Antibodies to neuronal surface antigens in patients with a clinical diagnosis of neurodegenerative disorder

2021 ◽  
Author(s):  
Maria Pia Giannoccaro ◽  
Matteo Gastaldi ◽  
Giovanni Rizzo ◽  
Leslie Jacobson ◽  
Veria Vacchiano ◽  
...  
Keyword(s):  
Clinical Diagnosis ◽  
Neurodegenerative Disorder ◽  
Surface Antigens

Parkinson Disease: Epidemiology, Pathology, and Clinical Diagnosis

2016 ◽  
Author(s):  
Maurizio Zibetti ◽  
Aristide Merola
Keyword(s):  
Parkinson Disease ◽  
Clinical Diagnosis ◽  
Genetic Basis ◽  
Neurodegenerative Disorder ◽  
Atypical Parkinsonism ◽  
Braak Staging ◽  
Disease Epidemiology ◽  
The Past ◽  
Substantial Progress ◽  
Near Future

Parkinson disease (PD) is the second most common neurodegenerative disorder after Alzheimer disease. PD predominantly affects elderly people, and most countries are now facing a marked demographic change, with a progressively larger proportion of the population reaching old age. Thus, PD is expected to cause an increasing social and economic burden on society in the near future. The causes of PD are still mostly unknown. There has been a substantial progress in our understanding of the genetic basis of PD in the past 15 years; however, these mutations explain only a small fraction of all PD cases and other non-genetic factors may play a role. This review covers the epidemiology, pathology, and clinical diagnosis of PD. Figures show age-specific incidence of PD, age-specific prevalence of PD, PD risks for exposures reported in high-quality studies, PD and atypical parkinsonism clinical features, and neuroimaging alterations in PD and atypical parkinsonism. Tables list factors associated with increased or decreased risk of PD, Braak staging scheme of α-synuclein pathology in PD, United Kingdom Parkinson’s Disease Society Brain Bank clinical diagnostic for PD, National Institute of Neurological Disorders and Stroke diagnostic criteria for PD, differential diagnosis of parkinsonian syndromes, and “atypical” atypical parkinsonism.

Parkinson Disease: Epidemiology, Pathology, and Clinical Diagnosis

2016 ◽  
Author(s):  
Maurizio Zibetti ◽  
Aristide Merola
Keyword(s):  
Parkinson Disease ◽  
Clinical Diagnosis ◽  
Genetic Basis ◽  
Neurodegenerative Disorder ◽  
Atypical Parkinsonism ◽  
Braak Staging ◽  
Disease Epidemiology ◽  
The Past ◽  
Substantial Progress ◽  
Near Future

Parkinson disease (PD) is the second most common neurodegenerative disorder after Alzheimer disease. PD predominantly affects elderly people, and most countries are now facing a marked demographic change, with a progressively larger proportion of the population reaching old age. Thus, PD is expected to cause an increasing social and economic burden on society in the near future. The causes of PD are still mostly unknown. There has been a substantial progress in our understanding of the genetic basis of PD in the past 15 years; however, these mutations explain only a small fraction of all PD cases and other non-genetic factors may play a role. This review covers the epidemiology, pathology, and clinical diagnosis of PD. Figures show age-specific incidence of PD, age-specific prevalence of PD, PD risks for exposures reported in high-quality studies, PD and atypical parkinsonism clinical features, and neuroimaging alterations in PD and atypical parkinsonism. Tables list factors associated with increased or decreased risk of PD, Braak staging scheme of α-synuclein pathology in PD, United Kingdom Parkinson’s Disease Society Brain Bank clinical diagnostic for PD, National Institute of Neurological Disorders and Stroke diagnostic criteria for PD, differential diagnosis of parkinsonian syndromes, and “atypical” atypical parkinsonism.

scholarly journals Antibody-associated CNS syndromes without signs of inflammation in the elderly

Neurology ◽  
2017 ◽  
Vol 89 (14) ◽  
pp. 1471-1475 ◽  
Author(s):  
Domingo Escudero ◽  
Mar Guasp ◽  
Helena Ariño ◽  
Carles Gaig ◽  
Eugenia Martínez-Hernández ◽  
...  
Keyword(s):  
Neurodegenerative Disorder ◽  
Brain Mri ◽  
Clinical Manifestations ◽  
Memory Loss ◽  
Autoimmune Encephalitis ◽  
Surface Antigens ◽  
Clinical Analysis ◽  
Correct Identification ◽  
Cognitive Deterioration ◽  
Antibody Testing

Objective:To report the CNS syndromes of patients ≥60 years of age with antibodies against neuronal surface antigens but no evidence of brain MRI and CSF inflammatory changes.Methods:This was a retrospective clinical analysis of patients with antibodies against neuronal surface antigens who fulfilled 3 criteria: age ≥60 years, no inflammatory abnormalities in brain MRI, and no CSF pleocytosis. Antibodies were determined with reported techniques.Results:Among 155 patients ≥60 years of age with neurologic syndromes related to antibodies against neuronal surface antigens, 35 (22.6%) fulfilled the indicated criteria. The median age of these 35 patients was 68 years (range 60–88 years). Clinical manifestations included faciobrachial dystonic seizures (FBDS) in 11 of 35 (31.4%) patients, all with LGI1 antibodies; a combination of gait instability, brainstem dysfunction, and sleep disorder associated with IgLON5 antibodies in 10 (28.6%); acute confusion, memory loss, and behavioral changes suggesting autoimmune encephalitis (AE) in 9 (25.7%; 2 patients with AMPAR, 2 with NMDAR, 2 with GABAbR, 2 with LGI1, and 1 with CASPR2 antibodies); and rapidly progressive cognitive deterioration in 5 (14.3%; 3 patients with IgLON5 antibodies, 1 with chorea; 1 with DPPX antibody–associated cerebellar ataxia and arm rigidity; and 1 with CASPR2 antibodies).Conclusions:In patients ≥60 years of age, the correct identification of characteristic CNS syndromes (FBDS, anti-IgLON5 syndrome, AE) should prompt antibody testing even without evidence of inflammation in MRI and CSF studies. Up to 15% of the patients developed rapidly progressive cognitive deterioration, which further complicated the differential diagnosis with a neurodegenerative disorder.

scholarly journals Corticobasal Degeneration Presenting with Depression and Dystonia: A Case Report

2009 ◽  
Vol 24 (S1) ◽  
pp. 1-1
Author(s):  
J. Cerejeira ◽  
R. André ◽  
P. Batista ◽  
P. Carriço ◽  
A.M. Ferreira ◽  
...  
Keyword(s):  
Clinical Diagnosis ◽  
Social Stress ◽  
Neurodegenerative Disorder ◽  
Psychiatric Symptoms ◽  
Corticobasal Degeneration ◽  
Clinical Syndrome ◽  
Stress Factors ◽  
Alien Limb ◽  
Degenerative Disorders ◽  
Severe Motor

Corticobasal degeneration is a rare neurodegenerative disorder affecting both cortex and basal ganglia with clinical and underlying pathological heterogeneity. Although motor features of CBD were emphasized in earlier descriptions psychiatric symptoms, including cognitive impairment and mood disorders, have been consistently reported during the course of the disease. Clinical diagnosis of CBD is challenging and can be difficult to differentiate from other neuropsychiatric disorders with overlapping features. This can lead to significant underdiagnosis of CBD particularly during its early stages.We report a case of a 48 year-old female patient presenting with insidious orofacial dystonia co-occurring with depression which remained controlled for five years. Later, while experiencing major psycho-social stress factors, she presented with a rapidly progressive clinical syndrome compatible with the diagnosis of cortico-basal degeneration with severe motor, cognitive and behavioural symptoms, including alien limb phenomenon, nonfluent aphasia and personality changes. Neuropsychological assessment revealed significant frontal lobe dysfunction and SPECT imaging showed asymetrical fronto-parietal hypoperfusion.This case illustrates the difficulties in the clinical diagnosis of CBD both in early and late stages due to its clinical overlap with mood and movement disorders as well as with Fronto-Temporal Dementia. At the same time, it highlights the influence of psycho-social stress factors in the manifestation of degenerative disorders.

scholarly journals Sporadic Creutzfeldt-Jakob disease: Real-Time Quaking Induced Conversion (RT-QuIC) assay represents a major diagnostic advance

2021 ◽  
Vol 65 (s1) ◽  
Author(s):  
Federico Angelo Cazzaniga ◽  
Edoardo Bistaffa ◽  
Chiara Maria Giulia De Luca ◽  
Giuseppe Bufano ◽  
Antonio Indaco ◽  
...  
Keyword(s):  
Real Time ◽  
Clinical Diagnosis ◽  
Neurodegenerative Disorder ◽  
Infectious Agent ◽  
Structural Rearrangement ◽  
Point Of View ◽  
Cellular Prion Protein ◽  
Peripheral Tissues ◽  
Clinical Onset ◽  
Jakob Disease

Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare and fatal neurodegenerative disorder with an incidence of 1.5 to 2 cases per million population/year. The disease is caused by a proteinaceous infectious agent, named prion (or PrPSc), which arises from the conformational conversion of the cellular prion protein (PrPC). Once formed, PrPSc interacts with the normally folded PrPC coercing it to undergo similar structural rearrangement. The disease is highly heterogeneous from a clinical and neuropathological point of view. The origin of this variability lies in the aberrant structures acquired by PrPSc. At least six different sCJD phenotypes have been described and each of them is thought to be caused by a peculiar PrPSc strain. Definitive sCJD diagnosis requires brain analysis with the aim of identifying intracerebral accumulation of PrPSc which currently represents the only reliable biomarker of the disease. Clinical diagnosis of sCJD is very challenging and is based on the combination of several clinical, instrumental and laboratory tests representing surrogate disease biomarkers. Thanks to the advent of the ultrasensitive Real-Time Quaking-Induced Conversion (RT-QuIC) assay, PrPSc was found in several peripheral tissues of sCJD patients, sometimes even before the clinical onset of the disease. This discovery represents an important step forward for the clinical diagnosis of sCJD. In this manuscript, we present an overview of the current applications and future perspectives of RT-QuIC in the field of sCJD diagnosis.

scholarly journals Contributions of Molecular and Optical Techniques to the Clinical Diagnosis of Alzheimer’s Disease

2020 ◽  
Vol 10 (11) ◽  
pp. 815
Author(s):  
Edoardo Bistaffa ◽  
Fabrizio Tagliavini ◽  
Paolo Matteini ◽  
Fabio Moda
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Clinical Diagnosis ◽  
Neurodegenerative Disorder ◽  
Phenotypic Variability ◽  
Surface Enhanced Raman Spectroscopy ◽  
Reaction Products ◽  
Surface Enhanced Raman ◽  
Amyloid Aβ ◽  
Folded Proteins

Alzheimer’s disease (AD) is the most common neurodegenerative disorder worldwide. The distinctive neuropathological feature of AD is the intracerebral accumulation of two abnormally folded proteins: β-amyloid (Aβ) in the form of extracellular plaques, and tau in the form of intracellular neurofibrillary tangles. These proteins are considered disease-specific biomarkers, and the definite diagnosis of AD relies on their post-mortem identification in the brain. The clinical diagnosis of AD is challenging, especially in the early stages. The disease is highly heterogeneous in terms of clinical presentation and neuropathological features. This phenotypic variability seems to be partially due to the presence of distinct Aβ conformers, referred to as strains. With the development of an innovative technique named Real-Time Quaking-Induced Conversion (RT-QuIC), traces of Aβ strains were found in the cerebrospinal fluid of AD patients. Emerging evidence suggests that different conformers may transmit their strain signature to the RT-QuIC reaction products. In this review, we describe the current challenges for the clinical diagnosis of AD and describe how the RT-QuIC products could be analyzed by a surface-enhanced Raman spectroscopy (SERS)-based systems to reveal the presence of strain signatures, eventually leading to early diagnosis of AD with the recognition of individual disease phenotype.

A pre-embedding, protein a-gold immunolabelling technique for cytocentrifuged cell monolayers for lm and tem

1986 ◽  
Vol 44 ◽  
pp. 220-221
Author(s):  
K. Chien ◽  
I.P. Shintaku ◽  
A.F. Sassoon ◽  
R.L. Van de Velde ◽  
R. Heusser
Keyword(s):  
Cell Surface ◽  
Staining Method ◽  
Protein A ◽  
Surface Antigens ◽  
Cell Suspensions ◽  
Cellular Morphology ◽  
Cellular Phenotype ◽  
Cell Surface Antigens ◽  
Cell Monolayers ◽  
Diagnostic Histopathology

Identification of cellular phenotype by cell surface antigens in conjunction with ultrastructural analysis of cellular morphology can be a useful tool in the study of biologic processes as well as in diagnostic histopathology. In this abstract, we describe a simple pre-embedding, protein A-gold staining method which is designed for cell suspensions combining the handling convenience of slide-mounted cell monolayers and the ability to evaluate specimen staining specificity prior to EM embedding.

Processing Immunoperoxidase Labeled Cell Monolayers and Cryostat Sesctions For Electron Microscopy

1985 ◽  
Vol 43 ◽  
pp. 714-715
Author(s):  
K. Chien ◽  
R. Van de Velde ◽  
I.P. Shintaku ◽  
A.F. Sassoon
Keyword(s):  
Cell Monolayer ◽  
Cell Types ◽  
Surface Antigens ◽  
Immunoperoxidase Method ◽  
Reaction Step ◽  
Hot Plate ◽  
Air Drying ◽  
New Approach ◽  
Cell Monolayers ◽  
Glass Slides

Immunoelectron microscopy of neoplastic lymphoma cells is valuable for precise localization of surface antigens and identification of cell types. We have developed a new approach in which the immunohistochemical staining can be evaluated prior to embedding for EM and desired area subsequently selected for ultrathin sectioning.A freshly prepared lymphoma cell suspension is spun onto polylysine hydrobromide- coated glass slides by cytocentrifugation and immediately fixed without air drying in polylysine paraformaldehyde (PLP) fixative. After rinsing in PBS, slides are stained by a 3-step immunoperoxidase method. Cell monolayer is then fixed in buffered 3% glutaraldehyde prior to DAB reaction. After the DAB reaction step, wet monolayers can be examined under LM for presence of brown reaction product and selected monolayers then processed by routine methods for EM and embedded with the Chien Re-embedding Mold. After the polymerization, the epoxy blocks are easily separated from the glass slides by heatingon a 100°C hot plate for 20 seconds.

Study on erythrocytes by SEM photogrammetry (SEMP) technique

1990 ◽  
Vol 48 (3) ◽  
pp. 724-725
Author(s):  
Tong Wensheng ◽  
Lu Lianhuang ◽  
Zhang Zhijun
Keyword(s):  
Quantitative Analysis ◽  
Cell Membrane ◽  
Clinical Diagnosis ◽  
Human Body ◽  
Blood Cells ◽  
Three Dimensional ◽  
Normal Person ◽  
Blood Disease ◽  
Computation Technique ◽  
Important Basis

This is a combined study of two diffirent branches, photogrammetry and morphology of blood cells. The three dimensional quantitative analysis of erythrocytes using SEMP technique, electron computation technique and photogrammetry theory has made it possible to push the study of mophology of blood cells from LM, TEM, SEM to a higher stage, that of SEM P. A new path has been broken for deeply study of morphology of blood cells.In medical view, the abnormality of the quality and quantity of erythrocytes is one of the important changes of blood disease. It shows the abnormal blood—making function of the human body. Therefore, the study of the change of shape on erythrocytes is the indispensable and important basis of reference in the clinical diagnosis and research of blood disease.The erythrocytes of one normal person, three PNH Patients and one AA patient were used in this experiment. This research determines the following items: Height;Length of two axes (long and short), ratio; Crevice in depth and width of cell membrane; Circumference of erythrocytes; Isoline map of erythrocytes; Section map of erythrocytes.

Auditory Dysfunction in Alzheimer's Disease

2010 ◽  
Vol 15 (1) ◽  
pp. 4-11 ◽  
Author(s):  
Sridhar Krishnamurti
Keyword(s):  
Alzheimer’S Disease ◽  
Older Adults ◽  
Alzheimer's Disease ◽  
Health Care ◽  
Care Setting ◽  
Neurodegenerative Disorder ◽  
Disease Process ◽  
Clinical Protocols ◽  
Speech Language Pathologist ◽  
Auditory Dysfunction

Alzheimer's disease is neurodegenerative disorder which affects a growing number of older adults every year. With an understanding of auditory dysfunction in Alzheimer's disease, the speech-language pathologist working in the health care setting can provide better service to these individuals. The pathophysiology of the disease process in Alzheimer's disease increases the likelihood of specific types of auditory deficits as opposed to others. This article will discuss the auditory deficits in Alzheimer's disease, their implications, and the value of clinical protocols for individuals with this disease.

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