scholarly journals Vestibular Impairment in Frontotemporal Dementia Syndrome

2016 ◽  
Vol 6 (2) ◽  
pp. 194-204 ◽  
Author(s):  
Kiyotaka Nakamagoe ◽  
Kotarou Kadono ◽  
Tadachika Koganezawa ◽  
Mao Takiguchi ◽  
Makoto Terada ◽  
...  
Keyword(s):  
Frontotemporal Dementia ◽  
Clinical Symptoms ◽  
Vestibular Function ◽  
Clinical Syndrome ◽  
Gait Disturbance ◽  
Healthy Elderly ◽  
Dementia Syndrome ◽  
Caloric Nystagmus ◽  
System Objective ◽  
Caloric Tests

Background: No studies to date have attempted to evaluate frontotemporal lobar degeneration from the perspective of the vestibular system. Objective: The present study examined vestibular function in patients with frontotemporal dementia (FTD) clinical syndrome and evaluated whether vestibular disorders are involved in the clinical symptoms due to FTD. Methods: Fourteen patients with FTD syndrome, as well as healthy elderly controls without dementia, were included in the present study. All subjects underwent vestibular function tests using electronystagmography, such as caloric tests and visual suppression (VS) tests, in which the induced caloric nystagmus was suppressed by visual stimuli. The association between clinical symptoms and vestibular function in the FTD syndrome group was further examined. Results: In the FTD syndrome group, caloric nystagmus was not necessarily suppressed during VS tests. Furthermore, VS was observed to be significantly impaired in FTD syndrome patients with gait disturbance as compared to those without such disturbance. Conclusion: The present study revealed that impairment of VS in patients with FTD results in an inability to regulate vestibular function by means of visual perception, regardless of multiple presumed neuropathological backgrounds. This could also be associated with gait disturbance in patients with FTD syndrome.

scholarly journals Right Anterior Temporal Degeneration, Emotions, and Loss of Nonverbal Semantics: Guidelines for Diagnosis of the Emotional Semantic Variant Frontotemporal Dementia

2021 ◽  
Author(s):  
Kyan Younes ◽  
Valentina Borghesani ◽  
Maxime Montembeault ◽  
Salvatore Spina ◽  
Ariane E Welch ◽  
...  
Keyword(s):  
Frontotemporal Dementia ◽  
Right Hemisphere ◽  
Clinical Symptoms ◽  
Neuropsychological Testing ◽  
Primary Progressive Aphasia ◽  
Structural Mri ◽  
Underlying Mechanism ◽  
Semantic Knowledge ◽  
Clinical Syndrome ◽  
Semantic Variant

Anterior temporal lobe (ATL) degeneration is caused by a pathological process that has a focal onset in the left or right hemisphere. Patients with left-lateralized ATL atrophy typically meet criteria for semantic variant primary progressive aphasia (PPA), a clinical syndrome characterized by loss of verbal semantic knowledge. There is less consensus regarding the symptoms that emerge when atrophy targets the right ATL (rATL), but previous studies have emphasized prosopagnosia as well as alterations in emotion, memory, behavior, and semantic knowledge, symptoms that often lead to a diagnosis of behavioral variant frontotemporal dementia (bvFTD). The goal of the present study was to characterize the cognitive and socioemotional deficits of patients with rATL degeneration in order to refine current conceptualizations of the rATL clinical syndrome. We identified individuals clinically diagnosed as bvFTD or PPA in our cohort of patients prospectively evaluated for FTD-spectrum disorders. We selected patients who also underwent structural MRI and a comprehensive, multidisciplinary evaluation (n = 478). Based on structural MRI atrophy index, individuals with predominant, rATL atrophy (n = 46) were identified and patients with co-occurrence of significant frontal atrophy were excluded. Nineteen patients with rATL degeneration had undergone autopsy. We used the clinical histories to identify early symptoms and examined the cognitive, socioemotional, genetic, and pathological profiles of patients with rATL degeneration. In patients with rATL degeneration, the most common early clinical symptoms were loss of empathy (27%), person-specific semantic knowledge (23%), and complex compulsions (18%). On socioemotional testing and informant-reported measures, patients exhibited diminished interpersonal warmth, empathy, and emotional theory of mind. Neuropsychological testing revealed deficits in identifying famous people and discriminating facial affect despite preserved face perception. FTLD-TDP was the most frequent pathological correlate of rATL degeneration (84%), followed by Pick type (10%), a subtype of FTLD-tau. Our results indicate that patients with early, rATL-predominant degeneration present with a behavioral syndrome that results from loss of empathy for others. The underlying mechanism is a progressive loss of semantic knowledge for concepts of social-emotional relevance. We herein refer to this syndrome as emotional semantic variant frontotemporal dementia. We propose novel diagnostic criteria for this rATL syndrome in order to facilitate early identification in clinical and research settings. This classification is relevant because, if appropriately diagnosed, these patients most often have FTLD-TDP Type-C pathology.

Vestibular Function and Motion Sickness Susceptibility: Videonystagmographic Evidence From Oculomotor and Caloric Tests

2020 ◽  
Vol 29 (2) ◽  
pp. 188-198
Author(s):  
Cynthia G. Fowler ◽  
Margaret Dallapiazza ◽  
Kathleen Talbot Hadsell
Keyword(s):  
Phase Velocity ◽  
Motion Sickness ◽  
Repeated Measures ◽  
Short Form ◽  
Vestibular Function ◽  
Slow Phase ◽  
Test Results ◽  
Normal Range ◽  
Slow Phase Velocity ◽  
Caloric Tests

Purpose Motion sickness (MS) is a common condition that affects millions of individuals. Although the condition is common and can be debilitating, little research has focused on the vestibular function associated with susceptibility to MS. One causal theory of MS is an asymmetry of vestibular function within or between ears. The purposes of this study, therefore, were (a) to determine if the vestibular system (oculomotor and caloric tests) in videonystagmography (VNG) is associated with susceptibility to MS and (b) to determine if these tests support the theory of an asymmetry between ears associated with MS susceptibility. Method VNG was used to measure oculomotor and caloric responses. Fifty young adults were recruited; 50 completed the oculomotor tests, and 31 completed the four caloric irrigations. MS susceptibility was evaluated with the Motion Sickness Susceptibility Questionnaire–Short Form; in this study, percent susceptibility ranged from 0% to 100% in the participants. Participants were divided into three susceptibility groups (Low, Mid, and High). Repeated-measures analyses of variance and pairwise comparisons determined significance among the groups on the VNG test results. Results Oculomotor test results revealed no significant differences among the MS susceptibility groups. Caloric stimuli elicited responses that were correlated positively with susceptibility to MS. Slow-phase velocity was slowest in the Low MS group compared to the Mid and High groups. There was no significant asymmetry between ears in any of the groups. Conclusions MS susceptibility was significantly and positively correlated with caloric slow-phase velocity. Although asymmetries between ears are purported to be associated with MS, asymmetries were not evident. Susceptibility to MS may contribute to interindividual variability of caloric responses within the normal range.

Increased telomere length in patients with frontotemporal dementia syndrome

2021 ◽  
pp. 117565
Author(s):  
Eun-Joo Kim ◽  
Seong-Ho Koh ◽  
Jungsoon Ha ◽  
Duk L. Na ◽  
Sang Won Seo ◽  
...  
Keyword(s):  
Telomere Length ◽  
Frontotemporal Dementia ◽  
Dementia Syndrome

Apraxia screening predicts Alzheimer pathology in frontotemporal dementia

2018 ◽  
Vol 90 (5) ◽  
pp. 562-569 ◽  
Author(s):  
Matthias Pawlowski ◽  
Viktoria Joksch ◽  
Heinz Wiendl ◽  
Sven G Meuth ◽  
Thomas Duning ◽  
...  
Keyword(s):  
Frontotemporal Dementia ◽  
Clinical Presentation ◽  
Neuropsychological Testing ◽  
High Sensitivity ◽  
Clinical Syndrome ◽  
Test Results ◽  
Discriminative Power ◽  
Bedside Test ◽  
Neurodegenerative Dementias ◽  
Alzheimer Pathology

ObjectivesFrontotemporal dementia (FTD) is a heterogeneous clinical syndrome linked to diverse types of underlying neuropathology. Diagnosis is mainly based on clinical presentation and accurate prediction of underlying neuropathology remains difficult.MethodsWe present a large cohort of patients with FTD spectrum diseases (n=84). All patients were thoroughly characterised by cerebrospinal fluid (CSF) Alzheimer’s disease (AD) biomarkers, neuroimaging, neuropsychological testing and standardised apraxia screening.ResultsA potential AD pathology was found in 43% of patients with FTD. CSF AD biomarker levels positively correlated with AD-typical apraxia scores in patients with FTD. The discriminative power of apraxia test results indicative of AD pathology was high (sensitivity: 90%, specificity: 66%).ConclusionsApraxia is common in neurodegenerative dementias but under-represented in clinical workup and diagnostic criteria. Standardised apraxia screening may serve as bedside test to objectify an AD-typical apraxia profile as an early and robust sign of AD pathology in patients with FTD.

Predicting progression in the late onset frontal lobe syndrome

2018 ◽  
Vol 31 (5) ◽  
pp. 743-748 ◽  
Author(s):  
Flora T. Gossink ◽  
Everard Vijverberg ◽  
Welmoed Krudop ◽  
Philip Scheltens ◽  
Max L. Stek ◽  
...  
Keyword(s):  
Frontal Lobe ◽  
Clinical Symptoms ◽  
Late Onset ◽  
Clinical Syndrome ◽  
Clinical Markers ◽  
Neuropsychological Profile ◽  
Stereotypical Behavior ◽  
Executive Deficits ◽  
Magnetic Resonance Imaging Mri ◽  
Frontal Lobe Syndrome

ABSTRACTA late onset frontal lobe syndrome (LOF) refers to a clinical syndrome with apathy, disinhibition, or stereotypical behavior arising in middle or late adulthood. Diagnostics are challenging, and both clinicians and patients need reliable predictors of progression to improve clinical guidance. In this longitudinal multicenter and genetically screened prospective study, 137 LOF patients with frontal behavior (FBI score≥11) and/or stereotypical behavior (SRI≥10) were included. Progression was defined as institutionalization, death, or progression of frontal or temporal atrophy at magnetic resonance imaging (MRI) after two years of follow up. Absence of progression at MRI in addition to stable or improved Mini Mental State Examination and Frontal Assessment Battery scores after two years was indicative for non-progression. The presence of stereotypy and a neuropsychological profile with executive deficits at baseline were found to be predictive for progression, while a history and family history with psychiatric disorders were predictors for non-progression. The combination of these clinical markers had a predictive value of 80.4% (p < 0.05). In patients presenting with late onset behavioral symptoms, an appraisal of the rate of deterioration can be made by detailed mapping of clinical symptoms. Distinction of progressive discourses from non-progressive or treatable conditions is to be gained.

scholarly journals Effects of the Wenyang Zhenshuai Granule on the Expression of LncRNA-MiR143HG/miR-143 Regulating ERK5 in H9C2 Cardiomyocytes

2021 ◽  
Vol 2021 ◽  
pp. 1-8
Author(s):  
Zelin Xu ◽  
Xinyu Chen ◽  
Qingyang Chen ◽  
Huzhi Cai
Keyword(s):  
Heart Failure ◽  
Survival Rate ◽  
Chronic Heart Failure ◽  
Clinical Symptoms ◽  
Malignant Tumors ◽  
Clinical Syndrome ◽  
Control Group ◽  
Heart Insufficiency ◽  
Injury Model ◽  
H9c2 Cardiomyocytes

Chronic heart failure (CHF) is a complex clinical syndrome caused by a variety of heart problems, with a high incidence. The 5-year survival rate of patients with clinical symptoms is similar to that of malignant tumors. Wenyang Zhenshuai granules are a safe and effective granule of traditional Chinese medicine components, including aconite, dried ginger, licorice, and red ginseng. In contemporary clinical applications, it is widely used in acute and chronic heart insufficiency, coronary heart disease, and arrhythmia. This research cultured H9C2 cardiomyocytes and divided them into the normal control group, LncRNA-MiR143HG overexpression group, LncRNA-MiR143HG silence group, Adriamycin (ADR) group, ADR + medicated serum group, ADR + LncRNA-MiR143HG overexpression + medicated serogroup, and ADR + LncRNA-MiR143HG silence + medicated serogroup. The cells of each group were treated differently, and the survival rate of each group of cells and the expression levels of LncRNA-MiR143HG/miR-143 and ERK5 were detected at the end of the experiment, and the expression of LncRNA-MiR143HG/miR-143 in H9C2 cardiomyocytes was regulated by Wenyang Zhenshuai granules’ impact. The results of this study showed that, in the doxorubicin-induced H9C2 cardiomyocyte injury model, the expression of miR-143 was upregulated, and the expression of LncRNA-MiR143HG and ERK5 was significantly downregulated. Wenyang Zhenshuai granules can downregulate the expression of miR-143 to promote ERK5 protein expression and phosphorylation. The process is regulated by LncRNA-MiR143HG/miR-143, which may be one of its important mechanisms for the treatment of chronic heart failure.

Compulsive Behaviors as Presenting Symptoms of Frontotemporal Dementia

1997 ◽  
Vol 10 (4) ◽  
pp. 154-157 ◽  
Author(s):  
Mario F. Mendez ◽  
Kent M. Perryman ◽  
Bruce L. Miller ◽  
J. Randolph Swartz ◽  
Jeffrey L. Cummings
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Frontotemporal Dementia ◽  
Single Photon ◽  
Photon Emission ◽  
Presenting Symptoms ◽  
Younger Age ◽  
Dementia Syndrome ◽  
Compulsive Behaviors ◽  
Neurodegenerative Dementia

Frontotemporal dementia (FTD) is a common neurodegenerative dementia syndrome. Compulsive behaviors frequently occur in FTD and may be presenting symptoms of this disorder. This study evaluated compulsive behaviors as presenting symptoms in 29 patients with FTD compared to 48 patients with Alzheimer's disease (AD) enrolled in the UCLA Alzheimer's Disease Center. The FTD patients met the Lund and Manchester criteria for FTD and had predominant frontal hypoperfusion on single-photon emission computer tomography neuroimaging. The AD patients met National Institute of Neurological and Communicative Disorders-Alzheimer's Disease and Related Disorders criteria for clinically probable AD. Compulsive behaviors occurred in 11 FTD patients (38%) versus 5 AD patients (10%) (χ2 = 6.73, P < .01). This difference persisted after controlling for the younger age of the FTD group. There was a range of compulsive behaviors, with the most frequent being repetitive checking activities. Compulsive behaviors are common presenting symptoms among FTD patients and may result from an inability to inhibit urges to perform compulsive movements from damage to frontal-striatal circuits.

Prognosis of Inner Ear Periphery and Central Vestibular Plasticity in Sudden Deafness with Vertigo

2005 ◽  
Vol 114 (10) ◽  
pp. 786-791 ◽  
Author(s):  
Tadashi Kitahara ◽  
Noriaki Takeda ◽  
Suetaka Nishiike ◽  
Shin-Ichi Okumura ◽  
Takeshi Kubo
Keyword(s):  
Everyday Life ◽  
Inner Ear ◽  
Pure Tone ◽  
Vestibular Function ◽  
The Other ◽  
Sudden Deafness ◽  
Vestibular Neurectomy ◽  
Canal Paresis ◽  
Clinical Problems ◽  
Caloric Tests

Objectives: We sought to elucidate the clinical problems and otopathology of patients with sudden deafness with vertigo (SDV). Methods: In 24 patients with SDV who had significant canal paresis (CP) at their first visit to our hospital between 1997 and 2001, we examined pure tone audiograms, caloric tests, and several questionnaires twice, at the first visit within 5 days after the onset and around 2 years after steroid therapy. Results: These examinations revealed that improvements of auditory and vestibular function in patients with SDV tended to be correlated with one another. Sixteen of the 24 patients (66.7%) still had CP. This rate in SDV was significantly worse than that reported previously for vestibular neuritis (VN). On the other hand, patients with SDV with long-lasting CP had a faster reduction of head-shaking afternystagmus and of handicaps in their everyday life due to dizziness than did patients with VN and CP. Conclusions: These findings suggest that SDV may deteriorate the inner ear function more severely but accelerate the central vestibular compensation more effectively than VN after the lesion. It is well known that vestibular neurectomy causes much more severe motion-induced dizziness after surgery than does labyrinthectomy. Taken together, these findings suggest different regions of damage in SDV (mainly the labyrinth, as in labyrinthectomy) and VN (mainly the ganglion, as in vestibular neurectomy).

Sinusoidal Harmonic Acceleration

1981 ◽  
Vol 90 (4_suppl2) ◽  
pp. 10-14
Author(s):  
Robert H. Mathog
Keyword(s):  
Diagnostic Tests ◽  
Angular Acceleration ◽  
Vestibular Function ◽  
Test System ◽  
The Other ◽  
Input Output ◽  
Normal Individuals ◽  
Sinusoidal Harmonic Acceleration ◽  
Caloric Tests ◽  
Sensitive Method

The need for accurate and reliable diagnostic tests for the evaluation of vestibular function has stimulated investigation of rotational methods and more recently, an evaluation of harmonic angular acceleration. Initial studies, using these techniques in humans, demonstrated a linear input-output function from 0.24 to 0.04 cycles/s at 0.4 radians/s2, but a reduction in the increase of response with an increase of acceleration or a decrease of frequency from this range. Nystagmic responses were symmetrical in these same normal individuals. In patients with vestibular disease, input-output functions, as well as symmetry, were altered, and appeared related to the degree of pathology. Evaluation of the test system in animals, rendered ototoxic with streptomycin, showed a depression in response similar to caloric tests. All data suggested that harmonic angular acceleration provided a reliable and sensitive method for evaluation of vestibular function, and a useful and important confirmation to the other methods that are available for these evaluations.

scholarly journals Clinical and Electronystagmographic findings in Friedreich's Ataxia

1978 ◽  
Vol 5 (1) ◽  
pp. 71-73 ◽  
Author(s):  
L. A. Monday ◽  
B. Lemieux ◽  
H. St-Vincent ◽  
A. Barbeau
Keyword(s):  
Vestibular Function ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia ◽  
Caloric Tests

SUMMARY:A thorough investigation of vestibular function has been carried out in 16 patients with typical Friedreich's ataxia. Electronystagmography and caloric tests revealed a number of inconstant abnormalities. Most abnormal findings were related to ocular dysmetria, disorganized pursuit and square waves.

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