Neurodegeneration: Causes & Reasons

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Possible Causes for Neurodegeneration

Vitamin B12 Deficiency

[…] on cognitive function, this study lends support to the notion that high serum folate concentrations in older people with VB12 deficiency may be associated with increased neurodegeneration [ncbi.nlm.nih.gov]

Oxidative stress is implicated in various human diseases and conditions, such as a neurodegeneration, which is the major symptom of vitamin B 12 deficiency, although the underlying [ncbi.nlm.nih.gov]

Neurodegeneration in methylmalonic aciduria involves inhibition of complex II and the tricarboxylic acid cycle, and synergistically acting excitotoxicity. [doi.org]

Combined Oxidative Phosphorylation Deficiency Type 24

Angeli JP, Conrad M, Strom TM, Meitinger T, Prokisch H, Distelmaier F Title Human thioredoxin 2 deficiency impairs mitochondrial redox homeostasis and causes early-onset neurodegeneration [genome.jp]

Partial lipodystrophy, congenital cataracts, and neurodegeneration syndrome 606721 601047 Autosomal dominant CAV3 3p25.3 Rippling muscle disease 606072 601253 Autosomal dominant [mnglabs.com]

[…] dystrophy-dystroglycanopathy (congenital without mental retardation), type B, 4 613152 Muscular dystrophy-dystroglycanopathy (limb-girdle), type C, 4 611588 FOLR1 11q13.4 Neurodegeneration [institutobernabeu.com]

Pallidopyramidal Syndrome

Pantothenate kinase-associated neurodegeneration (PKAN) and PLA2G6-associates neurodegeneration (PLAN): Review of two major neurodegeneration with brain iron accumulation [annalsofian.org]

AB - Kufor-Rakeb syndrome is an autosomal recessive nigro-striatal-pallidal-pyramidal neurodegeneration. [research.manchester.ac.uk]

Understanding how FBXO7 mutations cause disease will shed further light on the molecular mechanisms of neurodegeneration, with potential implications also for more common [moh-it.pure.elsevier.com]

X-Linked Parkinsonism with Spasticity

Test Registry Neurodegeneration With Brain Iron Accumulation 3 Basal Ganglia Disease, Adult-Onset Neuroferritinopathy NBIA3 606159 Genetic Test Registry Neurodegeneration [ukgtn.nhs.uk]

MeSH Descriptor Source: MeSH 2019 deskriptory Main Heading Hallervordenův-Spatzův syndrom English Heading Pantothenate Kinase-Associated Neurodegeneration See (Czech) neurodegenerace [medvik.cz]

Amyotrophic lateral sclerosis, susceptibility to DNAJC6 Parkinson disease 19a, juvenile-onset EIF4G1 Parkinson disease 18 FBXO7 Parkinson disease 15, autosomal recessive FTL Neurodegeneration [asperbio.com]

Primary Progressive Multiple Sclerosis

Spinal neurodegeneration is an important determinant of disability progression in patients with primary progressive multiple sclerosis. [ncbi.nlm.nih.gov]

The effect of current immunomodulatory treatments on neurodegeneration is insufficient. [ncbi.nlm.nih.gov]

Studies on the histology and pathogenesis show that neurodegeneration is predominant over inflammation in PPMS, even if cellular and humoral immune-mediated mechanisms are [ncbi.nlm.nih.gov]

Lafora Disease

Lafora disease is a metabolic disorder resulting in accumulation of water-insoluble glucan in the cytoplasm, and manifests as a debilitating neurodegeneration that ends with [ncbi.nlm.nih.gov]

These animals did not show the increase in markers of neurodegeneration, the impairments in electrophysiological properties of hippocampal synapses, nor the susceptibility [ncbi.nlm.nih.gov]

Our results emphasize the relevance of the laforin-malin complex in the control of glycogen metabolism and highlight altered glycogen accumulation as a key contributor to neurodegeneration [ncbi.nlm.nih.gov]

Progressive Myoclonic Epilepsy Type 3

Neurodegeneration and regression occur with seizure onset. Other features include mental retardation, dysarthria, truncal ataxia, and loss of fine finger movements. [malacards.org]

Symptoms The list of signs and symptoms mentioned in various sources for Epilepsy, progressive myoclonic 3 includes the 6 symptoms listed below: * Myoclonic epilepsy * Neurodegeneration [checkorphan.org]

[…] myoclonic type 3 The list of signs and symptoms mentioned in various sources for Epilepsy, progressive myoclonic 3 includes the 6 symptoms listed below: * Myoclonic epilepsy * Neurodegeneration [checkorphan.org]

Progressive Myoclonic Epilepsy Type 8

The patient presented here constitutes a rare case of pediatric epilepsy, which caused neurodegeneration in late-childhood and onset with typical epilepsy symptoms. [eastjmed.org]

Knockdown of Cers1 in mouse neuroblastoma cells triggered endoplasmic reticulum stress, the unfolded protein response, and apoptosis, suggesting a mechanism for neurodegeneration [onlinelibrary.wiley.com]

To our knowledge, this is the shortest period in literature.Keywords: Lafora Disease, progressive myoclonic epilepsy, neurodegeneration Ömer Bektaş, Arzu Yılmaz, Aylin Heper [eastjmed.org]

Progressive Myoclonic Epilepsy Type 7

Ataxia-telangiectasia-like disorder Atherosclerosis-deafness-diabetes-epilepsy-nephropathy syndrome Atypical juvenile parkinsonism Atypical pantothenate kinase-associated neurodegeneration [se-atlas.de]

Despite increasing knowledge of the etiology of most progressive myoclonus epilepsy disorders, the pathogenic mechanisms leading to neurodegeneration and epilepsy remain largely [medlink.com]

Neurodegeneration and regression occur with seizure onset. Other features include mental retardation, dysarthria, truncal ataxia, and loss of fine finger movements. [genecards.org]

Alzheimer Disease

Dysregulation of miRNAs is one of the major causes of neurodegeneration. [ncbi.nlm.nih.gov]

The disease is clinically characterised by dementia, loss of cognitive functions and massive neurodegeneration. [ncbi.nlm.nih.gov]

Parkinson Disease

In this review, we address the current concept of neuroinflammation and its involvement in PD-associated neurodegeneration. [ncbi.nlm.nih.gov]

Overexpression of the vesicular monoamine transporter (VMAT) rescued the dopamine toxicity and neurodegeneration, whereas mutations decreasing VMAT and tyrosine hydroxylase [doi.org]

However, 5-10% of PD cases are inherited as monogenic diseases, which provides a chance to understand the molecular mechanisms underlying neurodegeneration. [ncbi.nlm.nih.gov]

Wolfram Syndrome

In patients with Wolfram syndrome, these mechanisms underlie progressive neurodegeneration and endocrine dysfunction. [care.diabetesjournals.org]

This work is necessary to position the field for future clinical trials to test interventions for WFS neurodegeneration. [clinicaltrials.gov]

[…] thought to result in chronic ER stress–mediated apoptosis of pancreatic β-cells, neuroendocrine, and neuronal cells, leading to a progressive decline of endocrine function and neurodegeneration [care.diabetesjournals.org]

Spinal and Bulbar Muscular Atrophy

Pre-clinical studies using animal models show that the pathogenic AR-mediated neurodegeneration is suppressed by androgen inactivation, the efficacy of which has been tested [ncbi.nlm.nih.gov]

Animal studies have revealed that the pathogenesis of SBMA depends on the level of serum testosterone, and that androgen deprivation mitigates neurodegeneration through inhibition [ncbi.nlm.nih.gov]

Abstract Expanded polyglutamine tracts cause neurodegeneration through a toxic gain-of-function mechanism. [ncbi.nlm.nih.gov]

Amyotrophic Lateral Sclerosis

Here we review some of the recent progress in promoting therapeutic strategies for neurodegeneration. [doi.org]

This review aims to investigate how oxidative stress can affect other proposed mechanisms of neurodegeneration in ALS. [ncbi.nlm.nih.gov]

Strategies for clinical approach to neurodegeneration in amyotrophic lateral sclerosis Cecilia Carlesi, Livia Pasquali, Selina Piazza, Annalisa Lo Gerfo, Elena Caldarazzo [doi.org]

Motor Neuron Disease

Renaud -- Neurodegeneration with brain iron accumulation, Wilson disease, manganism / Alisdair McNeill. [worldcat.org]

[…] cellular signature of aggregated TDP-43 common to nearly all MND and a large proportion of frontotemporal dementia (FTD), has placed MND alongside more traditional cerebral neurodegeneration [ncbi.nlm.nih.gov]

Nelson -- Protein aggregation and neurodegeneration : tauopathies and synucleinopathies / Michel Goedert -- Prion degeneration / James A. [worldcat.org]

Hallervorden-Spatz Syndrome

(disorder) Pigmentary pallidal degeneration Neurodegeneration With Brain Iron Accumulation type 1 Pantothenate Kinase-Associated Neurodegeneration NEURODEGENERATION WITH [wikidata.org]

Pantothenate kinase-associated neurodegeneration (PKAN) is a type of neurodegeneration with brain iron accumulation (NBIA). [ncbi.nlm.nih.gov]

BRAIN IRON ACCUMULATION 1; NBIA1 PKAN Pkan Neuroaxonal Dystrophy, Juvenile-Onset edit English pantothenate kinase-associated neurodegeneration A neurodegeneration with brain [wikidata.org]

Progressive Supranuclear Palsy

CurePSP CurePSP is the leading source of information and support for patients and their families, other caregivers, researchers, and healthcare professionals on prime of life neurodegeneration [psp.org]

Neurodegeneration: the molecular pathology of dementia and movement disorders. Basel : ISN Neuropath Press ; 2003. p. 103 – 14. 5. [doi.org]

Progressive Supranuclear Palsy: A Review of Co-existing Neurodegeneration × Export citation Request permission Copyright COPYRIGHT: © The Canadian Journal of Neurological [doi.org]

Distal Spinal Muscular Atrophy Type 5

Previous studies have shown that HSP40 proteins play a crucial role in protein aggregation and neurodegeneration in several neuronal types, in animal models and human diseases [ncbi.nlm.nih.gov]

[…] due to cerebral folate transport deficiency FOLR1 Neurodegeneration with brain iron accululation type 5 WDR45 Neurodegeneration with brain iron accumulation type 4 C19orf12 [centogene.com]

Gaucher disease Gaucher disease - ophthalmoplegia - cardiovascular calcification Gaucher disease type 1 Gaucher disease type 2 Gaucher disease type 3 Early-onset progressive neurodegeneration [csbg.cnb.csic.es]

Neuronal Ceroid Lipofuscinosis

Mutant dnj-14 worms also exhibited age-dependent neurodegeneration of sensory neurons, which was preceded by severe progressive chemosensory defects. [ncbi.nlm.nih.gov]

Autosomal-dominant adult-onset neuronal ceroid lipofuscinosis (ANCL) is characterized by accumulation of autofluorescent storage material in neural tissues and neurodegeneration [ncbi.nlm.nih.gov]

KEYWORDS: Cysteine-string protein alpha (CSPα); Neurodegeneration; Neuronal ceroid lipofuscinosis (NCL); Palmitoyl-protein thioesterase 1 (PPT1); Palmitoylation [ncbi.nlm.nih.gov]

REM Sleep Behavior Disorder

So far, the number of studies assessing predictive markers of neurodegeneration in RBD is limited. [n.neurology.org]

[…] diagnosis. 1 The strongest clinical prodromal marker, by far, is REM sleep behavior disorder (RBD). 1 Most patients with idiopathic RBD are in fact in the prodromal stages of neurodegeneration [n.neurology.org]

McLeod Neuroacanthocytosis Syndrome

[…] label Core neuroacanthocytosis syndromes, including: Chorea-acanthocytosis (ChAc); McLeod syndrome (MLS); Huntington's disease-like 2 (HDL2); Pantothenate kinase associated neurodegeneration [bio2rdf.org]

The mechanism by which these mutations cause neurodegeneration is not known. [genome.jp]

The mechanism by which these mutations cause neurodegeneration is not known. dcterms:identifier kegg:H00832 void:inDataset http://bio2rdf.org/kegg_resource:bio2rdf.dataset.kegg.R3 [bio2rdf.org]

Autosomal Recessive Deafness 42

Deafness autosomal recessive type 42 (ILDR1) DNA Test Leave Message Reporting Time: 5 Week Test Cost: 19000.00 /- Rs Sample Type: EDTA blood or DNA Deafness autosomal recessive type 42 (ILDR1) Test Description Deafness autosomal recessive type 42 (ILDR1) Deafness autosomal recessive type 42 (ILDR1) genetic[…] [dnalabsindia.com]

Huntington Disease

In this review we consider the current status of research in the field and possible mechanisms whereby the HD mutation might result in neurodegeneration. [ncbi.nlm.nih.gov]

Identification of genes that modify ataxin-1-induced neurodegeneration. Nature 408, 101–106 (2000). 16 Marsh, J. L. et al. [doi.org]

Regulation of autophagy by polyphenols: Paving the road for treatment of neurodegeneration. Biotechnology Advances, Vol. 36, Issue. 6, p. 1768. [dx.doi.org]

Huntington Disease-Like Type 3

Basal ganglia Caudate Huntington's disease Inclusion Junctophilin Neurodegeneration Striatum Trinuleotide repeat expansion Clinical Neurology Psychiatry and Mental health [jhu.pure.elsevier.com]

PKAN is also referred to as Hallervorden-Spatz syndrome or neurodegeneration with brain iron type 1 (NBIA1). Onset occurs in childhood. [hdlf.org]

The pathology of HD reveals neurodegeneration in the corpus striatum and shrinkage of the brain. [themedicalbiochemistrypage.org]

Classic Progressive Supranuclear Palsy Syndrome

The factors that initiate tau-neurodegeneration are unknown. Diagnostic methods Diagnosis is based on the clinical picture and neuropsychological evaluation. [orpha.net]

Cerebral Cortical Atrophy

Etiology Etiology of MSA is unknown but presence of cytoplasmic aggregates of α-synuclein, primarily in oligodendroglia, in combination with neurodegeneration in striatonigral [orpha.net]

In Alzheimer’s Disease, neurons will stop working or die in a process called neurodegeneration. [en.wikipedia.org]

Shifting imaging targets in multiple sclerosis: from inflammation to neurodegeneration. [smw.ch]

Huntington Disease-Like Type 1

PKAN is also referred to as Hallervorden-Spatz syndrome or neurodegeneration with brain iron type 1 (NBIA1). Onset occurs in childhood. [hdlf.org]

We therefore hypothesize that the neurodegeneration in HDL2 stems from the expression of JPH3 transcripts with a long CUG repeat, and that the pathogenic pathway leading from [grantome.com]

Ching KH et al. (2002) HARP syndrome is allelic with pantothenate kinase-associated neurodegeneration. Neurology 58 : 1673–1674 76. [nature.com]

Muscular Dystrophy-Dystroglycanopathy Type C7

Metabolic Myopathies, Rhabdomyolysis and Exercise Intolerance Methylmalonic Acidemia Muscular Dystrophy Myasthenic Syndrome Myopathy Myotonia Congenita Neuroaxonal Dystrophy Neurodegeneration [preventiongenetics.com]

Partial lipodystrophy, congenital cataracts, and neurodegeneration syndrome 606721 601047 Autosomal dominant CAV3 3p25.3 Myopathy, distal, Tateyama type 614321 601253 Autosomal [mnglabs.com]

Partial lipodystrophy, congenital cataracts, and neurodegeneration syndrome 606721 601047 Autosomal dominant CAV3 3p25.3 Creatine phosphokinase, elevated serum 123320 601253 [mnglabs.com]

Neurodegenerative Disorder

All of these conditions lead to progressive brain damage and neurodegeneration. [news-medical.net]

What causes neurodegeneration? [ncbi.nlm.nih.gov]

Treatment There are currently no therapies available to cure neurodegeneration. [news-medical.net]

Machado-Joseph Disease

namely at early stages of another polyglutamine-related disorder such as Huntington's disease, we now tested their ability to control MJD-associated neurodegeneration. [ncbi.nlm.nih.gov]

Selective neurodegeneration in MJD is evident in several subcortical brain regions including the cerebellum. [ncbi.nlm.nih.gov]

Since these findings were detected both by lifetime images and by postmortem examination, MRI intensity changes could track the progression of neurodegeneration. [ncbi.nlm.nih.gov]