Axonal degenerationDemyelinationNeuronalDistalAxon degenerationSecondaryCenters for DiseasMyelinCMT3AtrophySchwannMultiple sclerosisNerveNeurodegenerative diseaseDorsalProximalProgressionDisorderNervous systemSpinal cordClinicalPathogenesisMetabolicOccursNeurological diseasesSensoryAmyotrophic Lateral SCongenitalAlzheimer's DiseaseDetectPathologySurvivalParaneoplasticGeneticNeurologicHuntington'sDementiaUnderstoodWeaknessWhite matterTissueDiagnosisDisability
Axonal degeneration8
- Axonal degeneration is followed by degradation of the myelin sheath and infiltration by macrophages. (wikipedia.org)
- Although most injury responses include a calcium influx signaling to promote resealing of severed parts, axonal injuries initially lead to acute axonal degeneration (AAD), which is rapid separation of the proximal (the part nearer the cell body) and distal ends within 30 minutes of injury. (wikipedia.org)
- The disintegration is dependent on Ubiquitin and Calpain proteases (caused by influx of calcium ion), suggesting that axonal degeneration is an active process and not a passive one as previously misunderstood. (wikipedia.org)
- Myelin clearance is the next step in Wallerian degeneration following axonal degeneration. (wikipedia.org)
- Peripheral nerves respond to injury or disease in one or more of the following ways: segmental remyelination, Wallerian degeneration, and axonal degeneration. (uspharmacist.com)
- 5,6 Segmental demyelination and Wallerian degeneration are repair mechanisms that are relevant to traumatic nerve injury, whereas axonal degeneration is more characteristically seen in metabolic and toxic nerve disorders such as diabetes mellitus and renal failure. (uspharmacist.com)
- Axonal degeneration is a prediction of disability. (medscape.com)
- Axonal injury is a major event in glaucoma and the cellular mechanisms involved in axonal degeneration are different from those that lead to retinal ganglion cell body death. (touchophthalmology.com)
Demyelination3
- Patients with primary Sjögren syndrome show loss of WM microstructural integrity, probably related to both Wallerian degeneration and demyelination. (ajnr.org)
- Alternatively, there are diseases where the primary pathology is demyelination. (abcmedicalnotes.com)
- However, a detailed analysis of the patterns of DeMyelination , Oligodendroglia Cell pathology and the reaction of other tissue components suggests that the PathoGenesis of Myelin destruction in this disease may be heterogeneous. (tripod.com)
Neuronal5
- SIGNIFICANCE STATEMENT Elucidation of the sources of ATP required for axon extension and maintenance has implications for understanding the mechanism of neuronal development and diseases of the nervous system. (jneurosci.org)
- Despite being a neurodegenerative disease, preservation of brain weight and a conspicuous absence of neuronal or glial cell death were signal features of this disease. (nature.com)
- 3,4 Axonotmesis leads to Wallerian degeneration , a process whereby the part of the axon that is separated from the neuronal cell body disintegrates distal to the injury. (uspharmacist.com)
- In the late 1960s, neurophysiologic testing allowed the classification of CMT into 2 groups, one with slow nerve conduction velocities and histologic features of a hypertrophic demyelinating neuropathy (hereditary motor and sensory neuropathy type 1 or CMT1) and another with relatively normal velocities and axonal and neuronal degeneration (hereditary motor and sensory neuropathy type 2 or CMT2). (medscape.com)
- Using a fruit fly model of Huntington's disease (HD), researchers found that curcumin - a substance in the spice turmeric- can reduce neuronal death and dysfunction and lead to better motor function in flies. (huntingtonsdiseasenews.com)
Distal8
- Wallerian degeneration is an active process of degeneration that results when a nerve fiber is cut or crushed and the part of the axon distal to the injury (which in most cases is farther from the neuron's cell body) degenerates. (wikipedia.org)
- Prior to degeneration, the distal section of the axon tends to remain electrically excitable. (wikipedia.org)
- In the absence of NGF, inhibition of glycolysis along distal axons results in axon degeneration independent of cell death. (jneurosci.org)
- Typically, a predilection exists for distal limbs as the site of disease onset and more severe symptoms and signs. (medscape.com)
- A condition caused by degeneration, atrophy, and destruction of the distal part of a nerve fiber''s axon and myelin, when continuity with the neural cell nucleus has been severed due to injury. (nih.gov)
- Background Traumatic injury to axons produces break down of axons and myelin in the site from the lesion and further distal to the where Wallerian degeneration develops. (bioshockinfinitereleasedate.com)
- After that axons and myelin also breakdown distal towards the lesion as Wallerian degeneration (WD) builds up [1]. (bioshockinfinitereleasedate.com)
- This degeneration most often affects the proximal musculature before it impacts the distal. (medscape.com)
Axon degeneration1
- Axon degeneration is a prominent feature of the injured nervous system, occurs across neurological diseases, and drives functional loss in neural circuits. (cornell.edu)
Secondary3
- Sjögren syndrome is a chronic systemic autoimmune disease that can be classified as primary Sjögren syndrome (pSS) when presenting in isolation or secondary when related to another connective tissue disease. (ajnr.org)
- Numerous other mechanisms can potentially affect RGC survival, including toxic and ischaemic injuries at the level of the retina and secondary degeneration, where RGCs not directly injured degenerate later as a consequence of neighbouring cell death. (touchophthalmology.com)
- As the nerve cells decrease in number, replacement gliosis, pyknosis, and secondary Wallerian degeneration in the roots and peripheral nerves are observed. (medscape.com)
Centers for Diseas3
- Centers for Disease Control and Prevention. (cdc.gov)
- Inclusion in the update does not necessarily represent the views of the Centers for Disease Control and Prevention nor does it imply endorsement of the article's methods or findings. (cdc.gov)
- The Centers for Disease Control and Prevention (CDC) cannot attest to the accuracy of a non-federal website. (cdc.gov)
Myelin4
- In addition, microglia phagocytoses brain-specific cargo, such as axonal and myelin debris in spinal cord injury or multiple sclerosis, amyloid-β deposits in Alzheimer's disease, and supernumerary synapses in postnatal development. (frontiersin.org)
- Susceptibility-based imaging aids accurate distinction of pediatric-onset MS from myelin oligodendrocyte glycoprotein antibody-associated disease. (ucsf.edu)
- This review highlights the spectrum of the Inflammatory DeMyelinating Diseases, the multitude of effector mechanisms that may produce Myelin destruction, and the pathologic Heterogeneity observed in MS lesions. (tripod.com)
- Further phagocytosis in serum-free moderate of Compact disc47+/+ myelin can be augmented after knocking down SIRPα amounts (SIRPα-KD) in phagocytes by lentiviral disease with SIRPα-shRNA whereas phagocytosis of myelin that does not have Compact disc47 (CD47-/-) is not. (bioshockinfinitereleasedate.com)
CMT32
- Dejerine-Sottas disease is also known as CMT3. (medscape.com)
- CMT3 (also known as Dejerine-Sottas disease) is a rare congenital hypomyelinating neuropathy, which can be an autosomal dominant or a recessive disorder with mutations in several genes, including PMP22 , MPZ , and EGR2 . (msdmanuals.com)
Atrophy2
- Howard Henry Tooth (1856-1926) described the same disease in his Cambridge dissertation in 1886, calling the condition peroneal progressive muscular atrophy. (medscape.com)
- Spinal muscular atrophy (SMA) is caused by successive motor unit degeneration. (medscape.com)
Schwann4
- The macrophages, accompanied by Schwann cells, serve to clear the debris from the degeneration. (wikipedia.org)
- Live imaging of amyotrophic lateral sclerosis pathogenesis: disease onset is characterized by marked induction of GFAP in Schwann cells. (ulaval.ca)
- To further investigate the involvement of glial cells (astrocytes and Schwann cells) in the pathogenesis of ALS, we generated ALS-(GFAP-luciferase/SOD(G93A)) reporter mouse in which upregulation of glial fibrillary acidic protein (GFAP) can be visualized from live animals throughout the different stages of disease. (ulaval.ca)
- Interestingly, however, the disease onset (90 days) was characterized by sharp and synchronized induction of GFAP in peripheral nerve Schwann cells suggesting that peripheral nerves pathology/denervation and associated Schwann cell stress may play an important role in the ALS pathogenesis. (ulaval.ca)
Multiple sclerosis2
- Inflammatory and neurodegenerative serum protein biomarkers increase sensitivity to detect disease activity in multiple sclerosis. (ucsf.edu)
- Multiple Sclerosis is an Inflammatory DeMyelinating Disease of the Central Nervous System. (tripod.com)
Nerve8
- Histograms evaluating large portions of tissue reveal significant increases in MD in individuals with Alzheimer's disease when compared with controls as well as a significant reduction in regional peak MD heights, which indicates nerve loss in gray matter and Wallerian degeneration in white matter. (diagnosticimaging.com)
- Now a large and ever increasing number of genetic subtypes has been described, and major advances in molecular and cellular biology have clarified the understanding of the role of different proteins in the physiology of peripheral nerve conduction in health and in disease. (medscape.com)
- Sciatic nerve transection, early after birth, results in significant degeneration of spinal motoneurons as well as sensory neurons present in the dorsal root ganglia. (hindawi.com)
- Max Planck Institute of Biochemistry researchers have shown that the intracellular location of protein aggregates, long suspected to be involved in the pathogenesis of neurodegenerative diseases like Huntington's, is extremely important to the survival of nerve cells. (huntingtonsdiseasenews.com)
- In addition to such clinical evidence, recent basic science studies have pointed to the lamina cribrosa region of the optic nerve as a major site of injury in the disease. (touchophthalmology.com)
- A measure of the ability of a nerve to perform this function would be of major clinical value in the identification of disease involving a nerve. (evokedpotential.com)
- A shift in the strength duration curve indicates a change in excitability and is seen in nerve diseases. (evokedpotential.com)
- a) Peripheral nerve in longitudinal section stained with Luxol fast blue-periodic acid-Schiff (PAS) showing scattered wallerian degeneration (arrowheads). (medscape.com)
Neurodegenerative disease2
- These mechanisms underlie its potent tissue protective effects in experimental models of stroke, cerebral hemorrhage, traumatic brain injury, neuroinflammatory and neurodegenerative disease. (biomedcentral.com)
- Mechanisms of Progression in Glaucoma Glaucoma is a neurodegenerative disease characterised by progressive loss of retinal ganglion cells (RGCs) over time with associated progressive visual field loss. (touchophthalmology.com)
Dorsal1
- Wallerian degeneration occurred in the dorsal funiculus where the white discoloration was observed on gross examination. (cdc.gov)
Proximal1
- in others (eg, certain cases of Charcot-Marie-Tooth disease type 1A (CMT1A) and inherited brachial plexus neuropathy [IBPN]/hereditary neuralgic amyotrophy [HNA]), proximal weakness predominates. (medscape.com)
Progression3
- However, even for CMT1 a heated debate has focused on the relative contribution of axonal versus demyelinative damage to the disease manifestations and progression. (medscape.com)
- A study with a small cohort of Huntington's disease patients differed from previous research in finding no metabolic alterations associated with disease progression in stage 2/3 patients compared to both premanifest and healthy subjects. (huntingtonsdiseasenews.com)
- At present, the pathological events precipitating disease onset and the exact pattern of disease progression are not fully understood. (ulaval.ca)
Disorder4
- Charcot-Marie-Tooth disease (CMT) is the most common inherited neurologic disorder. (medscape.com)
- Exclusion criteria were a history of major metabolic, neurologic, or psychiatric disorder and high risk for cardiovascular disease. (ajnr.org)
- Fabry Disease Fabry disease is a sphingolipidosis, an inherited disorder of metabolism, caused by deficiency of alpha-galactosidase A, which causes angiokeratomas, acroparesthesias, corneal opacities, recurrent. (msdmanuals.com)
- This report describes the role of neuroprotection in acute disorders such as stroke and injuries of the nervous system as well as in chronic diseases such as neurodegenerative disorders because many of the underlying mechanisms of damage to neural tissues are similar in all these conditions and several products are used in more than one disorder. (researchandmarkets.com)
Nervous system6
- Wallerian degeneration occurs after axonal injury in both the peripheral nervous system (PNS) and central nervous system (CNS). (wikipedia.org)
- Hereditary motor and sensory neuropathy associated with agenesis of the corpus callosum (HMSN/ACC) is an autosomal recessive disease of the central and peripheral nervous system that presents as early-onset polyneuropathy. (nature.com)
- 4 ⇓ - 6 Although involvement of the peripheral nervous system is a well-documented feature of the disease, the prevalence, the type, and the underlying mechanism of CNS involvement remain unclear. (ajnr.org)
- Pertinent findings are limited to the nervous system, except for cases of CADP associated with other diseases, as already mentioned. (medscape.com)
- However, a number of observations suggest that SARM1 biology is much more complicated, and there remains much to learn about how SARM1 governs nervous system responses to injury or disease. (cornell.edu)
- VistaGen Therapeutics, Inc., a clinical-stage biopharmaceutical company advancing treatments for depression, cancer, and diseases like Huntington's that involve the central nervous system, announced that it has secured exclusive worldwide commercial rights to three patent-pending stem-cell technologies from the University Health Network (UHN) of Canada. (huntingtonsdiseasenews.com)
Spinal cord3
- Combined MBK and EPN exposure caused Wallerian type degeneration and paranodal axonal swelling in the spinal cord. (cdc.gov)
- Spinal muscular atrophies (SMAs) represent a rare group of inherited disorders that cause progressive degeneration of the anterior horn cells of the spinal cord. (medscape.com)
- Our results suggest that the disease in mice is initiated simultaneously in the spinal cord and in the peripheral nerves and is characterized by several cycles of GFAP upregulation. (ulaval.ca)
Clinical7
- Charcot-Marie-Tooth disease (CMT) is actually a heterogeneous group of genetically distinct disorders with a similar clinical presentation. (medscape.com)
- Blood analyses and thoracic radiographs did not show a cause for the clinical disease, and blood eosinophil count was not elevated. (cdc.gov)
- In research and clinical settings, DTI has generated information on tissue infrastructure and indications of inflammation, tissue degeneration, and neurodevelopmental abnormalities, said Dr. Marco Bozzali from Don C. Gnocchi Foundation in Milan. (diagnosticimaging.com)
- Neuropathological examination of post-mortem brains of patients with dementia due to neurodegenerative and cerebrovascular changes remains important, as the family wants to be sure about the clinical diagnosis and the risk of a hereditary disease. (touchneurology.com)
- Although post-mortem neuropathological examination is increasingly performed less often in most western countries, it is still needed in patients with dementia, due to neurodegenerative and cerebrovascular changes, It is important for the family to be sure about the clinical diagnosis and to exclude the risk of a hereditary disease. (touchneurology.com)
- Raptor Pharmaceutical Corp. has announced the efficacy and safety results from CYST-HD, a Phase 2/3 clinical trial conducted in collaboration with the Centre Hospitalier Universitaire d'Angers, evaluating the potential of RP103 as a Huntington's disease therapy. (huntingtonsdiseasenews.com)
- The preclinical data in support of the use of EPO in brain disease have already been translated to first clinical pilot studies with encouraging results with the use of EPO as a neuroprotective agent. (biomedcentral.com)
Pathogenesis2
- Scientists have identified a proteomic pathway, involving the proteins Homer-3 and mTORC1, that is involved in the degeneration of Purkinje cells, a pathogenesis central to Huntington's disease. (huntingtonsdiseasenews.com)
- A careful evaluation of MS NeuroPathology should provide important clues regarding the induction, target, evolution, and PathoGenesis of this complex disease. (tripod.com)
Metabolic2
- The field is converging on a model whereby SARM1 acts as a sensor for metabolic changes that occur after injury, and then drives catastrophic NAD+ loss to promote degeneration. (cornell.edu)
- The study, titled "A Metabolic Study of Huntington's Disease," was published in the PLOS One journal. (huntingtonsdiseasenews.com)
Occurs1
- A related process of dying back or retrograde degeneration known as 'Wallerian-like degeneration' occurs in many neurodegenerative diseases, especially those where axonal transport is impaired such as ALS and Alzheimer's disease. (wikipedia.org)
Neurological diseases1
- Wallerian degeneration (WD) is a conserved axonal self-destruction program implicated in several neurological diseases. (bvsalud.org)
Sensory1
- Loss of these cells results in a progressive lower motor neuron disease that has no sensory involvement and that is manifested as hypotonia, weakness, and progressive paralysis. (medscape.com)
Amyotrophic Lateral S1
- Amyotrophic lateral sclerosis (ALS) is a late-onset neurological disease characterized by progressive loss of motor neurons. (ulaval.ca)
Congenital1
- Hereditary neuropathies include a variety of congenital degenerative peripheral neuropathies (eg, Charcot-Marie-Tooth disease). (msdmanuals.com)
Alzheimer's Disease2
- In this work, we evaluated the applicability of a hybrid framework, in which CSD based FT is combined with conventional DTI metrics to assess white matter abnormalities in 25 patients with early Alzheimer's disease. (plos.org)
- Despite problems in the selection of appropriate imaging sequences and methods of visualization, the technique holds wide potential applications in depicting brain diseases ranging from stroke to Alzheimer's disease and psychiatric disorders such as schizophrenia. (diagnosticimaging.com)
Detect1
- Biopsies detect axonal (wallerian) degeneration. (msdmanuals.com)
Pathology1
- The nature of disease pathology for the health professions, Philadelphia: Lippincott Williams & Wilkins. (nursekey.com)
Survival1
- Early studies of Wallerian degeneration highlighted a central role for NAD+ metabolites in axon survival, and this association has grown even stronger in recent years with a deeper understanding of SARM1 biology. (cornell.edu)
Paraneoplastic1
- Familial AN, drug-induced AN, AN occurring in hyperinsulinemic states (eg, diabetes, obesity), AN associated with polycystic ovary disease, and AN associated with a spectrum of autoimmune disease in women should be considered before AN is determined to represent a paraneoplastic syndrome. (medscape.com)
Genetic1
- With the advent of genetic testing, all of the different diseases that fall under the heading of CMT syndrome eventually are likely to become distinguishable. (medscape.com)
Neurologic1
- We report the first identification of P. cantonensis , as the cause of a debilitating neurologic disease in a captive primate in Florida. (cdc.gov)
Huntington's2
- What is Huntington's disease? (huntingtonsdiseasenews.com)
- Ionis Pharmaceuticals, Inc. - formerly Isis Pharmaceuticals - announced the U.S. Food and Drug Administration (FDA) has approved its Orphan Drug Designation application for IONIS-HTTRx as a potential treatment of Huntington's disease (HD). (huntingtonsdiseasenews.com)
Dementia1
- Although it is a rare disease, iNPH needs to be differentiated from other common diseases causing dementia, gait disturbance, or both. (ajnr.org)
Understood1
- The signaling pathways leading to axolemma degeneration are currently poorly understood. (wikipedia.org)
Weakness1
- Although initially it resembles Charcot-Marie-Tooth disease, the motor weakness progresses more quickly. (msdmanuals.com)
White matter1
- Recently, DTI has been used frequently to evaluate white matter alteration in various neurodegenerative diseases. (ajnr.org)
Tissue1
- The present review will summarize the current state of the literature regarding the role of microglial phagocytosis in maintaining tissue homeostasis in health as in disease. (frontiersin.org)
Diagnosis2
- Though a definitive post-mortem diagnosis still needs to be confirmed by an extensive macroscopic and microscopic examination of the brain using validated neuropathological criteria, 4 7.0-tesla MRI can be used as an additional tool to examine post-mortem brains of patients with neurodegenerative diseases. (touchneurology.com)
- RESULTS Inflammatory disease (36/66 [55%]) was the most common histologic diagnosis. (avma.org)
Disability1
- And extra- importance than of this disease prior to be training and unstable atten- of disability. (dieserso.com)