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Neurological Manifestations of Vascular Dementia

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Role of the metabolism of branched-chain amino acids in the development of Alzheimer's disease and other metabolic disorders.

Baruh Polis, Abraham O Samson,

Alzheimer's disease is an incurable chronic neurodegenerative disorder and the leading cause of dementia, imposing a growing economic burden upon society. The disease progression is associated with gradual deposition of amyloid plaques and the formation of neurofibrillary tangles within the brain parenchyma, yet severe dementia is the culminating phase of ... Read more >>

Neural Regen Res (Neural regeneration research)
[2020, 15(8):1460-1470]

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Cerebral Small Vessel Disease.

Natalia S Rost, Mark Etherton,

PURPOSE OF REVIEW:This article reviews the clinical significance and neuroimaging characteristics of cerebral small vessel disease and the impact on neurologic disease and current and potential therapeutic approaches. RECENT FINDINGS:Cerebral small vessel disease is increasingly prevalent and highly heterogeneous in neuroimaging and clinical presentation. Small subcortical infarcts, lacunes, cerebral microbleeds, ... Read more >>

Continuum (Minneap Minn) (Continuum (Minneapolis, Minn.))
[2020, 26(2):332-352]

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Homozygous NOTCH3 p.R587C mutation in Chinese patients with CADASIL: a case report.

Ruojie He, Huan Li, Yiming Sun, Menglong Chen, Liang Wang, Yuling Zhu, Cheng Zhang,

BACKGROUND:Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited small vessel disease caused by mutations in NOTCH3 gene with remarkable phenotypic heterogeneity. Cases of CADASIL associated with homozygous NOTCH3 mutations are rare and subsequently understudied. In this study, we investigate the genetic and phenotypic features within ... Read more >>

BMC Neurol (BMC neurology)
[2020, 20(1):72]

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Editorial: Cerebral Small Vessel Diseases: From Vessel Alterations to Cortical Parenchymal Injury.

Susanne J van Veluw, Eric Jouvent, Andreas Charidimou,

Front Neurol (Frontiers in neurology)
[2020, 11:92]

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Differential clinicopathological features of EGPA-associated neuropathy with and without ANCA.

Ryoji Nishi, Haruki Koike, Ken Ohyama, Yuki Fukami, Shohei Ikeda, Yuichi Kawagashira, Masahiro Iijima, Masahisa Katsuno, Gen Sobue,

OBJECTIVE:To investigate the clinicopathologic features of eosinophilic granulomatosis with polyangiitis (EGPA)-associated neuropathy with a focus on the presence or absence of anti-neutrophil cytoplasmic antibodies (ANCAs). METHODS:We examined the clinical features and pathologic findings of sural nerve biopsy specimens from 82 patients with EGPA-associated neuropathy. Of these patients, 32.9% were myeloperoxidase ... Read more >>

Neurology (Neurology)
[2020, :]

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Low Vitamin B12 Levels: An Underestimated Cause Of Minimal Cognitive Impairment And Dementia.

Shazia Jatoi, Abdul Hafeez, Syeda Urooj Riaz, Aijaz Ali, Muhammad Ishaq Ghauri, Maham Zehra,

Background Vitamin B12 deficiency is linked to impaired cognition and memory along with a sensation of tingling and numbness, an outcome of poor myelination. Elevated methylmalonic acid and serum homocysteine levels are markers of Vitamin B12 deficiency. Elevated homocysteine levels are also often associated with Alzheimer's disease and stroke. We ... Read more >>

Cureus (Cureus)
[2020, 12(2):e6976]

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Cefepime-Induced Neurotoxicity Presenting with Nonconvulsive Status Epilepticus Admitted as a Stroke Alert.

John M Cunningham, Katherine V Sachs, Rebecca Allyn,

BACKGROUND Cefepime-induced neurotoxicity has been described in intensive care units (ICUs) and neuro ICU settings, occurring in patients started on cefepime for management of severe infections and sepsis. Most cases occur within 1 to 10 days after starting the drug. We publish a case that occurred on the general medical ... Read more >>

Am J Case Rep (The American journal of case reports)
[2020, 21:e921643]

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Monogenic cerebral small-vessel diseases: diagnosis and therapy. Consensus recommendations of the European Academy of Neurology.

M Mancuso, M Arnold, A Bersano, A Burlina, H Chabriat, S Debette, C Enzinger, A Federico, A Filla, J Finsterer, D Hunt, S Lesnik Oberstein, E Tournier-Lasserve, H S Markus,

BACKGROUND AND PURPOSE:Guidelines on monogenic cerebral small-vessel disease (cSVD) diagnosis and management are lacking. Endorsed by the Stroke and Neurogenetics Panels of the European Academy of Neurology, a group of experts has provided recommendations on selected monogenic cSVDs, i.e. cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), cerebral ... Read more >>

Eur. J. Neurol. (European journal of neurology)
[2020, :]

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Atherosclerosis Can Be Mitochondrial: A Review.

Josef Finsterer,

One of the systems that are potentially affected in mitochondrial disorders, but hardly get systematically investigated, are the arteries. One of the phenotypic manifestations in arteries is atherosclerosis. This review focuses on the current knowledge and recent advances of mitochondrial atherosclerosis. We conducted a systematic literature review via PubMed using ... Read more >>

Cureus (Cureus)
[2020, 12(2):e6987]

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Alzheimer's disease, neural stem cells and neurogenesis: cellular phase at single-cell level.

Mehmet Ilyas Cosacak, Prabesh Bhattarai, Caghan Kizil,

Alzheimer's disease cannot be cured as of yet. Our current understanding on the causes of Alzheimer's disease is limited. To develop treatments, experimental models that represent a particular cellular phase of the disease and more rigorous scrutiny of the cellular pathological mechanisms are crucial. In recent years, Alzheimer's disease research ... Read more >>

Neural Regen Res (Neural regeneration research)
[2020, 15(5):824-827]

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A Patient with Combined CADASIL and MTHFR Homozygosity.

Sidonie Ibrikji, Tarek El Halabi, Bassem Yamout,

Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) is an inherited disorder caused by a mutation in the NOTCH 3 gene, characterized by early onset of subcortical lacunar infarcts in the absence of vascular risk factors and cerebral microbleeds. Homozygosity for the factor Methylenetetrahydrofolate Reductase (MTHFR) is also ... Read more >>

Case Rep Neurol Med (Case reports in neurological medicine)
[2020, 2020:4980847]

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Phenotypic characterization of CADASIL patients with the Arg332Cys mutation in the NOTCH3.

Chen-Si Li, Tian-Wei Wang, Jie Wang, Shuai-Hong Li, Na Li, Xiao-Shuang Wang, Le Fang,

Background:Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary vascular disease caused by mutations in NOTCH3, that are primarily localized in exons 4, 3, and 11. The Arg332Cys mutation in exon 6 has been rarely reported in patients with CADASIL. Methods:A case study and the results ... Read more >>

Ann Transl Med (Annals of translational medicine)
[2020, 8(1):10]

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Predictors of cognitive impairment in Parkinson's disease: a systematic review and meta-analysis of prospective cohort studies.

Yu Guo, Feng-Tao Liu, Xiao-He Hou, Jie-Qiong Li, Xi-Peng Cao, Lan Tan, Jian Wang, Jin-Tai Yu,

INTRODUCTION:Cognitive impairment is a debilitating manifestation in Parkinson's disease (PD). We sought to investigate predictors of PD-CI (PD with cognitive impairment). METHODS:We systematically searched PubMed and Cochrane Library for prospective cohort studies and pooled estimates via random-effects models. Primary analyses for all types of cognitive impairments and subgroup analyses by ... Read more >>

J. Neurol. (Journal of Neurology)
[2020, :]

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Biological signatures of Alzheimer Disease.

Poornima Sharma, Anjali Sharma, Faizana Fayaz, Sharad Wakode, Faheem Hyder Pottoo,

Alzheimer disease (AD) is the most prevalent and severe neurodegenerative disease affecting more than 0.024 billion people globally, more common in women as compared to men. Senile plaques and amyloid deposition is one of the main causes of AD, amyloid deposition isconsidered as a central event which induces the link ... Read more >>

Curr Top Med Chem (Current topics in medicinal chemistry)
[2020, :]

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Rapid Eye Movement Sleep Behavior Disorder and Neurodegenerative Diseases: An Update.

Feng Zhang, Long Niu, Xinyao Liu, Yufei Liu, Song Li, Huan Yu, Weidong Le,

Rapid eye movement sleep behavior disorder (RBD) is a sleep behavior disorder characterized by abnormal behaviors and loss of muscle atonia during rapid eye movement (REM) sleep. RBD is generally considered to be associated with synucleinopathies, such as Parkinson’s disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy ... Read more >>

Aging Dis (Aging and Disease)
[2020, 11(2):315-326]

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Delusional Parasitosis in a Patient with an Infarction in the Territory of the Right Posterior Cerebral Artery.

Tae Hwan Yoon, Tae-Beom Ahn,

Dement Neurocogn Disord (Dementia and neurocognitive disorders)
[2019, 18(4):149-151]

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Hemichorea Associated With Non-ketotic Hyperglycemia: A Case Report and Literature Review.

Wei Zheng, Lin Chen, Jian-Hao Chen, Xiang Lin, Yi Tang, Xiao-Juan Lin, Jing Wu, Zhao-Min Lin, Jing-Yuan Lin,

Objective: To explore the clinical manifestation, diagnosis, therapy, and mechanism of hemichorea associated with non-ketotic hyperglycemia (HC-NH) so as to enhance awareness and avoid misdiagnosis or missed diagnosis of the disease. Methods: A case of HC-NH was reported and reviewed in terms of the clinical features, diagnosis and treatment. Results: ... Read more >>

Front Neurol (Frontiers in neurology)
[2020, 11:96]

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Progressive Supranuclear Palsy-Parkinsonism Predominant (PSP-P)-A Clinical Challenge at the Boundaries of PSP and Parkinson's Disease (PD).

Piotr Alster, Natalia Madetko, Dariusz Koziorowski, Andrzej Friedman,

Progressive Supranuclear Palsy (PSP) and Parkinson's Disease (PD), especially in their early stages, show overlapping clinical manifestations. The criteria for the diagnosis of PSP, released in 2017, indicate four basic features of the disease-postural instability (P), akinesia (A), oculomotor dysfunction (O) and cognitive and lingual disorders (C), which clarify the ... Read more >>

Front Neurol (Frontiers in Neurology)
[2020, 11:180]

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[White matter hyperintensities in ageing: Pathophysiology, associated cognitive disorders and prevention].

A Garnier-Crussard, V Desestret, F Cotton, G Chételat, P Krolak-Salmon,

White matter hyperintensities (WMH), also known as leukoaraïosis are very common neuroradiological manifestations in the elderly. The main risk factors for WMH are age and high blood pressure. The vascular origin of these lesions is classically accepted and WMH are considered as one feature of the small vessel disease. WMH ... Read more >>

Rev Med Interne (La Revue de medecine interne)
[2020, :]

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Pathophysiological Mechanisms and Potential Therapeutic Targets in Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy (CADASIL).

Martina Locatelli, Alessandro Padovani, Alessandro Pezzini,

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), is a hereditary small-vessels angiopathy caused by mutations in the NOTCH 3 gene, located on chromosome 19, usually affecting middle-ages adults, whose clinical manifestations include migraine with aura, recurrent strokes, mood disorders, and cognitive impairment leading to dementia and disability. ... Read more >>

Front Pharmacol (Frontiers in pharmacology)
[2020, 11:321]

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MELAS Missed for Years: Stroke-Like Lesions Are No Indication for Brain Biopsy.

J Finsterer,

A 56-year-old female with a history of chronic alcoholism until age 38 y with a relapse between ages 45 and 46 y developed seizures, psychosis, and hemianopia to the left at age 46 y. Imaging revealed a right parieto-occipital lesion with intralesional bleeding. Five months after the first lesion she developed a second ... Read more >>

Case Rep Neurol Med (Case reports in neurological medicine)
[2019, 2019:9312451]

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Riluzole: a therapeutic strategy in Alzheimer's disease by targeting the WNT/β-catenin pathway.

Alexandre Vallée, Jean-Noël Vallée, Rémy Guillevin, Yves Lecarpentier,

Alzheimer's disease (AD) is a neurodegenerative disease, where the etiology remains unclear. AD is characterized by amyloid-(Aβ) protein aggregation and neurofibrillary plaques deposits. Oxidative stress and chronic inflammation have been suggested as causes of AD. Glutamatergic pathway dysregulation is also mainly associated with AD process. In AD, the canonical WNT/β-catenin ... Read more >>

Aging (Albany NY) (Aging)
[2020, 12(3):3095-3113]

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Ethnic Variation in the Manifestation of Parkinson's Disease: A Narrative Review.

Aaron Ben-Joseph, Charles R Marshall, Andrew J Lees, Alastair J Noyce,

The global prevalence of Parkinson's disease is increasing, yet the characteristics, risk factors and genetics of PD in Black, Asian and Hispanic populations is little understood. In this paper we review the published literature on clinical variation in the symptoms and signs of Parkinson's disease in different ethnic groups and ... Read more >>

J Parkinsons Dis (Journal of Parkinson's disease)
[2020, 10(1):31-45]

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Globular Glial Tauopathy Type I Presenting as Atypical Progressive Aphasia, With Comorbid Limbic-Predominant Age-Related TDP-43 Encephalopathy.

Robert Rusina, Zsolt Csefalvay, Gabor G Kovacs, Jiri Keller, Alena Javurkova, Radoslav Matej,

Globular glial tauopathies (GGTs) have heterogeneous presentations with little available information regarding typical clinical manifestations. We report on a case of atypical primary progressive aphasia (PPA) due to comorbid GGT and limbic transactive response DNA binding protein of 43 kDa (TDP-43) proteinopathy. The initial clinical phenotype was compatible with the ... Read more >>

Front Aging Neurosci (Frontiers in Aging Neuroscience)
[2019, 11:336]

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Current Biomedical Use of Copper Chelation Therapy.

Silvia Baldari, Giuliana Di Rocco, Gabriele Toietta,

Copper is an essential microelement that plays an important role in a wide variety of biological processes. Copper concentration has to be finely regulated, as any imbalance in its homeostasis can induce abnormalities. In particular, excess copper plays an important role in the etiopathogenesis of the genetic disease Wilson's syndrome, ... Read more >>

Int J Mol Sci (International journal of molecular sciences)
[2020, 21(3):]

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