Aspects of motor neuron disease. Medical search. Frequent questions

Degeneration of dopamine neurons in the substantia nigra (SN) and the consequent deficit of dopamine release in the striatum and other target areas appear to be responsible for the characteristic manifestations of PD. (biomedcentral.com)
The pathophysiology of PD is characterized by the degeneration of dopamine neurons and a reduction in striatal dopamine release. (biomedcentral.com)
The classic motor deficits of Parkinson's disease (PD) are caused by degeneration of dopamine neurons, resulting in the loss of part of the neurons called axonal projections that modulate a cluster of nerve cells called the striatum. (michaeljfox.org)
As a step toward clinical translation, we will utilize TE-NSPs biofabricated using human induced pluripotent stem cell (iPSC)-derived dopamine neurons (also developed through our initial MJFF funding). (michaeljfox.org)
BDRF has been suggested to increase turnover rate of dopamine in vitro and provide a neuroprotective role in nigral dopamine neurons ( 7 ). (frontiersin.org)
After re- interesting work was happening at medi- entirety, exploring their growth and con- vealing that dopamine neurons could be cal schools. (lu.se)
This dramatic increase coincides with the introduction of a range of new neurochemical and pharmacological tools for the study of dopamine neurons and their function in the brain, as well as the identification of the dopamine receptors, their pharmacology, and their role in mediating the antipsychotic action of neuroleptics [12,13]. (lu.se)

Researchers have identified a gene, called matrix metalloproteinase-9 (MMP-9), that appears to play a major role in motor neuron degeneration in amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease. (sciencedaily.com)
This correlation holds true in MNs expressing mutations causing the most common motor neuron diseases (amyotrophic lateral sclerosis or ALS and spinal muscular atrophy or SMA) and even in MNs obtained from unaffected patients. (dzne.de)
For the disease amyotrophic lateral sclerosis, also known as motor neurone disease, see Amyotrophic lateral sclerosis . (mdwiki.org)
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder in which there is preferential loss of both the upper and lower motor neurons. (bmj.com)
Identification of a disease-causing variant may assist with diagnosis, prognosis, clinical management, recurrence risk assessment, familial screening, and genetic counseling for frontotemporal dementia and/or amyotrophic lateral sclerosis. (mayocliniclabs.com)
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease of the central nervous system that results in a progressive loss of motor function and ultimately death. (biorxiv.org)
AIMS: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease with complex aetiology. (bvsalud.org)
Background: As a disease of motor nervous system (motor neuron disease), amyotrophic lateral sclerosis (ALS) has a great impact on several aspects of quality of life (QoL). (ac.ir)

Spanning seven modules, the course reviews four key conditions: dementia, Parkinson's, multiple sclerosis and motor neurone disease. (neurologyacademy.org)
The following 5 modules comprise 22 sessions and cover focused neuro-palliative learning around dementia, Parkinson's, multiple sclerosis (MS) and motor neurone disease (MND), focusing on aspects of care that are specific to, or nuanced by, that condition. (neurologyacademy.org)

But peripheral axonal excitability studies have suggested that APB/FDI motor axons have more prominent persistent sodium currents than ADM axons, leading to higher axonal excitability and thereby more ready degeneration. (bmj.com)
At present, different forms of dementia are distinguished, including Alzheimer's disease (AD), dementia with Lewy bodies, frontotemporal dementia, corticobasal degeneration, progressive nuclear palsy and Parkinson dementia. (icgeb.org)
However, even in the more common and usually slowly progressive dementias such as Alzheimer's disease, frontotemporal lobar degeneration, dementia with Lewy bodies and other degenerative dementias, as well as vascular dementia, establishment and progression of the disease is occasionally surprisingly accelerated, leading to a clinical presentation of RPD. (touchneurology.com)
The presentation of degenerative disease in focal areas of the cerebral cortex is the hallmark of the family of diseases referred to as frontotemporal dementia (also termed frontotemporal lobar degeneration). (medscape.com)
Parkinson's disease (PD) is a progressive bradykinetic disorder commonly presenting unilaterally, affecting over 1% of people over 55 years of age and marked by the degeneration of dopaminergic neurons in the substantia nigra ( 1 , 2 ). (frontiersin.org)
A group of disorders characterized by degeneration and loss of motor neurons. (vin.com)

In this study, we used the Catwalk system to investigate changes in gait parameters in adult rats with unilateral 6-OHDA-induced lesions and the rescue effect of dopaminergic neuron transplantation on gait function. (biomedcentral.com)
At 8 weeks post-transplantation, engrafted dopaminergic neurons expressed tyrosine hydroxylase. (biomedcentral.com)
Ventral midbrain dopaminergic neurons project to the striatum as well as the cortex and are involved in movement control and reward-related cognition. (lu.se)
In Parkinson's disease, nigrostriatal midbrain dopaminergic neurons degenerate and cause typical Parkinson's disease motor-related impairments, while the dysfunction of mesocorticolimbic midbrain dopaminergic neurons is implicated in addiction and neuropsychiatric disorders. (lu.se)
Here, we have developed a human in vitro model that recapitulates key aspects of dopaminergic innervation of the striatum and cortex. (lu.se)
These spatially arranged ventral midbrain-striatum-cortical organoids (MISCOs) can be used to study dopaminergic neuron maturation, innervation and function with implications for cell therapy and addiction research. (lu.se)
is brain dysfunction that is characterized by basal ganglia dopaminergic blockade and that is similar to Parkinson disease, but it is caused by something other than Parkinson disease (eg, drugs, cerebrovascular disease, trauma, postencephalitic changes). (msdmanuals.com)
Research on dopaminergic neurotransmission has remained highly dynamic over the years and been extremely important in shaping our understanding of how the brain works in health and disease. (lu.se)

There can be lower motor neuron findings (e.g. muscle wasting, muscle twitching), upper motor neuron findings (e.g. brisk reflexes, Babinski reflex, Hoffman's reflex, increased muscle tone), or both. (wikipedia.org)
ALS, PBP, PLS) Motor neuron diseases are on a spectrum in terms of upper and lower motor neuron involvement. (wikipedia.org)
Lower motor neuron (LMN) findings include muscle atrophy and fasciculations, and upper motor neuron (UMN) findings include hyperreflexia, spasticity, muscle spasm, and abnormal reflexes. (wikipedia.org)
Pure lower motor neuron diseases, or those with just LMN findings, include PMA. (wikipedia.org)
Each of these neuromuscular diseases will produce lower motor neuron (LMN) disease, however significant variations in clinical signs may occur. (vin.com)

Some have just lower or upper motor neuron findings, while others have a mix of both. (wikipedia.org)
Pure upper motor neuron diseases, or those with just UMN findings, include PLS. (wikipedia.org)

Various patterns of muscle weakness occur in different motor neuron diseases. (wikipedia.org)
Also, the different motor neuron subtypes express specific molecular markers and show differential susceptibility towards neurodegenerative diseases. (rwth-aachen.de)

One of the most striking aspects of ALS is that some motor neurons -- specifically, those that control eye movement and eliminative and sexual functions -- remain relatively unimpaired in the disease," said study leader Christopher E. Henderson, PhD, the Gurewitsch and Vidda Foundation Professor of Rehabilitation and Regenerative Medicine, professor of pathology & cell biology and neuroscience (in neurology), and co-director of Columbia's Motor Neuron Center. (sciencedaily.com)
Researchers from the Sheffield Institute for Translational Neuroscience (SITraN), part of the AMBRoSIA biobank, suggest that offering routine genetic testing to MND patients could impact on disease sub-classification and clinical care. (mndassociation.org)

Motor neuron diseases or motor neurone diseases (MNDs) are a group of rare neurodegenerative disorders that selectively affect motor neurons, the cells which control voluntary muscles of the body. (wikipedia.org)
Currently, there are no approved treatments for the majority of motor neuron disorders, and care is mostly symptomatic. (wikipedia.org)
Motor neuron diseases are a group of disorders in which motor neurons (MNs) of the spinal cord and/or the motor cortex are the primary cell type affected where this perplexing feature can also be observed. (dzne.de)
Various conditions involving the central nervous system (CNS) can emerge as RPD, including Creutzfeld-Jakob disease (CJD) and other spongiform encephalopathies, vascular disorders, autoimmune and paraneoplastic encephalopathies, subacute infections, metabolic and toxic disorders and systemic diseases (see Table 1 ). (touchneurology.com)
However, it is important to point out that even neurodegenerative disorders such as Alzheimer's disease (AD), dementia with Lewy bodies and frontotemporal dementia present in rare cases as a subacute dementia instead of a slowly progressive deterioration of higher functions. (touchneurology.com)
While PD is most commonly associated with motor symptoms, such as tremor, rigidity, and gait disorders, there are numerous non-motor symptoms (NMS) associated with the condition such as hyposmia, constipation, cognitive impairment, anxiety, and depression ( 3 ). (frontiersin.org)
I also discuss how new diagnostic criteria for Diagnostic and Statistical Manual of Mental Disorders-5 and changes proposed for International Classification of Diseases may facilitate changes that allow neurologists to bring their management of patients with functional disorders in line with other multidisciplinary neurological disorders in the outpatient clinic. (bmj.com)
Overview of Movement and Cerebellar Disorders Voluntary movement requires complex interaction of the corticospinal (pyramidal) tracts, basal ganglia, and cerebellum (the center for motor coordination) to ensure smooth, purposeful movement. (msdmanuals.com)
The most common underlying diseases were neurologic disorders, such as cerebrovascular disease and motor neuron disease (3 patients), followed by diabetes mellitus (2 patients) and solid tumor (1 patient) (1 patient had both motor neuron disease and diabetes mellitus). (cdc.gov)
Here, we created a robust protocol that can be used later on for disease modeling of developmental or more early-onset brain disorders. (lu.se)
Huntington disease (HD) is a genetic, autosomal dominant, neurodegenerative disorder characterized clinically by disorders of movement, progressive dementia, and psychiatric and/or behavioral disturbance. (medscape.com)
Articles included in this review were primarily identified through a Medline search of the terms dementia, mild cognitive impairment, Alzheimer's disease, vascular dementia, frontotemporal dementia, Lewy body dementia, mental disorders, and stigma . (cdc.gov)

These data, along with the neuropathologic findings available in one case, suggest that dementia of motor neuron disease differs from that of Alzheimer's disease (AD). (northwestern.edu)
Parkinson's disease (PD) is the second most common neurodegenerative disorder after Alzheimer's disease. (biomedcentral.com)
We identified articles for review primarily by conducting a Medline search using the subject headings dementia, mild cognitive impairment, Alzheimer's disease, vascular dementia, frontotemporal dementia, and Lewy body dementia . (cdc.gov)

Although gait change is considered a useful indicator of severity in animal models of Parkinson's disease, systematic and extensive gait analysis in animal models of neurological deficits is not well established. (biomedcentral.com)
Gait disturbances are one of the most common motor problems in Parkinson's disease. (biomedcentral.com)
Parkinson's disease (PD) is a neurological disorder that is associated with both motor and non-motor symptoms (NMS). (frontiersin.org)

So, MMP-9 is not only labeling the most vulnerable groups of motor neurons, it is labeling the most vulnerable subtypes within those groups, as well," said Dr. Spiller. (sciencedaily.com)
Our ultimate goal is to understand the molecular origin of this distinctive neuronal vulnerability to design effective therapeutics targeting specific neuronal subtypes or phases of the disease. (dzne.de)
Spinal motor neurons are classified into functional motor neuron types and subtypes based on the muscle fibre type they innervate and they possess distinct morphological as well as electrophysiological properties. (rwth-aachen.de)

Often, these atypical dementias are associated with neurological symptoms and with motor neuron disease. (icgeb.org)
And as this neurological disease progresses, many may find themselves needing to access the support of a Speech Pathologist. (speechease.net.au)
St Francis Hospice takes on any patient with a terminal disease including kidney failure, terminal heart conditions and various neurological conditions such as motor neurone disease. (ehospice.com)
In endemic areas, the disease should be ruled out in all patients who develop unexplained neurological symptoms. (who.int)

While each motor neuron disease affects patients differently, they all cause movement-related symptoms, mainly muscle weakness. (wikipedia.org)
Symptoms of motor neuron diseases can be first seen at birth or can come on slowly later in life. (wikipedia.org)
Signs and symptoms depend on the specific disease, but motor neuron diseases typically manifest as a group of movement-related symptoms. (wikipedia.org)
Differential diagnosis can be challenging due to the number of overlapping symptoms, shared between several motor neuron diseases. (wikipedia.org)
The current experimental plan is structured to determine the future translational potential of this strategy by addressing the following question: can human stem cell-derived TE-NSPs physically reconstruct the nigrostriatal pathway to chronically restore dopamine tone in the striatum and improve PD motor symptoms? (michaeljfox.org)
This strategy has the potential to provide a transformative solution to replace lost neuroanatomy and alleviate the cause of motor symptoms for people with PD. (michaeljfox.org)
Doctors in nearly all medical specialties see patients with physical symptoms that are genuine but cannot be explained on the basis of a recognised 'organic' disease. (bmj.com)
As a result, motor neuron function declines and eventually these nerve cells die, leading to the signs and symptoms of juvenile primary lateral sclerosis. (medlineplus.gov)
Symptoms result from the selective loss of neurons, mostly in the caudate nucleus and putamen. (medscape.com)

TAR DNA-binding protein 43 (TDP-43), is a critical component of the non-homologous end joining (NHEJ) enzymatic pathway that repairs DNA double-strand breaks in pluripotent stem cell-derived motor neurons. (wikipedia.org)
In a follow-up experiment, the researchers confirmed that the product of MMP-9, MMP-9 protein, is present in ALS-vulnerable motor neurons, but not in ALS-resistant ones. (sciencedaily.com)
Fast-fatigable neurons (which are involved in movements like jumping and sprinting and are the first to die in ALS) were found to have the most MMP-9 protein, whereas slow neurons (which control posture and are only partially affected in ALS) had none. (sciencedaily.com)
TARDBP (TAR DNA-binding protein 43), also referred to as TDP-43, is a critical component of the non-homologous end joining (NHEJ) enzymatic pathway that repairs DNA double-strand breaks in pluripotent stem cell -derived motor neurons. (mdwiki.org)
TDP-43, a key mediator protein in ALS, is overexpressed in neurons in Thy1-hTDP-43WT mice. (bvsalud.org)
FINDINGS: We have found that targeting a single bioenergetic protein, PGK1, modulates motor neuron vulnerability in vivo. (bvsalud.org)
High sensitivity C-reactive protein (hsCRP) is a marker for cardiovascular and cerebrovascular diseases. (ac.ir)
SMA is caused by a deficiency of the survival of motor neuron (SMN) protein. (healthline.com)
The MND Research Hub is studying several aspects of Motor Neuron Disease (MND), such as the "waste" in neurons that mainly constitutes a nuclear RNA-binding protein known as TDP-43, that forms 'clumps' or aggregates in MND. (parnz.co.nz)
This protein is particularly abundant in motor neurons, the specialized nerve cells in the brain and spinal cord that control the movement of muscles. (medlineplus.gov)

In most neurodegenerative diseases certain neuronal subgroups degenerate fast while others, carrying the same mutations -if any-, subjected to theoretically analogous stress and displaying comparable functional properties remain unaffected, even at the latest stages of the disease. (dzne.de)
Ultimately, why do many neurons degenerate while some remain fully resistant? (dzne.de)

there are three main weakness patterns that are seen in motor neuron diseases, which are: Asymmetric distal weakness without sensory loss (e.g. (wikipedia.org)
Interneurons are the central nodes of neural circuits, enabling communication between sensory or motor neurons and the central nervous system. (lu.se)

abstract = "We describe the neuropsychological data from two cases of dementia of motor neuron disease. (northwestern.edu)

Centers for Disease Control and Prevention. (cdc.gov)
Good afternoon, I'm Commander Ibad Khan and I'm representing the Clinician Outreach and Communication Activity, COCA, with the Emergency Risk Communication Branch at the Centers for Disease Control and Prevention. (cdc.gov)
Before we begin, the following presentation contains some content made by external presenters and not by the Centers for Disease Control and Prevention, or the Department of Health and Human Services. (cdc.gov)
The Centers for Disease Control and Prevention (CDC) cannot attest to the accuracy of a non-federal website. (cdc.gov)

ALS is a brain disease that is closely related to Parkinson's, Alzheimer's, and Huntington's. (dontjuststand.com)
Effects of mutant huntingtin in oxytocin neurons on non-motor features of Huntington's disease. (lu.se)
Comorbidities and clinical outcomes in adult- and juvenile-onset Huntington's disease: a study of linked Swedish National Registries (2002-2019). (lu.se)
Decreased CSF oxytocin relates to measures of social cognitive impairment in Huntington's disease patients. (lu.se)
Microarray profiling of hypothalamic gene expression changes in Huntington's disease mouse models. (lu.se)
IKKβ signaling mediates metabolic changes in the hypothalamus of a Huntington's disease mouse model. (lu.se)
Hypothalamic expression of huntingtin causes distinct metabolic changes in Huntington's disease mice. (lu.se)

About 95% of ALS patients have abnormalities in the nucleus-cytoplasmic localization in spinal motor neurons of TDP43. (wikipedia.org)
Molecular mechanisms underlying spinal motor neuron diversification and differe. (rwth-aachen.de)
Spinal motor neurons are the elements of the neuromuscular system that regulate muscle fibre contractions. (rwth-aachen.de)

Motor neurone disease is a fatal and rapidly progressing disease that affects the brain and spinal cord. (radiotimes.com)
The researchers are still investigating how MMP-9 affects motor neuron function. (sciencedaily.com)
By definition, a rare disease affects fewer than 200,000 people, but many represent just hundreds or even dozens of patients. (jax.org)
If Whipple disease affects the small intestine, steatorrhea often is present. (medscape.com)

Neuromuscular junction (NMJ) dysfunction underlies several diseases, including congenital myasthenic syndromes (CMSs) and motor neuron disease (MND). (bvsalud.org)
Overexpression of DOK7 using AAV9 has been shown to ameliorate neuromuscular pathology in pre-clinical disease models of CMS and MND. (bvsalud.org)
Feline neuromuscular diseases may be classified according to their location as those involving peripheral nerves and/or nerve roots, those involving the neuromuscular junction, and those that involve muscle (1). (vin.com)
Physiological aspects of neuromuscular function. (cdc.gov)

That was significant, as ALS is an adult-onset disease," said co-lead author Krista J. Spiller, a former graduate student in Dr. Henderson's laboratory who is now a postdoctoral fellow at the University of Pennsylvania. (sciencedaily.com)
Onset between ages 21 and 40 years is sometimes called young or early-onset Parkinson disease. (msdmanuals.com)
In this case, the purpose was disease modeling of late-onset neurodegenerative diseases. (lu.se)
So, if you have a late-onset neurodegenerative disease that you are studying, you can model this more easily using direct conversion. (lu.se)
They reviewed clinical and genetic features in 34 cases of late-onset Huntington disease. (medscape.com)

The diagnosis of motor neurone disease (MND) is often difficult and may require observing symptom progression for some time before the diagnosis can be given with some certainty. (mndnsw.org.au)
Any parent with a sick child faces obstacles, but these compound exponentially when the diagnosis is for a rare genetic disease. (jax.org)
MND can affect every aspect of a person's life and has been associated with a wide range of psychological difficulties, which can occur from pre-diagnosis through to the condition's later stages. (lancs.ac.uk)
His motor and cognitive function deteriorated over several years following diagnosis. (medscape.com)

This peculiar pattern of dissociated atrophy of the intrinsic hand muscles is termed the 'split hand' and is rarely seen in diseases other than ALS. (bmj.com)
Motor potentials evoked by magnetic stimulation are significantly smaller when recorded from the thenar complex, compared with the hypothenar muscles, supporting a cortical mechanism. (bmj.com)
CONCLUSIONS: Thy1-hTDP-43WT mice represent a severe model of ALS, with NMJ pathology/denervation of distal muscles and motor neuron loss, as observed in ALS patients. (bvsalud.org)
Motor neurons send messages from the brain to muscles throughout the body. (dontjuststand.com)

Over the course of our program funded by the Michael J. Fox Foundation, we have developed a suite of micro-biofabrication techniques to enable the creation of the first living, fully-implantable tissue-engineered nigrostriatal pathways (TE-NSPs) that recapitulate key aspects of the structure and function of the native pathway. (michaeljfox.org)
While our initial MJFF program demonstrated proof-of-concept for this tissue engineering strategy to physically replace the nigrostriatal pathway, the observed improvements in striatal dopamine concentration and motor recovery were not consistent and/or sustained across all models. (michaeljfox.org)

Whilst I routinely see all aspect of general neurology, I maintain a particular academic and clinical interest in peripheral neuropathies and also lead the Cambridge Motor Neurone Disease (MND) Care Centre which provides coordinated multidisciplinary care to people living with MND across the East of England. (bupa.co.uk)
Peripheral nerve and muscle diseases result in varying degrees of paresis, muscle atrophy, hyporeflexia, and hypotonia. (vin.com)
Hyporeflexia, hypotonia, ataxia and proprioceptive positioning deficits are most characteristic of peripheral nerve disease. (vin.com)

This model therefore provides an ideal platform to investigate mechanisms of dying-back pathology, as well as NMJ-targeting disease-modifying therapies in ALS. (bvsalud.org)
Effective therapies are therefore likely to act on convergent pathways such as dysregulated energy metabolism, linked to multiple neurodegenerative diseases including ALS. (bvsalud.org)
I study the ability for implanted neuroblasts therapies for the disease. (lu.se)

Multiple disease phenotypes were assessed to determine the therapeutic potential of this approach, including axon growth and motor behaviour, survival and cell death following oxidative stress. (bvsalud.org)
Moreover, it was proposed that ESC-derived motor neurons possess some degree of diversity in vitro as both, alpha and gamma motor neuron-like phenotypes, are present in cell culture. (rwth-aachen.de)

Motor neuron diseases with both UMN and LMN findings include both familial and sporadic ALS. (wikipedia.org)
The findings, made in mice, explain why most but not all motor neurons are affected by the disease and identify a potential therapeutic target for this still-incurable neurodegenerative disease. (sciencedaily.com)
The findings of this review emphasise the importance of developing and validating psychometrically robust disease-specific measures of carer QOL. (lse.ac.uk)
Other common physical examination findings are a slight protrusion of the dorsal aspects of the scapulae when weight is placed on thoracic limbs, and a stiff thoracic limb gait. (vin.com)
Patients with Whipple disease may have any of the physical findings associated with malabsorption. (medscape.com)

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)

To understand why only some motor neurons are vulnerable to ALS, the researchers used DNA microarray profiling to compare the activity of tens of thousands of genes in neurons that resist ALS (oculomotor neurons/eye movement and Onuf's nuclei/continence) with neurons affected by ALS (lumbar 5 spinal neurons/leg movement). (sciencedaily.com)
Further, the researchers found that MMP-9 can be detected not just in lumbar 5 neurons, but also in other types of motor neurons affected by ALS. (sciencedaily.com)
Taking a closer look at the groups of vulnerable motor neurons, the researchers found differences in MMP-9 expression at the single-cell level. (sciencedaily.com)
That community - which includes academic researchers, disease-based organizations and pharmaceutical and biotech companies - is involved with every aspect of the center's work, from proposing which diseases to focus on to developing new treatments. (jax.org)
Researchers suggest that motor neurons and their long axons may be particularly vulnerable to changes in cell development. (medlineplus.gov)

Through this initial funding, we also demonstrated proof-of-concept that this strategy is able reconstruct the architecture of the native pathway, restore striatal dopamine levels and ease motor deficits in a Parkinson's model. (michaeljfox.org)
To address this challenge, the current program will systemically optimize the neuronal-axonal density (i.e., tune the "dose" of reestablished axonal inputs to the striatum) and the biomaterial encasement (i.e., more biodegradable and bioresorbable), ultimately towards the goal of consistent, sustained striatal dopamine levels and sustained amelioration of motor deficits. (michaeljfox.org)

In an attempt to better understand the epidemiologic and clinical aspects of XDR S. pneumoniae , we investigated clinical and microbiological characteristics of these cases of XDR S. pneumoniae . (cdc.gov)

Dementia with Lewy Bodies and Parkinson Disease Dementia Lewy body dementia includes clinically diagnosed dementia with Lewy bodies and Parkinson disease dementia. (msdmanuals.com)

The key aspect of selective motor neuron death in motor neuron diseases had been difficult to address using available mouse models. (dzne.de)

In both cases, a gradually progressive presenile dementia began prior to the development of motor neuron disease involving predominantly bulbar musculature. (northwestern.edu)

Progeny from this cross with no MMP-9 exhibited an 80-day delay in loss of fast-fatigable motor neuron function and a 25 percent longer lifespan, compared with littermates with two copies of the MMP-9 gene. (sciencedaily.com)
It has been suggested that neuronal loss spreads contiguously from the site of origin, becoming diffuse and resulting in complex motor deficits. (bmj.com)
Thy1-driven hTDP-43 expression increased steadily, correlating with developing hindlimb motor weakness and associated motor neuron loss in the spinal cord with a median survival of 21 days. (bvsalud.org)
NMJ pathology was restricted to motor nerve terminals, with denervation following the same time course as motor neuron loss. (bvsalud.org)
TIMP3 loss-of-function experiments were additionally performed to investigate its function in motor neurons. (rwth-aachen.de)
This shows loss of neurons, plump astrocytes (arrow), and microvacuolation of the superficial cortical layers. (medscape.com)
Research suggests that a lack of alsin and the subsequent loss of GTPase functions, such as endocytosis and the development of axons and dendrites, contribute to the progressive atrophy of motor neurons that is characteristic of this condition. (medlineplus.gov)
Exclusions included mild health states, motor impairment, borderline intellectual disability, mild hearing loss, mild vision loss and minor injuries, assuming people with these conditions would be less likely to require rehabilitation. (who.int)
Approximately 90% of patients with Whipple disease present with weight loss, and 70% of patients with Whipple disease complain of either diarrhea or arthralgias. (medscape.com)

Motor control was discussed as it relates to motor programs, reflexes, static and dynamic contractions and recruitment and rate coding. (cdc.gov)

The same effect on motor neuron function was seen when MMP-9 was inactivated in SOD1 mutant mice using chemical injections or virally mediated gene therapy. (sciencedaily.com)
He became convinced that given focus from mapping brain anatomy and the right conditions, immature neurons function to studying brain regeneration. (lu.se)

Why a given neuropathology does not impact all neurons of the same type to a similar level? (dzne.de)

Also known as Lou Gehrig's disease, ALS targets brain cells called motor neurons. (dontjuststand.com)

It was shown that the established in vitro differentiation protocol results in a highly pure motor neuron cell culture consisting of 90% ChAT-positive cells. (rwth-aachen.de)

Motor neuron disease (MND) is a rapidly progressive neurodegenerative condition with no known cure. (lancs.ac.uk)

Corrie producer Iain Macleod said: 'Motor Neurone Disease is something that many people might have heard of but perhaps don't know a lot about, even given the recent cases of public figures talking about their experiences of living with the condition. (radiotimes.com)
We have been privileged to work with the Motor Neurone Disease Association - including talking to people who have the condition and their families - to ensure we do justice to people's real-life experiences. (radiotimes.com)
Putting MND in front of millions of viewers every week will raise incredible awareness and help educate people who have never come across this disease - showing the day to day reality for those living with it and the impact on their families, friends and neighbours too. (radiotimes.com)
The Motor Neurone Disease Association (MNDA) supports people affected by MND, their families and carers and there are two regional advisers in Middlesbrough. (southtees.nhs.uk)
The Motor Neurone Disease Association (MNDA) is the largest charitable organisation supporting people affected by MND across England, Wales and Northern Ireland. (southtees.nhs.uk)
60 page book compiled following many requests from care providers, residential care staff and from people with motor neurone disease and their carers for information about caring for people with MND in the residential care setting. (mndnsw.org.au)
The aim of both of these types of research is to help people with Motor Neuron Disease and to find drug targets. (parnz.co.nz)

This two-day residential carers workshop focuses on the physical and emotional aspects of caring for a person with motor neurone disease. (mndnsw.asn.au)
It is difficult for motor neurone disease carers to take 'time out' from caring. (mndnsw.asn.au)
Objective Neurodegenerative diseases, such as dementia, have a profound impact on those with the conditions and their family carers. (lse.ac.uk)
Only studies which reported the development and/or validation of a disease-specific measure for adult family carers, and which were written in English, were eligible for inclusion. (lse.ac.uk)

The 1990-2019 Global Burden of Diseases data from the Institute for Health Metrics and Evaluation based in Seattle, Washington, United States of America, was used to estimate the need for rehabilitation services by presenting the prevalence, and associated years lived with disability, of 25 health conditions - disease causes, impairments and sequelae - that are amenable to rehabilitation at some point in the course of disease. (who.int)

Parkinson disease is a slowly progressive, degenerative disorder characterized by resting tremor, stiffness (rigidity), slow and decreased movement (bradykinesia), and eventually gait and/or postural instability. (msdmanuals.com)
Parkinson disease is usually idiopathic. (msdmanuals.com)
Lewy bodies appear in a temporal sequence, and many experts believe that Parkinson disease is a relatively late development in a systemic synucleinopathy. (msdmanuals.com)
Parkinson disease may share features of other synucleinopathies, such as autonomic dysfunction and dementia. (msdmanuals.com)
Though his family had no known history of HD, a number of family members were believed to have been afflicted by dementia, a staggering gait, emphysema, and Parkinson disease. (medscape.com)

A mutation in a single gene can cause a devastating genetic disease, such as cystic fibrosis, and collectively mutations cause from 7,000 to 10,000 different rare diseases. (jax.org)
In many cases, the challenges begin with what parents in the rare disease community term a "diagnostic odyssey," to pin down the exact genetic cause and find a clinical team with expertise in, or even knowledge of, the condition. (jax.org)
both experts in mouse models of human disease, are the principal investigators of the NIH-funded JAX The center will use the latest gene-editing technology to develop new mouse models of specific genetic diseases, characterize the models to ensure they reflect the human disease, and make the mice and data available to the worldwide scientific community. (jax.org)
How will Lutz and Murray determine which genetic disease will be the next SMA? (jax.org)
Spinal muscular atrophy (SMA), a genetic condition, can affect all aspects of your child's day-to-day life. (healthline.com)

However, synuclein can accumulate in many other parts of the nervous system, including the dorsal motor nucleus of the vagus nerve, basal nucleus of Meynert, hypothalamus, neocortex, olfactory bulb, sympathetic ganglia, and myenteric plexus of the gastrointestinal tract. (msdmanuals.com)

Anatomy19

Organisms4

Diseases31

Chemicals and Drugs14

Analytical, Diagnostic and Therapeutic Techniques and Equipment10

Psychiatry and Psychology5

Phenomena and Processes13

Disciplines and Occupations1

Health Care1

Dopamine neurons7

Amyotrophic lateral s8

Multiple Sclerosis2

Degeneration6

Dopaminergic8

Lower motor5

Upper motor2

Different motor neuron2

Neuroscience2

Disorders12

Alzheimer's Disease3

Parkinson's Disease3

Subtypes3

Neurological4

Symptoms9

Protein10

Degenerate2

Sensory2

Abstract1

Centers4

Huntington's7

Spinal motor3

Affects4

Neuromuscular4

Onset5

Diagnosis4

Muscles4

Nigrostriatal2

Peripheral3

Therapies3

Phenotypes2

Findings5

Syndromes1

Researchers5

Striatal dopamine2

Clinical aspects1

Dementia with L1

Selective1

Bulbar1

Loss9

Contractions1

Function2

Neuropathology1

Known as Lou Gehrig'1

Vitro1

Rapidly1

People7

Carers4

Impairments1

Parkinson5

Genetic5

Nervous system1