Frontotemporal Dementia

Research in Frontotemporal Dementias and Young-Onset Dementias at Johns Hopkins

Frontotemporal Dementia | Johns Hopkins Medicine

Principal Investigators:
Chiadi Onyike, M.D., M.H.S.
Kathleen Kortte, Ph.D.

Co-investigator:
Eleni Aretouli, Ph.D.

The primary goal of this study is to characterize impairments of social cognition in frontotemporal dementia and Alzheimer disease, to describe their correlates with other aspects of cognition and to explore relationships with self-maintenance, life participation and burden of care.

The study plans to enroll 60 patients (30 with FTD and 30 with AD) at the Johns Hopkins Hospital and The Johns Hopkins Bayview Medical Center.

  These patients will undergo detailed testing of the social, executive and other domains of cognitive function, in addition to other elements of behavioral and neurological assessment.

  Participation in the study is a one-time event, i.e., only one visit is required for completion of all assessments.

Contact
410-955-5147 or conyike1@jhmi.edu

Location The Johns Hopkins HospitalDivision of Geriatric Psychiatry and Neuropsychiatry550 N. Broadway, Suite 308

Baltimore, MD

Multicenter Validation of Research Diagnostic Criteria for Frontotemporal Dementia and Primary Progressive Aphasia

Principal Investigator:
Katya Rascovsky, Ph.D. – University of California at San Francisco

Hopkins Principal investigators:
Chiadi Onyike, M.D., M.H.S.
Argye Hillis, M.D.

The primary goal of this study is to examine the sensitivity and specificity of recently developed research diagnostic criteria for frontotemporal Dementia (FTD) and primary progressive aphasia (PPA).

This multicenter study plans to enroll cases of FTD confirmed by neuropathologic examination and examine all relevant clinical examination records, in order to determine the accuracy of the newly developed clinical criteria for the diagnosis of FTD and PPA.

Contact
410-955-5147 or conyike1@jhmi.edu

Location The Johns Hopkins HospitalDivision of Geriatric Psychiatry and Neuropsychiatry550 N. Broadway, Suite 308

Baltimore, MD

A Multi-Center Trial of Memantine for the Frontal and Temporal
Subtypes of Frontotemporal Dementia

Principal Investigator:Adam Boxer, M.D.

University of California – San Francisco

Hopkins Principal Investigator:
Chiadi Onyike, M.D., M.H.S.

The primary goal of this trial is to determine whether memantine is effective in slowing the rate of behavioral decline in frontotemporal dementia (FTD).

  The study will also assess the safety and tolerability of long-term treatment with memantine in patients with FTD or semantic dementia (SD); the effectiveness of memantine in slowing the rate of cognitive decline and other complictions in FTD.

The study will also examine whether treatment reduces the burden of the care-giver's experience.

The study plans to enroll 140 patients with FTD or SD at 11 U.S. medical centers (about 13 subjects per site).  These patients will be randomly assigned to receive memantine or placebo for 26 weeks.

  In addition to a telephone screening interview, data collection assessments at the study site will occur at baseline (i.e. week 0), and at 6, 12 and 26 weeks and will include physical and neurological evaluations, routine laboratory tests, electrocardiogram (ECG) and neuropsychological and functional assessments.

 The estimated duration of the study is 8 months, including the interval between the screening and the last visit.

Research Program Assistant
Sandeepa Sur, M.Sc.

Contact
410-502-3747 or ssur3@jhmi.edu

Location The Johns Hopkins HospitalDivision of Geriatric Psychiatry and Neuropsychiatry550 N. Broadway, Suite 308

Baltimore, MD

The Relationship between Frontotemporal Dementia (FTD) and Amyotrophic Lateral Sclerosis (ALS): A study of cognition and behavior in ALS

Principal Investigator: Chiadi Onyike, M.D., M.H.S.

This two-year longitudinal study is a collaborative between the clinic and the Johns Hopkins ALS Clinic, and is supported by the Johns Hopkins Alzheimer’s Disease Research Center and the National Institutes on Aging. The purpose of this study is to describe the prevalence and incidence of cognitive and behavioral impairments in ALS, the risk factors for their development, and their potential association with FTD. 

A total of 225 participants will be enrolled with the study.

 The study is designed to collect measures of cognitive and behavioral function from patients ALS and two controls groups: patients with myasthenia gravis (a disease in which patients also develop muscle weakness in similar ways to ALS patients) and individuals who are healthy.

 Participants with ALS will be assessed at 3, 6, 9, 12, 15 and 18 months (in the clinic or in home visits), while those that have MG or are healthy will be assessed at 6, 12 and 18 months.

 This will provide valuable information about the onset and evolution of cognitive and behavioral problems in ALS. If the present-day hypothesis is correct that ALS and FTD represent different poles of a spectrum of disease, this study should increase our understanding of the earliest stages of FTD.

Research Program Assistant
Sandeepa Sur, M.Sc.

Contact
410-502-3747 or ssur3@jhmi.edu

Location The Johns Hopkins HospitalDivision of Geriatric Psychiatry and Neuropsychiatry550 N. Broadway, Suite 305

Baltimore, MD

Measurement of the Clinical Progression and Severity of Frontotemporal Dementia

Principal Investigator:  Chiadi Onyike, M.D., M.H.S.

Advancements in our understanding of the neurobiology of the FTDs have spurred interest in the development of measures that can track the progression of subtypes of FTD over the entire span of the disease.

  This study examines how cognitive, behavioral, and functional deterioration unfolds in FTD, with an eye towards the identification of clinical staging markers and the development of an instrument that can be used in practice and research to track disease progression.

  All individuals who have FTD and receive their care from the clinic are eligible for the study.

Contact
410-955-6158 or conyike1@jhmi.edu

Location The Johns Hopkins HospitalDivision of Geriatric Psychiatry and Neuropsychiatry550 N. Broadway, Suite 305

Baltimore, MD

Source: https://www.hopkinsmedicine.org/psychiatry/specialty_areas/geriatric_neuro/frontotemporal_dementia/research/research_ftd.html

Frontotemporal Dementia | Johns Hopkins Psychiatry Guide

Frontotemporal Dementia | Johns Hopkins Medicine

— The first section of this topic is shown below —

  • Frontotemporal dementia (FTD) is a clinically and histopathologically heterogeneous neurodegenerative disorder that presents as a focal disorder of conduct, temperament, judgment, and/or speech and language.
  • Three canonical syndromes are recognized:
    • Behavior variant FTD (bvFTD): the commonest; characterized by a focal decline in conduct, temperament, and judgment
    • Primary progressive aphasia (PPA) variants: primary non-fluent aphasia features halting, telegraphic speech with agrammatism and phonological errors
    • Semantic dementia: manifests progressive anomia, with focal loss of word and object knowledge, and fluent and vacuous speech with semantic errors
  • In time, the illness includes a wider range of cognitive deficits.
  • Frontotemporal lobar degeneration refers to the histopathological state that underlies the clinical syndromes.
  • Cognitive impairment due to frontotemporal dementia is classified under the neurocognitive disorders (NCDs) section of the Diagnostic and Statistical Manual of Mental Disorders, 5th Edition (DSM-5).[1]

— To view the remaining sections of this topic, please sign in or purchase a subscription —

  • Frontotemporal dementia (FTD) is a clinically and histopathologically heterogeneous neurodegenerative disorder that presents as a focal disorder of conduct, temperament, judgment, and/or speech and language.
  • Three canonical syndromes are recognized:
    • Behavior variant FTD (bvFTD): the commonest; characterized by a focal decline in conduct, temperament, and judgment
    • Primary progressive aphasia (PPA) variants: primary non-fluent aphasia features halting, telegraphic speech with agrammatism and phonological errors
    • Semantic dementia: manifests progressive anomia, with focal loss of word and object knowledge, and fluent and vacuous speech with semantic errors
  • In time, the illness includes a wider range of cognitive deficits.
  • Frontotemporal lobar degeneration refers to the histopathological state that underlies the clinical syndromes.
  • Cognitive impairment due to frontotemporal dementia is classified under the neurocognitive disorders (NCDs) section of the Diagnostic and Statistical Manual of Mental Disorders, 5th Edition (DSM-5).[1]

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McClam, Tamela, and Chiadi Onyike‎. “Frontotemporal Dementia.” Johns Hopkins Psychiatry Guide, 2017. Johns Hopkins Guide, www.hopkinsguides.com/hopkins/view/Johns_Hopkins_Psychiatry_Guide/787108/all/Frontotemporal_Dementia. McClam T, Onyike‎ C. Frontotemporal Dementia. Johns Hopkins Psychiatry Guide. 2017. https://www.hopkinsguides.com/hopkins/view/Johns_Hopkins_Psychiatry_Guide/787108/all/Frontotemporal_Dementia. Accessed May 18, 2020.McClam, T., & Onyike‎, C. (2017). Frontotemporal Dementia. In Johns Hopkins Psychiatry Guide Retrieved May 18, 2020, from https://www.hopkinsguides.com/hopkins/view/Johns_Hopkins_Psychiatry_Guide/787108/all/Frontotemporal_DementiaMcClam T, Onyike‎ C. Frontotemporal Dementia [Internet]. In: Johns Hopkins Psychiatry Guide. ; 2017. [cited 2020 May 18]. Available from: https://www.hopkinsguides.com/hopkins/view/Johns_Hopkins_Psychiatry_Guide/787108/all/Frontotemporal_Dementia.* Article titles in AMA citation format should be in sentence-caseMLAAMAAPAVANCOUVERTY – ELECT1 – Frontotemporal DementiaID – 787108A1 – McClam,Tamela,M.D.AU – Onyike‎,Chiadi,M.D.Y1 – 2017/03/01/BT – Johns Hopkins Psychiatry GuideUR – https://www.hopkinsguides.com/hopkins/view/Johns_Hopkins_Psychiatry_Guide/787108/all/Frontotemporal_DementiaDB – Johns Hopkins GuideDP – Unbound MedicineER –

Source: https://www.hopkinsguides.com/hopkins/view/Johns_Hopkins_Psychiatry_Guide/787108/all/Frontotemporal_Dementia