosta, S. Galantucci, E.g. Spinelli, D. Galimberti, A. Falini, Massimo Filippi
    Abstract:

    Abstract Little is known about the longitudinal changes of brain damage in patients with sporadic nonfluent/agrammatic variant of primary progressive aphasia (nfvPPA) and in progranulin (GRN) mutation carriers. This study reports the clinical and MRI longitudinal data of a patient with nfvPPA carrying GRN Cys157LysfsX97 mutation (GRN +). Voxel-based morphometry, tensor-based morphometry and diffusion tensor MRI were applied to evaluate gray matter (GM) and white matter (WM) changes over three years. The prominent clinical feature was motor speech impairment associated with only mild Agrammatism. MRI demonstrated a progressive and severe GM atrophy of inferior fronto–insular–temporo–parietal regions with focal damage to frontotemporal and frontoparietal WM connections. This is the first report of longitudinal MRI data in a nfvPPA- GRN + patient and this report offers new insights into the pathophysiology of the disease.

  • in vivo signatures of nonfluent agrammatic primary progressive aphasia caused by ftld pathology
    Neurology, 2014
    Co-Authors: Francesca Caso, Maya L Henry, Benno Gesierich, Maria Luisa Mandelli, Brianne M Bettcher, Jennifer M Ogar, Massimo Filippi, Giancarlo Comi, Giuseppe Magnani
    Abstract:

    Objective: To identify early cognitive and neuroimaging features of sporadic nonfluent/agrammatic variant of primary progressive aphasia (nfvPPA) caused by frontotemporal lobar degeneration (FTLD) subtypes. Methods: We prospectively collected clinical, neuroimaging, and neuropathologic data in 11 patients with sporadic nfvPPA with FTLD-tau (nfvPPA-tau, n = 9) or FTLD–transactive response DNA binding protein pathology of 43 kD type A (nfvPPA-TDP, n = 2). We analyzed patterns of cognitive and gray matter (GM) and white matter (WM) atrophy at presentation in the whole group and in each pathologic subtype separately. We also considered longitudinal clinical data. Results: At first evaluation, regardless of pathologic FTLD subtype, apraxia of speech (AOS) was the most common cognitive feature and atrophy involved the left posterior frontal lobe. Each pathologic subtype showed few distinctive features. At presentation, patients with nfvPPA-tau presented with mild to moderate AOS, mixed dysarthria with prominent hypokinetic features, clear Agrammatism, and atrophy in the GM of the left posterior frontal regions and in left frontal WM. While speech and language deficits were prominent early, within 3 years of symptom onset, all patients with nfvPPA-tau developed significant extrapyramidal motor signs. At presentation, patients with nfvPPA-TDP had severe AOS, dysarthria with spastic features, mild Agrammatism, and atrophy in left posterior frontal GM only. Selective mutism occurred early, when general neurologic examination only showed mild decrease in finger dexterity in the right hand. Conclusions: Clinical features in sporadic nfvPPA caused by FTLD subtypes relate to neurodegeneration of GM and WM in frontal motor speech and language networks. We propose that early WM atrophy in nfvPPA is suggestive of FTLD-tau pathology while early selective GM loss might be indicative of FTLD-TDP.

  • Clinical and MRI correlates of disease progression in a case of nonfluent/agrammatic variant of primary progressive aphasia due to progranulin (GRN) Cys157LysfsX97 mutation
    'Elsevier BV', 2014
    Co-Authors: F. Caso, Giancarlo Comi, Giuseppe Magnani, F. Agosta, S. Galantucci, E.g. Spinelli, D. Galimberti, A. Falini, Massimo Filippi
    Abstract:

    Little is known about the longitudinal changes of brain damage in patients with sporadic nonfluent/agrammatic variant of primary progressive aphasia (nfvPPA) and in progranulin (GRN) mutation carriers. This study reports the clinical and MRI longitudinal data of a patient with nfvPPA carrying GRN Cys157LysfsX97 mutation (GRN +). Voxel-based morphometry, tensor-based morphometry and diffusion tensor MRI were applied to evaluate gray matter (GM) and white matter (WM) changes over three years. The prominent clinical feature was motor speech impairment associated with only mild Agrammatism. MRI demonstrated a progressive and severe GM atrophy of inferior fronto-insular-temporo-parietal regions with focal damage to frontotemporal and frontoparietal WM connections. This is the first report of longitudinal MRI data in a nfvPPA- GRN + patient and this report offers new insights into the pathophysiology of the disease