Can Medial Temporal Impairment Be an Imaging Red Flag for Neurodegeneration in Disproportionately Enlarged Subarachnoid Space Hydrocephalus?

2021 ◽  
pp. 1-11
Author(s):  
Keita Sakurai ◽  
Daita Kaneda ◽  
Yuto Uchida ◽  
Shohei Inui ◽  
Masahiko Bundo ◽  
...  
Keyword(s):  
Neurodegenerative Diseases ◽  
Subarachnoid Space ◽  
Lewy Bodies ◽  
Normal Pressure ◽  
Temporal Horn ◽  
Red Flag ◽  
Argyrophilic Grain ◽  
Magnetic Resonance Imaging Mri ◽  
Specific Symptoms ◽  
Multiple Cerebral Infarctions

Background: The differentiation of idiopathic normal pressure hydrocephalus (iNPH) from neurodegenerative diseases such as Alzheimer’s disease (AD) and dementia with Lewy bodies (DLB) is often challenging because of their non-specific symptoms. Therefore, various neuroradiological markers other than ventriculomegaly have been proposed. Despite the utility of disproportionately enlarged subarachnoid-space hydrocephalus (DESH) for the appropriate selection of shunt surgery candidates, the specificity and neuropathology of this finding have not been sufficiently evaluated. Objective: Investigation of the clinicopathological features and comparison of the neuroradiological findings between DESH with postmortem neuropathological diagnoses (pDESH) and clinically-diagnosed iNPH (ciNPH) patients are the main purposes of this study. Method: In addition to the retrospective evaluation of clinicopathological information, quantitative, semiquantitative, and qualitative magnetic resonance imaging (MRI) indices were compared between pathologically-investigated 10 patients with pDESH and 10 patients with ciNPH Results: Excluding one patient with multiple cerebral infarctions, the postmortem neuropathological diagnoses of the pathologically-investigated patients were mainly neurodegenerative diseases (five AD, one DLB with AD pathologies, one DLB, one argyrophilic grain disease, and one Huntington’s disease). In addition to the common neuroradiological features Conclusion: Hippocampal atrophy and deformation with temporal horn enlargement seem to be characteristic neuroradiological findings of long-standing severely demented patients with DESH and neurodegenerative diseases, mainly advanced-stage AD.

scholarly journals The association of disproportionately enlarged subarachnoid space hydrocephalus with cognitive deficit in a general population: the Ohasama study

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Tomofumi Nishikawa ◽  
Ichiro Akiguchi ◽  
Michihiro Satoh ◽  
Azusa Hara ◽  
Mikio Hirano ◽  
...  
Keyword(s):  
Subarachnoid Space ◽  
Cognitive Deficit ◽  
Large Population ◽  
Positive Association ◽  
Normal Pressure ◽  
Population Based ◽  
Logistic Regression Models ◽  
New Development ◽  
Evans Index ◽  
Magnetic Resonance Imaging Mri

AbstractDisproportionately enlarged subarachnoid space hydrocephalus (DESH) is the characteristic feature of idiopathic normal pressure hydrocephalus. We aimed to characterize the prevalence, development, and association of DESH to cognitive deficit in a large population. We reviewed the data of 1384 subjects eligible for the present study among 1590 participants who underwent magnetic resonance imaging (MRI) in the Ohasama Study, a population-based study in Ohasama, Japan. The participants with Mini-Mental State Examination (MMSE) score <  = 25 were assumed to have cognitive deficit and DESH was evaluated by reviewing the MRIs. We assessed the association between DESH, Evans index (EI), and cognitive deficit using multivariate logistic regression models adjusted for relevant confounders. Furthermore, we evaluated the new development of DESH and the deterioration of cognitive function in the participants with DESH. There were nine participants with DESH (0.65%), seven of whom showed cognitive deficit. DESH was significantly associated with cognitive deficit in multivariate regression analyses (odds ratio; 8.50 [95% confidence interval: 1.61–44.88]). In the 669 participants who underwent follow-up MRI, we found four participants newly presenting with DESH; the development of DESH was observed before/after the presence of EI > 0.3. We also found two participants with existing DESH showing no remarkable worsening in MMSE and EI. The present study demonstrated a positive association between the presence of DESH and cognitive deficit. DESH can develop independently of EI > 0.3, and ventricular enlargement in combination with DESH may be an important factor in the worsening of cognitive deficit.

scholarly journals Cross-platform transcriptional profiling identifies common and distinct molecular pathologies in Lewy body diseases

2021 ◽  
Author(s):  
Rahel Feleke ◽  
Regina H. Reynolds ◽  
Amy M. Smith ◽  
Bension Tilley ◽  
Sarah A. Gagliano Taliun ◽  
...  
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Neurodegenerative Diseases ◽  
Lewy Body ◽  
Molecular Mechanisms ◽  
Lewy Bodies ◽  
Subsequent Development ◽  
Lewy Body Dementia ◽  
Anterior Cingulate ◽  
Lewy Body Diseases

AbstractParkinson’s disease (PD), Parkinson’s disease with dementia (PDD) and dementia with Lewy bodies (DLB) are three clinically, genetically and neuropathologically overlapping neurodegenerative diseases collectively known as the Lewy body diseases (LBDs). A variety of molecular mechanisms have been implicated in PD pathogenesis, but the mechanisms underlying PDD and DLB remain largely unknown, a knowledge gap that presents an impediment to the discovery of disease-modifying therapies. Transcriptomic profiling can contribute to addressing this gap, but remains limited in the LBDs. Here, we applied paired bulk-tissue and single-nucleus RNA-sequencing to anterior cingulate cortex samples derived from 28 individuals, including healthy controls, PD, PDD and DLB cases (n = 7 per group), to transcriptomically profile the LBDs. Using this approach, we (i) found transcriptional alterations in multiple cell types across the LBDs; (ii) discovered evidence for widespread dysregulation of RNA splicing, particularly in PDD and DLB; (iii) identified potential splicing factors, with links to other dementia-related neurodegenerative diseases, coordinating this dysregulation; and (iv) identified transcriptomic commonalities and distinctions between the LBDs that inform understanding of the relationships between these three clinical disorders. Together, these findings have important implications for the design of RNA-targeted therapies for these diseases and highlight a potential molecular “window” of therapeutic opportunity between the initial onset of PD and subsequent development of Lewy body dementia.

scholarly journals Differential diagnosis of Alzheimer’s disease using spectrochemical analysis of blood

2017 ◽  
Vol 114 (38) ◽  
pp. E7929-E7938 ◽  
Author(s):  
Maria Paraskevaidi ◽  
Camilo L. M. Morais ◽  
Kássio M. G. Lima ◽  
Julie S. Snowden ◽  
Jennifer A. Saxon ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Neurodegenerative Diseases ◽  
Sensitivity And Specificity ◽  
Lewy Bodies ◽  
Total Reflection ◽  
Attenuated Total Reflection ◽  
Repeated Measurements ◽  
Healthy Individuals ◽  
Different Types

The progressive aging of the world’s population makes a higher prevalence of neurodegenerative diseases inevitable. The necessity for an accurate, but at the same time, inexpensive and minimally invasive, diagnostic test is urgently required, not only to confirm the presence of the disease but also to discriminate between different types of dementia to provide the appropriate management and treatment. In this study, attenuated total reflection FTIR (ATR-FTIR) spectroscopy combined with chemometric techniques were used to analyze blood plasma samples from our cohort. Blood samples are easily collected by conventional venepuncture, permitting repeated measurements from the same individuals to monitor their progression throughout the years or evaluate any tested drugs. We included 549 individuals: 347 with various neurodegenerative diseases and 202 age-matched healthy individuals. Alzheimer’s disease (AD;n= 164) was identified with 70% sensitivity and specificity, which after the incorporation of apolipoprotein ε4 genotype (APOEε4) information, increased to 86% when individuals carried one or two alleles of ε4, and to 72% sensitivity and 77% specificity when individuals did not carry ε4 alleles. Early AD cases (n= 14) were identified with 80% sensitivity and 74% specificity. Segregation of AD from dementia with Lewy bodies (DLB;n= 34) was achieved with 90% sensitivity and specificity. Other neurodegenerative diseases, such as frontotemporal dementia (FTD;n= 30), Parkinson’s disease (PD;n= 32), and progressive supranuclear palsy (PSP;n= 31), were included in our cohort for diagnostic purposes. Our method allows for both rapid and robust diagnosis of neurodegeneration and segregation between different dementias.

scholarly journals Neuroimaging in normal pressure hydrocephalus

2015 ◽  
Vol 9 (4) ◽  
pp. 350-355 ◽  
Author(s):  
Benito Pereira Damasceno
Keyword(s):  
Normal Pressure Hydrocephalus ◽  
Cerebral Atrophy ◽  
High Sensitivity ◽  
Normal Pressure ◽  
Evans Index ◽  
Flow Void ◽  
High Positive Predictive Value ◽  
Subarachnoid Spaces ◽  
Magnetic Resonance Imaging Mri ◽  
Improved Accuracy

ABSTRACT Normal pressure hydrocephalus (NPH) is a syndrome characterized by the triad of gait disturbance, mental deterioration and urinary incontinence, associated with ventriculomegaly and normal cerebrospinal fluid (CSF) pressure. The clinical presentation (triad) may be atypical or incomplete, or mimicked by other diseases, hence the need for supplementary tests, particularly to predict postsurgical outcome, such as CSF tap-tests and computed tomography (CT) or magnetic resonance imaging (MRI). The CSF tap-test, especially the 3 to 5 days continuous external lumbar drainage of at least 150 ml/day, is the only procedure that simulates the effect of definitive shunt surgery, with high sensitivity (50-100%) and high positive predictive value (80-100%). According to international guidelines, the following are CT or MRI signs decisive for NPH diagnosis and selection of shunt-responsive patients: ventricular enlargement disproportionate to cerebral atrophy (Evans index >0.3), and associated ballooning of frontal horns; periventricular hyperintensities; corpus callosum thinning and elevation, with callosal angle between 40º and 90º; widening of temporal horns not fully explained by hippocampal atrophy; and aqueductal or fourth ventricular flow void; enlarged Sylvian fissures and basal cistern, and narrowing of sulci and subarachnoid spaces over the high convexity and midline surface of the brain. On the other hand, other imaging methods such as radionuclide cisternography, SPECT, PET, and also DTI or resting-state functional MRI, although suitable for NPH diagnosis, do not yet provide improved accuracy for identifying shunt-responsive cases.

Normal Pressure Hydrocephalus as a Possible Reversible Cause of Dementia, Neuroimaging Findings

2017 ◽  
Vol 41 (S1) ◽  
pp. S629-S630 ◽  
Author(s):  
A. Zacharzewska-Gondek ◽  
T. Gondek ◽  
M. Sąsiadek ◽  
J. Bladowska
Keyword(s):  
Normal Pressure Hydrocephalus ◽  
Brain Atrophy ◽  
Correct Diagnosis ◽  
Normal Pressure ◽  
Third Ventriculostomy ◽  
Imaging Features ◽  
Central Brain ◽  
Flow Void ◽  
Competing Interest ◽  
Magnetic Resonance Imaging Mri

IntroductionNormal pressure hydrocephalus (NPH) occurs in 0.5% of persons over 65 years old. The etiology of NPH is still unknown. Clinically NPH is characterised by cognitive deterioration, gait impairment and urinary incontinence. NPH is a possible reversible cause of dementia. Neuroimaging techniques such as computed tomography (CT) and magnetic resonance imaging (MRI) allow to assess typical brain changes in this disorder.The objectives are to present the typical findings of NPH on CT and MRI and to demonstrate differences between NPH and central brain atrophy in neuroimaging.ResultsThe imaging features of NPH include: supratentorial ventriculomegaly with callosal angle less than 90o, tight sulci at the vertex and considerable out of proportion enlargement of Sylvian fissures. In case of central brain atrophy there may be a predominance of ventriculomegaly and/or widened sulci without crowding of the gyri at the vertex and callosal angle greater than 90o. In both entities, the decrease of density in periventricular region may be seen: in NPH could be a sign of transependymal oedema or in brain atrophy as an accompanying leukoaraiosis. Additionally, it is possible to assess changes in flow of cerebrospinal fluid (CSF) on MRI: in NPH an increased pulsatile CSF circulation in aqueduct as flow void sign may be observed.ConclusionsCorrect diagnosis of NPH on CT or MRI in relation to clinical data is very important. Treatment with ventriculoperitoneal shunt or third ventriculostomy may partially improve the quality of life in some patients with cognitive impairment due to NPH.Disclosure of interestThe authors have not supplied their declaration of competing interest.

Which other disorders cause dementia?

2021 ◽  
pp. 19-30
Author(s):  
Andrew E. Budson ◽  
Maureen K. O’Connor
Keyword(s):  
Vascular Dementia ◽  
Normal Pressure Hydrocephalus ◽  
Dementia With Lewy Bodies ◽  
Lewy Bodies ◽  
Primary Progressive Aphasia ◽  
Normal Pressure ◽  
Behavioral Variant Frontotemporal Dementia ◽  
Severe Stage ◽  
Language Behavior ◽  
Symptoms And Signs

In addition to Alzheimer’s disease, other brain disorders of aging that affect thinking and memory include vascular dementia, dementia with Lewy bodies, Parkinson’s disease dementia, behavioral variant frontotemporal dementia, primary progressive aphasia that has logpenic, semantic, and non-fluent agrammatic variants, and normal pressure hydrocephalus. Each produces characteristic changes in thinking, memory, language, behavior, and/or movement that allow you and the doctor to know when to consider them as possible causes of your loved one’s dementia. Note that the dementia of every individual is unique, so the symptoms and signs that they will manifest are all different. However, when dementias reach the moderate to severe stage, most dementias looks similar, despite having different causes.

Neuropathology

2020 ◽  
pp. 77-98
Author(s):  
Johannes Attems ◽  
Kurt A. Jellinger
Keyword(s):  
Frontotemporal Lobar Degeneration ◽  
Dementia With Lewy Bodies ◽  
Prion Diseases ◽  
Hippocampal Sclerosis ◽  
Neurofibrillary Tangle ◽  
Lewy Bodies ◽  
Corticobasal Degeneration ◽  
Amyloid Angiopathy ◽  
Argyrophilic Grain ◽  
Cerebral Amyloid

This chapter describes the main neuropathological features of the most common age-associated neurodegenerative diseases, including Alzheimer’s disease, Parkinson’s disease, and dementia with Lewy bodies, as well as other less frequent ones such as multiple system atrophy, Pick’s disease, corticobasal degeneration, progressive supranuclear palsy, argyrophilic grain disease, neurofibrillary tangle-dominant dementia, frontotemporal lobar degeneration with TDP-43 pathology, and Huntington’s disease. Likewise, cerebral amyloid angiopathy, hippocampal sclerosis, vascular dementia, and prion diseases are described. A main aim of this chapter is to assist the reader in interpreting neuropathological reports; hence criteria for the neuropathological classifications of the major diseases are provided. One section covers general considerations on neurodegeneration, and basic pathophysiological mechanisms of tau, amyloid-β‎, α‎-synuclein, TDP-43, and prions are briefly described in the sections on the respective diseases. Finally, one section is dedicated to cerebral multimorbidity, and a view on currently emerging neuropathological methods is given.

scholarly journals A Case of Spontaneous Spinal Subdural Hematoma Complicated by Cranial Subarachnoid Hemorrhage and Spinal Adhesive Arachnoiditis

2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Taihei Go ◽  
Toshiyuki Tsutsui ◽  
Yasuaki Iida ◽  
Katsunori Fukutake ◽  
Ryoichi Fukano ◽  
...  
Keyword(s):  
Back Pain ◽  
Subarachnoid Hemorrhage ◽  
Subdural Hematoma ◽  
Subarachnoid Space ◽  
Clinical Symptoms ◽  
Neurological Manifestation ◽  
Subdural Space ◽  
Spinal Subdural Hematoma ◽  
Magnetic Resonance Imaging Mri ◽  
To Come

A 76-year-old woman with a spinal subdural hematoma (SDH) was presented with severe back pain without headache. Magnetic resonance imaging (MRI) performed 4 days after onset showed SDH extending from Th2 to L3. She was diagnosed with spontaneous SDH without neurological manifestation, and conservative treatment was selected. Transient disturbance of orientation appeared 7 days after onset. Small subarachnoid hemorrhage (SAH) was detected on head CT, and strict antihypertensive therapy was started. Symptoms changed for the better. Back pain disappeared 4 weeks after onset. On follow-up MRI at 6 months after onset, the SDH had been resolved spontaneously. Although adhesive arachnoiditis was observed at Th4-6, the recurrence of clinical symptoms was not observed at one year and a half after onset. Spinal subdural space is almost avascular; a hematoma in a subdural space is considered to come from a subarachnoid space when it is a lot. A hemorrhage in subarachnoid space was flushed by cerebral spinal fluid; hematoma or arachnoiditis was not formed in general. In our case, hemorrhage was a lot and expansion of SDH was large enough to cause cranial SAH and arachnoiditis. But longitudinally expanded SDH did not show neurological manifestation and resolved spontaneously in our case.

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