scholarly journals Shape Profile of Corpus Callosum As a Signature to Phenotype Different Dementia

2020 ◽  
Author(s):  
Sandhya Mangalore ◽  
Shiva Shanker Reddy Mukku ◽  
Sriharish Vankayalapati ◽  
Palanimuthu Thangaraju Sivakumar ◽  
Mathew Varghese
Keyword(s):  
Corpus Callosum ◽  
Frontotemporal Dementia ◽  
Corticobasal Degeneration ◽  
Lewy Body Dementia ◽  
Clinical History ◽  
The Body ◽  
Cortical Atrophy ◽  
Alzheimer Dementia ◽  
Geriatric Clinic ◽  
Cortical Regions

Abstract Background Phenotyping dementia is always a complex task for a clinician. There is a need for more practical biomarkers to aid clinicians. Objective The aim of the study is to investigate the shape profile of corpus callosum (CC) in different phenotypes of dementia. Materials and Methods Our study included patients who underwent neuroimaging in our facility as a part of clinical evaluation for dementia referred from Geriatric Clinic (2017–2018). We have analyzed the shape of CC and interpreted the finding using a seven-segment division. Results The sample included MPRAGE images of Alzheimer’ dementia (AD) (n = 24), posterior cortical atrophy- Alzheimer’ dementia (PCA-AD) (n = 7), behavioral variant of frontotemporal dementia (Bv-FTD) (n = 17), semantic variant frontotemporal dementia (Sv-FTD) (n = 11), progressive nonfluent aphasia (PNFA) (n = 4), Parkinson’s disease dementia (PDD) (n = 5), diffuse Lewy body dementia (n = 7), progressive supranuclear palsy (PSP) (n = 3), and corticobasal degeneration (CBD) (n = 3). We found in posterior dementias such as AD and PCA-AD that there was predominant atrophy of splenium of CC. In Bv-FTD, the genu and anterior half of the body of CC was atrophied, whereas in PNFA, PSP, PDD, and CBD there was atrophy of the body of CC giving a dumbbell like profile. Conclusion Our study findings were in agreement with the anatomical cortical regions involved in different phenotypes of dementia. Our preliminary study highlighted potential usefulness of CC in the clinical setting for phenotyping dementia in addition to clinical history and robust biomarkers.

scholarly journals An autopsy-proven case of Corticobasal degeneration heralded by Pontine infarction

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Dallah Yoo ◽  
Sung-Hye Park ◽  
Sungwook Yu ◽  
Tae-Beom Ahn
Keyword(s):  
Neurodegenerative Disorders ◽  
Clinical Manifestations ◽  
Neuronal Loss ◽  
Corticobasal Degeneration ◽  
Executive Dysfunction ◽  
Lacunar Infarction ◽  
Cortical Atrophy ◽  
Dystonic Posturing ◽  
Proven Case ◽  
The Right

Abstract Background Neurodegenerative disorders are characterized by insidious progression with poorly-delineated long latent period. Antecedent clinical insult could rarely unmask latent neurodegenerative disorders. Here, we report an autopsy-proven case of corticobasal degeneration which was preceded by a lacunar infarction. Case presentation A 58-year-old man presented with acute ataxia associated with a lacunar infarction in the right paramedian pons. His ataxia persisted with additional progressive gait difficulty and left arm clumsiness. Six months later, a follow-up neurological examination showed asymmetrical bradykinesia, apraxia, dystonic posturing, postural instability, and mild ataxia of the left limbs. Cognitive examination revealed frontal executive dysfunction and visuospatial difficulties. Dopamine transporter imaging scan demonstrated bilateral reduced uptakes in mid-to-posterior putamen, more prominent on the right side. Levodopa-unresponsive parkinsonism, asymmetric limb dystonia, and ideomotor apraxia became more conspicuous, while limb ataxia gradually vanished. The patient became unable to walk without assistance after 1 year, and died 4 years after the symptom onset. Autopsy findings showed frontoparietal cortical atrophy, ballooned neurons, and phosphorylated tau-positive astrocytic plaques and neuropil threads with gliosis and neuronal loss, confirming the corticobasal degeneration. Conclusions The case illustrates that precedent clinical events such as stroke might tip a patient with subclinical CBS into overt clinical manifestations.

scholarly journals Structural brain signature of cognitive decline in Parkinson’s disease: DTI-based evidence from the LANDSCAPE study

2019 ◽  
Vol 12 ◽  
pp. 175628641984344 ◽  
Author(s):  
Martin Gorges ◽  
Hans-Peter Müller ◽  
Inga Liepelt-Scarfone ◽  
Alexander Storch ◽  
Richard Dodel ◽  
...  
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
White Matter ◽  
Corpus Callosum ◽  
Cognitive Decline ◽  
Diffusion Tensor ◽  
Structural Connectivity ◽  
Structural Data ◽  
The Body ◽  
Normal Cognitive Function

Background: The nonmotor symptom spectrum of Parkinson’s disease (PD) includes progressive cognitive decline mainly in late stages of the disease. The aim of this study was to map the patterns of altered structural connectivity of patients with PD with different cognitive profiles ranging from cognitively unimpaired to PD-associated dementia. Methods: Diffusion tensor imaging and neuropsychological data from the observational multicentre LANDSCAPE study were analyzed. A total of 134 patients with PD with normal cognitive function (56 PD-N), mild cognitive impairment (67 PD-MCI), and dementia (11 PD-D) as well as 72 healthy controls were subjected to whole-brain-based fractional anisotropy mapping and covariance analysis with cognitive performance measures. Results: Structural data indicated subtle changes in the corpus callosum and thalamic radiation in PD-N, whereas severe white matter impairment was observed in both PD-MCI and PD-D patients including anterior and inferior fronto-occipital, uncinate, insular cortices, superior longitudinal fasciculi, corona radiata, and the body of the corpus callosum. These regional alterations were demonstrated for PD-MCI and were more pronounced in PD-D. The pattern of involved regions was significantly correlated with the Consortium to Establish a Registry for Alzheimer’s Disease (CERAD) total score. Conclusions: The findings in PD-N suggest impaired cross-hemispherical white matter connectivity that can apparently be compensated for. More pronounced involvement of the corpus callosum as demonstrated for PD-MCI together with affection of fronto-parieto-temporal structural connectivity seems to lead to gradual disruption of cognition-related cortico-cortical networks and to be associated with the onset of overt cognitive deficits. The increase of regional white matter damage appears to be associated with the development of PD-associated dementia.

fMRI–Adaptation Studies of Viewpoint Tuning in the Extrastriate and Fusiform Body Areas

2010 ◽  
Vol 103 (3) ◽  
pp. 1467-1477 ◽  
Author(s):  
John C. Taylor ◽  
Alison J. Wiggett ◽  
Paul E. Downing
Keyword(s):  
Human Body ◽  
Adaptation Effect ◽  
The Body ◽  
Neural Mechanisms ◽  
Similar Degree ◽  
Neuronal Responses ◽  
Adaptation Studies ◽  
View Invariance ◽  
Cortical Regions ◽  
Fmri Adaptation

People are easily able to perceive the human body across different viewpoints, but the neural mechanisms underpinning this ability are currently unclear. In three experiments, we used functional MRI (fMRI) adaptation to study the view-invariance of representations in two cortical regions that have previously been shown to be sensitive to visual depictions of the human body—the extrastriate and fusiform body areas (EBA and FBA). The BOLD response to sequentially presented pairs of bodies was treated as an index of view invariance. Specifically, we compared trials in which the bodies in each image held identical poses (seen from different views) to trials containing different poses. EBA and FBA adapted to identical views of the same pose, and both showed a progressive rebound from adaptation as a function of the angular difference between views, up to ∼30°. However, these adaptation effects were eliminated when the body stimuli were followed by a pattern mask. Delaying the mask onset increased the response (but not the adaptation effect) in EBA, leaving FBA unaffected. We interpret these masking effects as evidence that view-dependent fMRI adaptation is driven by later waves of neuronal responses in the regions of interest. Finally, in a whole brain analysis, we identified an anterior region of the left inferior temporal sulcus (l-aITS) that responded linearly to stimulus rotation, but showed no selectivity for bodies. Our results show that body-selective cortical areas exhibit a similar degree of view-invariance as other object selective areas—such as the lateral occipitotemporal area (LO) and posterior fusiform gyrus (pFs).

scholarly journals Analysis of brain atrophy and local gene expression in genetic frontotemporal dementia

2020 ◽  
Vol 2 (2) ◽  
Author(s):  
Andre Altmann ◽  
David M Cash ◽  
Martina Bocchetta ◽  
Carolin Heller ◽  
Regina Reynolds ◽  
...  
Keyword(s):  
Gene Expression ◽  
Frontotemporal Dementia ◽  
Cell Function ◽  
Neurodegenerative Disorder ◽  
Positive Association ◽  
Negative Association ◽  
Marker Genes ◽  
Gene Set ◽  
Genetic Groups ◽  
Cortical Regions

Abstract Frontotemporal dementia is a heterogeneous neurodegenerative disorder characterized by neuronal loss in the frontal and temporal lobes. Despite progress in understanding which genes are associated with the aetiology of frontotemporal dementia, the biological basis of how mutations in these genes lead to cell loss in specific cortical regions remains unclear. In this work, we combined gene expression data for 16 772 genes from the Allen Institute for Brain Science atlas with brain maps of grey matter atrophy in symptomatic C9orf72, GRN and MAPT mutation carriers obtained from the Genetic Frontotemporal dementia Initiative study. No significant association was seen between C9orf72, GRN and MAPT expression and the atrophy patterns in the respective genetic groups. After adjusting for spatial autocorrelation, between 1000 and 5000 genes showed a negative or positive association with the atrophy pattern within each individual genetic group, with the most significantly associated genes being TREM2, SSBP3 and GPR158 (negative association in C9Orf72, GRN and MAPT respectively) and RELN, MXRA8 and LPA (positive association in C9Orf72, GRN and MAPT respectively). An overrepresentation analysis identified a negative association with genes involved in mitochondrial function, and a positive association with genes involved in vascular and glial cell function in each of the genetic groups. A set of 423 and 700 genes showed significant positive and negative association, respectively, with atrophy patterns in all three maps. The gene set with increased expression in spared cortical regions was enriched for neuronal and microglial genes, while the gene set with increased expression in atrophied regions was enriched for astrocyte and endothelial cell genes. Our analysis suggests that these cell types may play a more active role in the onset of neurodegeneration in frontotemporal dementia than previously assumed, and in the case of the positively associated cell marker genes, potentially through emergence of neurotoxic astrocytes and alteration in the blood–brain barrier, respectively.

scholarly journals Cutaneous Leishmaniasis in a Bangladeshi Adult: A Case Report

2015 ◽  
Vol 25 (2) ◽  
pp. 78-80
Author(s):  
Abdur Rahim ◽  
Md Moniruzzan ◽  
Rashedul Hassan ◽  
Monira Sarmin ◽  
Md Abdullah Yusuf ◽  
...  
Keyword(s):  
Cutaneous Leishmaniasis ◽  
Plasma Cells ◽  
Case Reports ◽  
Ring Finger ◽  
Clinical History ◽  
The Body ◽  
Nasal Bridge ◽  
Cutaneous Tuberculosis ◽  
Exposed Part ◽  
Ulcerative Lesions

Cutaneous leishmaniasis is rare in Bangladesh although very few case reports are seen since last few years. But Visceral Leishmaniasis (kala azar) and PKDL are common in this region. In country like ours where tuberculosis and leprosy are more prevalent Cutaneous Leishmaniasis is very likely to be mistreated as Cutaneous tuberculosis especially lupus vulgaris or leprosy. Cases of Cutaneous Leishmaniasis (CL) are usually imported to Bangladesh from other endemic countries. A patient from an endemic area of Cutaneous Leishmaniasis, a non-healing nodulo-ulcerative lesion on exposed part of the body, dermal infiltration with lymphocytes, histiocytes and plasma cells and demonstration of intracellular parasites in lesional skin establish the diagnosis of Cutaneous Leishmaniasis. We present a case of Cutaneous Leishmaniasis in a Bangladeshi adult working in Saudi Arabia for more than 15 years. He presented with multiple ulcerative lesions on nasal bridge, right ear lobule and dorsum of right ring finger. The patient’s clinical history, morphology of the lesions and laboratory analysis were consistent with Cutaneous Leishmaniasis, a rare entity for Bangladesh.Bangladesh J Medicine Jul 2014; 25 (2) : 78-80

scholarly journals Fronto-striatal atrophy correlates of neuropsychiatric dysfunction in frontotemporal dementia (FTD) and Alzheimer's disease (AD)

2013 ◽  
Vol 7 (1) ◽  
pp. 75-82 ◽  
Author(s):  
Dong Seok Yi ◽  
Maxime Bertoux ◽  
Eneida Mioshi ◽  
John R. Hodges ◽  
Michael Hornberger
Keyword(s):  
Frontotemporal Dementia ◽  
Rating Scale ◽  
Brain Regions ◽  
Abnormal Behaviour ◽  
Behavioural Symptoms ◽  
Visual Rating ◽  
Prefrontal Cortical ◽  
Mri Scans ◽  
Visual Rating Scale ◽  
Cortical Regions

ABSTRACT Behavioural disturbances in frontotemporal dementia (FTD) are thought to reflect mainly atrophy of cortical regions. Recent studies suggest that subcortical brain regions, in particular the striatum, are also significantly affected and this pathology might play a role in the generation of behavioural symptoms. Objective: To investigate prefrontal cortical and striatal atrophy contributions to behavioural symptoms in FTD. Methods: One hundred and eighty-two participants (87 FTD patients, 39 AD patients and 56 controls) were included. Behavioural profiles were established using the Cambridge Behavioural Inventory Revised (CBI-R) and Frontal System Behaviour Scale (FrSBe). Atrophy in prefrontal (VMPFC, DLPFC) and striatal (caudate, putamen) regions was established via a 5-point visual rating scale of the MRI scans. Behavioural scores were correlated with atrophy rating scores. Results: Behavioural and atrophy ratings demonstrated that patients were significantly impaired compared to controls, with bvFTD being most severely affected. Behavioural-anatomical correlations revealed that VMPFC atrophy was closely related to abnormal behaviour and motivation disturbances. Stereotypical behaviours were associated with both VMPFC and striatal atrophy. By contrast, disturbance of eating was found to be related to striatal atrophy only. Conclusion: Frontal and striatal atrophy contributed to the behavioural disturbances seen in FTD, with some behaviours related to frontal, striatal or combined fronto-striatal pathology. Consideration of striatal contributions to the generation of behavioural disturbances should be taken into account when assessing patients with potential FTD.

Abdominal wall hernias

2018 ◽  
pp. 529-540
Author(s):  
Abdullah Jibawi ◽  
Mohamed Baguneid ◽  
Arnab Bhowmick
Keyword(s):  
Connective Tissue ◽  
Abdominal Wall ◽  
Chronic Cough ◽  
Incidental Finding ◽  
Clinical History ◽  
The Body ◽  
Abdominal Pressure ◽  
Ehlers Danlos Syndrome ◽  
Danlos Syndrome ◽  
Groin Hernias

Hernias are abnormal protrusion of an organ through a weakness/defect in the body wall that contains it. Classifications include groin hernias, ventral abdominal wall hernias (umbilical, femoral), incisional, Spigelian, and lumbar hernias. Inguinal hernias are the commonest types of abdominal wall hernias (~75%). Male are affected 15-times more frequently. Hernias are more common in smokers, patients with underlying connective tissue disorders (Ehlers Danlos Syndrome, Marfan syndrome), and patients with increased intra-abdominal pressure (obesity, heavy lifting, chronic cough, and chronic straining during defecation and urination). Hernias present as incidental finding on imaging, asymptomatic lumps, painful lumps, or incarcerated or strangulated hernias. Clinical history and examination are the mainstay of diagnosis. Most hernias are treated with surgical repair (open or laparoscopic). Conservative wait and watch policy is indicated in some cases.

scholarly journals Neglected intrauterine fetal demise for more than two decades leading to the development of a lithopedion: a case report

2019 ◽  
Vol 13 (1) ◽  
Author(s):  
Fitsum Fikru Gebresenbet ◽  
Abdu Mengesha Mulat ◽  
Namus Muhajir Nur ◽  
Ferehiwot Bekele Getaneh
Keyword(s):  
Vesicovaginal Fistula ◽  
Rare Complication ◽  
Clinical Manifestations ◽  
Clinical History ◽  
The Body ◽  
Obstructed Labor ◽  
Fetal Demise ◽  
Case Presentation ◽  
Intrauterine Fetal Demise ◽  
Long Time

Abstract Background Lithopedion is a word derived from the Greek words lithos, meaning stone, and paidion, meaning child, to describe a fetus that has become stony or petrified. Lithopedion is a rare complication of pregnancy which occurs when a fetus dies and becomes too large to be reabsorbed by the body. This entity in rare circumstances can be challenging for physicians to diagnose since it has a range of clinical manifestations. Case presentation We present a case of a 55-year-old, gravida IV para III, Ethiopian woman from Ethiopia with a retained fetus and vesicovaginal fistula after an obstructed labor and a neglected intrauterine fetal demise of approximately 22 years. The diagnosis was confirmed by suggestive clinical history, physical examination findings, and an abdominopelvic computed tomography scan. Laparotomy and removal of the lithopedion was done and our patient was sent to a fistula hospital for vesicovaginal fistula repair. Conclusion This case is a rare phenomenon in which the dead fetus remained in the uterus for a long time after a neglected obstructed labor and uterine rupture.

scholarly journals The Missing Heritability of Sporadic Frontotemporal Dementia: New Insights from Rare Variants in Neurodegenerative Candidate Genes

2019 ◽  
Vol 20 (16) ◽  
pp. 3903 ◽  
Author(s):  
Miriam Ciani ◽  
Cristian Bonvicini ◽  
Catia Scassellati ◽  
Matteo Carrara ◽  
Carlo Maj ◽  
...  
Keyword(s):  
Neurodegenerative Diseases ◽  
Frontotemporal Dementia ◽  
Candidate Genes ◽  
Early Onset ◽  
Genetic Factors ◽  
Rare Variants ◽  
Late Onset ◽  
Genetic Effect ◽  
Lewy Body Dementia ◽  
Missing Heritability

Frontotemporal dementia (FTD) is a common form of dementia among early-onset cases. Several genetic factors for FTD have been revealed, but a large proportion of FTD cases still have an unidentified genetic origin. Recent studies highlighted common pathobiological mechanisms among neurodegenerative diseases. In the present study, we investigated a panel of candidate genes, previously described to be associated with FTD and/or other neurodegenerative diseases by targeted next generation sequencing (NGS). We focused our study on sporadic FTD (sFTD), devoid of disease-causing mutations in GRN, MAPT and C9orf72. Since genetic factors have a substantially higher pathogenetic contribution in early onset patients than in late onset dementia, we selected patients with early onset (<65 years). Our study revealed that, in 50% of patients, rare missense potentially pathogenetic variants in genes previously associated with Alzheimer’s disease, Parkinson disease, amyotrophic lateral sclerosis and Lewy body dementia (GBA, ABCA7, PARK7, FUS, SORL1, LRRK2, ALS2), confirming genetic pleiotropy in neurodegeneration. In parallel, a synergic genetic effect on FTD is suggested by the presence of variants in five different genes in one single patient. Further studies employing genome-wide approaches might highlight pathogenic variants in novel genes that explain the still missing heritability of FTD.

scholarly journals Asymmetric TDP pathology in primary progressive aphasia with right hemisphere language dominance

Neurology ◽  
2018 ◽  
Vol 90 (5) ◽  
pp. e396-e403 ◽  
Author(s):  
Garam Kim ◽  
Shahrooz Vahedi ◽  
Tamar Gefen ◽  
Sandra Weintraub ◽  
Eileen H. Bigio ◽  
...  
Keyword(s):  
Right Hemisphere ◽  
Primary Progressive Aphasia ◽  
Cortical Atrophy ◽  
Progressive Aphasia ◽  
Language Dominance ◽  
Primary Progressive ◽  
Activated Microglia ◽  
Unbiased Stereology ◽  
Cortical Regions ◽  
The Right

ObjectiveTo quantitatively examine the regional densities and hemispheric distribution of the 43-kDa transactive response DNA-binding protein (TDP-43) inclusions, neurons, and activated microglia in a left-handed patient with right hemisphere language dominance and logopenic-variant primary progressive aphasia (PPA).MethodsPhosphorylated TDP-43 inclusions, neurons, and activated microglia were visualized with immunohistochemical and histologic methods. Markers were quantified bilaterally with unbiased stereology in language- and memory-related cortical regions.ResultsClinical MRI indicated cortical atrophy in the right hemisphere, mostly in the temporal lobe. Significantly higher densities of TDP-43 inclusions were present in right language-related temporal regions compared to the left or to other right hemisphere regions. The memory-related entorhinal cortex (ERC) and language regions without significant atrophy showed no asymmetry. Activated microglia displayed extensive asymmetry (R > L). A substantial density of neurons remained in all areas and showed no hemispheric asymmetry. However, perikaryal size was significantly smaller in the right hemisphere across all regions except the ERC. To demonstrate the specificity of this finding, sizes of residual neurons were measured in a right-handed case with PPA and were found to be smaller in the language-dominant left hemisphere.ConclusionsThe distribution of TDP-43 inclusions and microglial activation in right temporal language regions showed concordance with anatomic distribution of cortical atrophy and clinical presentation. The results revealed no direct relationship between density of TDP-43 inclusions and activated microglia. Reduced size of the remaining neurons is likely to contribute to cortical atrophy detected by MRI. These findings support the conclusion that there is no obligatory relationship between logopenic PPA and Alzheimer pathology.

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