• The term olivopontocerebellar atrophy is used when the disorder starts later in life and the process is a primary degeneration of cerebellar neurons. (nih.gov)
  • Dejerine and Thomas first used the term olivopontocerebellar atrophy (OPCA) in 1900 when they described 2 patients with a degenerative disorder leading to progressive cerebellar dysfunction and parkinsonism. (medscape.com)
  • We identified three cases of CBD with olivopontocerebellar atrophy (CBD-OPCA) that did not have a-synuclein-positive glial cytoplasmic inclusions of multiple system atrophy (MSA). (elsevierpure.com)
  • It includes 3 disorders previously thought to be distinct: olivopontocerebellar atrophy, striatonigral degeneration, and Shy-Drager syndrome. (msdmanuals.com)
  • Cerebellar abnormalities predominate in olivopontocerebellar atrophy. (msdmanuals.com)
  • Kjellin syndrome is characterized by retinal degeneration, autosomal recessive hereditary spastic paraplegia, and thin corpus callosum initially associated with spastic paraplegia 15 (SPG15) but more often occurring in individuals with SPG11. (nih.gov)
  • A single intra-vitreal treatment by this crySI fusion significantly inhibits retinal degeneration in comparison to free mini cry. (nih.gov)
  • These results show that intra-ocular ELP-linked peptides such as crySI hold promise as protective agents to prevent RPE atrophy and progressive retinal degeneration in AMD. (nih.gov)
  • Biallelic variants in the cilia gene DYNC2H1 have been associated with two severe ciliopathies: Jeune asphyxiating thoracic dystrophy (JATD, MIM 613091) and short-rib polydactyly (SRP, MIM 613091) with only four documented cases of associated complex early retinal degeneration at ages 2 months, and 2, 5, and 11 years old. (nature.com)
  • This test is useful in diagnosing early stages of PRA, and in differentiating between optic neuritis and sudden acquired retinal degeneration (SARD). (vin.com)
  • Common causes of blindness in this category include Sudden Acquired Retinal Degeneration (SARD), optic neuritis involving the distal optic nerve, and neoplasia compressing the optic nerve or chiasm. (vin.com)
  • History of or presence of retinal disease other than GA: diabetic retinopathy, central serous chorioretinopathy, inherited retinal degeneration, toxic maculopathies (ie, hydroxychloroquine maculopathy), arterial and venous occlusive disease, macular hole that is present or has been previously repaired, or choroidal melanoma. (who.int)
  • However, a standardized clinical evaluation of asymmetric neuronal degeneration and its impact on clinical findings has only sporadically been investigated for F-18-fluorodeoxyglucose positron emission tomography (F-18-FDG-PET). (uni-muenchen.de)
  • This study aimed to evaluate the impact of lateralized neuronal degeneration on the detection of AD by detailed clinical testing. (uni-muenchen.de)
  • Furthermore, we compared associations between clinical evaluation and lateralized neuronal degeneration between FDG-PET hypometabolism and hippocampal atrophy. (uni-muenchen.de)
  • Finally, we investigated if specific subtests show associations with lateralized neuronal degeneration. (uni-muenchen.de)
  • For assessment of neuronal degeneration, FDG-PET hypometabolism in brain regions typically affected in AD were graded by visual (3D-surface projections) and semiquantitative analysis. (uni-muenchen.de)
  • Measures of asymmetry were calculated to quantify lateralized neuronal degeneration and asymmetry scores were subsequently correlated with CERAD. (uni-muenchen.de)
  • Direct comparison of CERAD-PET associations in cases with right- and left-lateralized neuronal degeneration estimated a detection gap of 2.7 years for right-lateralized cases. (uni-muenchen.de)
  • 0.001) and constructional praxis (semiquantitative: β = 0.292, p = 0.008) showed significant associations with right-hemispheric neuronal degeneration. (uni-muenchen.de)
  • Conclusions: Asymmetry of deteriorated cerebral glucose metabolism has a significant impact on the coupling between neuronal degeneration and cognitive function. (uni-muenchen.de)
  • Right dominant neuronal degeneration shows a delayed detection by global CERAD testing and requires evaluation of specific subdomains of cognitive testing. (uni-muenchen.de)
  • In 1960, van de Eecken, Adams, and van Bogaert reported 3 patients with striatonigral degeneration (SND) with atrophy of the caudate nucleus and putamen. (medscape.com)
  • predominate in striatonigral degeneration. (msdmanuals.com)
  • Infantile onset progressive cerebellar atrophy and anterior horn cell degeneration--a late onset variant of PCH-1? (nih.gov)
  • However, anterior horn cell degeneration has also been described in cases with later onset pontocerebellar atrophy and recently the spectrum has even been further extended to include the association of anterior horn cell degeneration and cerebellar atrophy without pontine involvement. (nih.gov)
  • The clinical definition of multiple system atrophy (MSA) is a progressive, idiopathic, degenerative process beginning in adulthood, manifesting in various degrees of autonomic failure, parkinsonism, cerebellar dysfunction, and pyramidal signs that are poorly responsive to levodopa or dopamine agonists. (medscape.com)
  • Neuronal loss and grumose degeneration in the cerebellar dentate nucleus were comparable in CBD-OPCA and PSP. (elsevierpure.com)
  • 1- 4 Multiple system atrophy is usually defined by the predominance of parkinsonian (MSA-P type) or cerebellar (MSA-C type) features. (bmj.com)
  • Multiple system atrophy is a relentlessly progressive neurodegenerative disorder causing pyramidal, cerebellar, and autonomic dysfunction. (msdmanuals.com)
  • PRA-prcd occurs as a result of degeneration of both rod and cone type Photoreceptor Cells of the Retina , which are important for vision in dim and bright light, respectively. (pawprintgenetics.com)
  • Other signs of progressive retinal atrophy involve changes in reflectivity and appearance of a structure behind the retina called the Tapetum that can be observed on a veterinary eye exam. (pawprintgenetics.com)
  • Age-related macular degeneration (AMD) is a disease that impacts the central area of the retina in the eye, called the macula. (roche.com)
  • Macular degeneration (MD) usually affects older adults and results in a loss of vision in the center of the visual field (the macula) because of damage to the retina. (patientslikeme.com)
  • One of the first behavioral signs of inherited, degenerative diseases of the outer retina (commonly known as Progressive Retinal Atrophy, PRA) is loss of night vision as rods are affected before cones. (vin.com)
  • Benign conditions of the vitreous (ie, posterior vitreous detachment) or peripheral retina (ie, paving stone degeneration, lattice degeneration, etc.) are permitted. (who.int)
  • We describe two siblings from a consanguineous Moslem Arabic family who presented with progressive degeneration of both the cerebellum and the anterior horn cells. (nih.gov)
  • Parkinson disease (PD) , also known as idiopathic parkinsonism , is a neurodegenerative disease and movement disorder characterized by resting tremor, rigidity and hypokinesia due to progressive degeneration of dopaminergic neurons in the substantia nigra . (radiopaedia.org)
  • Adult-onset motor neuron diseases are a group of neurologic disorders that present in adult life and are characterized primarily by progressive degeneration and loss of motor neurons. (medscape.com)
  • ALS involves upper and lower motor neurons and presents as an idiopathic, progressive degeneration of anterior horn cells and their associated neurons, resulting in progressive muscle weakness, atrophy, and fasciculations. (medscape.com)
  • These disorders have complex clinical presentations that reflect degeneration in various neuronal systems. (medscape.com)
  • Clinical features and natural history of multiple system atrophy (MSA) have been established in four recent series. (bmj.com)
  • Atrophy patterns in early clinical stages across distinct phenotypes of Alzheimer's disease. (sens.org)
  • Patients with each syndrome were stratified based on: (1) degree of functional impairment, as measured by the clinical dementia rating (CDR) scale, and (2) overall extent of brain atrophy, as measured by a neuroimaging approach that sums the number of brain voxels showing significantly lower gray matter volume than cognitively normal controls (n=80). (sens.org)
  • Even at the earliest clinical stage (CDR=0.5 or bottom quartile of overall atrophy), patients with each syndrome showed both common and variant-specific atrophy. (sens.org)
  • Corticobasal degeneration (CBD) is a disorder affecting cognition and movement due to a progressive neurodegeneration associated with distinctive neuropathologic features, including abnormal phosphorylated tau protein in neurons and glia in cortex, basal ganglia, diencephalon, and brainstem, as well as ballooned neurons and astrocytic plaques. (elsevierpure.com)
  • However, it can be due to other neurological conditions such as Lewy body dementia or corticobasal degeneration. (mayoclinic.org)
  • Other less common causes include corticobasal degeneration, Lewy body dementia and Creutzfeldt-Jakob disease. (mayoclinic.org)
  • The dopaminergic tract is predominantly affected in Parkinson disease, and histologically, it is characterized by nigrostriatal dopaminergic degeneration leading to neuronal loss in the substantia nigra pars compacta (SNpc), most conspicuous in the ventrolateral tier of neurons 11 . (radiopaedia.org)
  • Purpose: To compare the incidence and progression of macular atrophy (MA) in eyes with neovascular age-related macular degeneration (nAMD) treated with anti-vascular endothelial growth factor (VEGF) agents using either a treat-and-extend (T&E) or a pro re nata (PRN) regimen over 4 years in a real-world setting. (unimi.it)
  • Main Outcomes Measures: Macular atrophy incidence and progression over 4 years, association between treatment strategies, and number of injections. (unimi.it)
  • Macular Atrophy Incidence and Progression in Eyes with Neovascular Age-Related Macular Degeneration Treated with Vascular Endothelial Growth Factor Inhibitors Using a Treat-and-Extend or a Pro Re Nata Regimen: Four-Year Results of the MANEX Study / K.L. Spooner, S. Fraser-Bell, M. Cozzi, G. Staurenghi, A. Invernizzi, D. Monteduro, M.R. Munk, T. Hong, A.A. Chang. (unimi.it)
  • Prevalence of age-related macular degeneration associated genetic risk factors and 4-year progression data in the Irish population. (roche.com)
  • Early syndrome-specific atrophy mirrored functional brain networks underlying functions that are uniquely affected in each variant: Language network in lvPPA, posterior cingulate cortex-hippocampal circuit in amnestic EOAD and LOAD, and visual networks in PCA. (sens.org)
  • Posterior cortical atrophy is a brain and nervous system syndrome that causes brain cells to die over time. (mayoclinic.org)
  • Soon after, Professor Johann Hoffmann from Heidelberg University presented a paper describing a syndrome of progressive atrophy, weakness, and death during the early childhood period of siblings with genetically normal parents. (medscape.com)
  • Glial cytoplasmic inclusions (GCIs) and neuronal multisystem degeneration are the pathologic hallmarks of this clinically variable disorder (see the image below). (medscape.com)
  • Progressive retinal Atrophy , progressive Rod -cone degeneration (PRA-prcd) is a late onset, inherited eye disease affecting many breeds of dog. (pawprintgenetics.com)
  • Cardiovascular disease: Diseases that affect the blood vessels and the heart puts you at a high risk of getting macular degeneration. (vrcny.com)
  • Taking the Vitamins can slow down macular degeneration disease. (vrcny.com)
  • In more than 80% of cases, posterior cortical atrophy is due to Alzheimer's disease. (mayoclinic.org)
  • The most common cause of posterior cortical atrophy is a form of Alzheimer's disease that's not typical. (mayoclinic.org)
  • Further study is needed to determine whether the risk factors for Alzheimer's disease may play a role in posterior cortical atrophy. (mayoclinic.org)
  • Global prevalence of age-related macular degeneration and disease burden projection for 2020 and 2040: a systematic review and meta-analysis. (roche.com)
  • Developmental disuse (i.e. hind limb immobilization) associated with PA induced muscle fiber atrophy, extracellular matrix changes in the muscle, and mild to moderate ankle and knee joint degeneration at levels greater than disuse alone. (cdc.gov)
  • There is a longstanding belief that a given nucleus controls a defined volume of cytoplasm, so when a muscle grows (hypertrophy) or shrinks (atrophy), the number of myonuclei change accordingly. (frontiersin.org)
  • Recently, two independent models, one from rodents and the other from insects, have demonstrated that nuclei are not lost from skeletal muscle fibers when they undergo either atrophy or programmed cell death. (frontiersin.org)
  • Common atrophy across variants was found in temporoparietal regions that comprise the posterior default mode network (DMN). (sens.org)
  • Posterior cortical atrophy causes the loss of brain cells in back of the brain. (mayoclinic.org)
  • A meta-analysis revealed a significant association between age-related macular degeneration and systemic dyslipidemia, which varies across different AMD stages and subtypes. (medpagetoday.com)
  • This report describes the protective potential of a peptide derived from the αB crystallin protein using a sodium iodate (NaIO 3 ) induced mouse model of GA. Systemic NaIO 3 challenge causes degeneration of the RPE and neighboring photoreceptors, which have similarities to retinas of GA patients. (nih.gov)
  • They identified 32 metabolites associated with 3 OCT findings: hyperreflective foci, atrophy, and ellipsoid zone disruption. (medpagetoday.com)
  • The findings about the diagnostic accuracy of the Amsler grid test for early detection of neovascular age-related macular degeneration suggest that the procedure will not replace regular ophthalmic exams. (medpagetoday.com)
  • Inherited retinal diseases (IRDs) are a clinically and genetically heterogeneous group of disorders that often lead to photoreceptor degeneration. (nature.com)
  • 2. Presence of macular fibrosis or retinal epithelial tear, clinically relevant myopic degeneration, or vitreous hemorrhage a. (who.int)
  • Dostal J, Hrdlicova A, Horak P. Progressive rod-cone degeneration (PRCD) in selected dog breeds and variability in its phenotypic expression. (pawprintgenetics.com)
  • Zinc help patients with aged macular degeneration maintain their vision. (vrcny.com)
  • Data from patients with macular degeneration, who have ever reported treatments, ordered by their reporting frequency during the last 5 years. (patientslikeme.com)
  • RÉSUMÉ Nous avons évalué les causes des déficiences visuelles et de la cécité chez 694 patients diabétiques ayant consulté dans notre centre des soins oculaires à Sanaa (Yémen) entre 2001 et 2005 en examinant leur dossier médical. (who.int)
  • Macular degeneration is considered age-related when it occurs in people over 55 years of age. (healthline.com)
  • Myofibroblasts in macular fibrosis secondary to neovascular age-related macular degeneration-the potential sources and molecular cues for their recruitment and activation. (roche.com)
  • At more advanced stages, atrophy patterns largely converged across AD variants. (sens.org)
  • His case study "On the relationship between aphasia and senile atrophy of the brain" still serves as a frame of reference for apparently focal brain syndromes in diffuse or generalized degenerative diseases of the brain. (medscape.com)
  • Defining Phenotypes of Movement Disorders :Parkinson's Plus Disorders (PD), Essential Tremor (ET), Cortical Basal Degeneration (CBD), Multiple Systems Atrophy (MSA), Magnetoencephalography. (checkorphan.org)
  • In the 1980s and 1990s, two parallel streams of information accumulated related to focal brain degenerations. (medscape.com)
  • Early dry form typically has drusen (usually around 63u) and RPE degeneration and only needs periodic follow-ups, intermediate dry form has large drusen (>125u) and RPE degeneration and proliferation which requires examinations every 3 to 6 months depending on the severity of the presentation. (optos.com)