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Results for “SCLEROSIS, AMYOTROPHIC LATERAL”

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PubMed1993

Ubiquitin-positive inclusion in anterior horn cells in subgroups of motor neuron diseases: a comparative study of adult-onset amyotrophic lateral sclerosis, juvenile amyotrophic lateral sclerosis and Werdnig-Hoffmann disease.

This report concerns the expression of ubiquitin in anterior horn cells of various subgroups of adult and infantile motor neuron disease (MNDs); immunohistochemical techniques were employed. Ubiquitin-positive skein-like inclusions (SLIs) were found in all cases of adult-onset amyotrophic lateral sclerosis (ALS), including 16 cases with sporadic ALS, two cases of familial ALS with posterior column degeneration and Lewy body-like hyaline inclusions (LBHIs), two sporadic ALS cases with LBHIs, and three cases of sporadic ALS with dementia. SLIs were not found in anterior horn cells of 5 cases with Werdnig-Hoffmann disease (WHD). However, granular ubiquitin-positive deposits were seen in ballooned neurons of WHD patients. No ubiquitinated materials were found in the perikarya of two sporadic juvenile ALS patients with basophilic inclusions (BIs), but granular ubiquitin-immunoreactive deposits were occasionally observed in the BIs. These results suggest that ubiquitin-positive SLIs are characteristic features of various forms of adult-onset ALS and that aggregated ubiquitinated granules are characteristic of ballooned neurons of WHD. Ubiquitinated structures and their distribution patterns may reflect degenerative processes of anterior horn cells, and may be useful for classifying subgroups of motor neuron diseases.

Adult↗

PubMed1999

[Our first experiences with intermittent assisted ventilation in patients with amyotrophic lateral sclerosis].

Amyotrophic lateral sclerosis is one of the most frequent neuromuscular diseases in adults. Chronic respiratory failures is an almost compulsory symptom in the progression of this disease, and in association with pulmonary infections, responsible for the majority of deaths. We report on a series of 43 patients. An advanced stage of clinical disease was seen in half of them. After detection of respiratory failure corresponding to the guidelines of muscle centres of the DGM (Deutsche Gesellschaft für Muskelerkrankungen), seven patients (16.3%) were willing to be provided with a system for intermittent non-invasive ventilation. All patients achieved stabilisation of respiratory function, both with respect to the normalisation of arterial gases and subjective improvement of well-being. During the course of treatment four patients deliberately underwent permanent invasive ventilation. In our opinion home ventilation is a valid additional tool in the palliative treatment of amyotrophic lateral sclerosis. The treatment, however, must be supported by an interdisciplinary team.

Adult↗

PubMed2026

Circular RNAs in amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by the progressive loss of motor neurons, with most cases lacking a clear genetic basis. Emerging evidence highlights the involvement of non-coding RNAs, particularly circular RNAs (circRNAs), in disease onset and progression. Here, we investigated circRNAs implicated in ALS and related motor neuron diseases (MNDs). Here, we provide a general overview of circular RNA metabolism and cellular functions. We then present our systematic literature review that identified ALS-associated circRNAs, followed by in silico analyses of 15 circular RNA candidates that were selected based on the most compelling data regarding ALS. Our results revealed that several circular RNAs regulate ALS-related genes, such as unfolded protein response, oxidative stress, cell cycle regulation, and apoptosis. Protein-RNA interaction analysis further showed that ALS-related circRNAs can sponge 20 RNA-binding proteins. Additionally, molecular docking analysis demonstrated that ALS-associated FUS variants significantly alter its binding affinity to circular RNAs. RNA-seq data from ALS patients confirmed significant alterations in the expression of host genes of ALS-related circRNAs and hub proteins in ALS-affected CNS tissues. Collectively, our findings identify circRNAs as potential key contributors to ALS pathogenesis.

Amyotrophic Lateral Sclerosis↗

PubMedDate not supplied

[Motor neuron disease: primary lateral sclerosis and amyotrophic lateral sclerosis].

AIM: Primary lateral sclerosis and amyotrophic lateral sclerosis are amongst motor neuron diseases. Differences between these two disorders are stressed by this paper. SOURCE OF DATA: Articles pertinent to this subject from the past 10 years. CONCLUSION: Both disorders are of neurodegenerative pathogenesis, and motor neurons are selectively involved. Unless only motor neurons from central nervous system are involved in primary lateral sclerosis, in amyotrophic lateral sclerosis motor neurons are involved both in central and in peripheral nervous system. Clinical neurophysiological and radiological features are helpful in differential diagnosis of these diseases. Primary lateral sclerosis has better prognosis and much higher survival rate.

Amyotrophic Lateral Sclerosis↗

PubMed2026

Comparison of Proteomic Analysis of Cerebrospinal Fluid From Neurological Patients With and Without Amyotrophic Lateral Sclerosis.

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterised by progressive muscle weakness in both bulbar and extremity muscles, leading to a diverse clinical phenotype with motor and non-motor symptoms. Approximately 85% of ALS cases are sporadic (sALS), while the remaining 10%-15% are familial (fALS). Biological biomarkers of sporadic ALS remain poorly understood, hindering precise patient screening, delaying diagnosis and negatively affecting prognosis. This study aims to identify potential proteomic biomarkers by comparing the cerebrospinal fluid (CSF) of sALS patients with that of patients suffering from other neurological diseases. Liquid chromatography-tandem mass spectrometry (LC-MS/MS) was used for proteomic profiling of CSF samples from 24 sALS patients and 26 patients with other neurological diseases. The complete protein expression profiles were compared using a two-tailed Student's t-test, with a p < 0.05 considered statistically significant with additional FDR correction at the 0.1 level. Proteomic analysis of CSF samples identified significant quantitative changes in 96 proteins with threshold p < 0.05 and 74 proteins with FDR < 0.1 between sALS and non-ALS patients, including alterations in proteins associated with neurodegenerative processes, such as amyloid precursor proteins and inflammatory markers. CSF proteomic analysis reveals altered inflammatory and neurodegenerative metabolic pathways, providing valuable insights into the proteomic landscape of sALS. Several dysregulated proteins were consistent with the disease mechanisms highlighted in previous studies. These findings represent a step forward in developing personalised approaches for diagnosing and managing the disease.

Humans↗

PubMed2006

[Differential diagnosis and atypical subsets of amyotrophic lateral sclerosis].

Amyotrophic lateral sclerosis (ALS) is a progressive degeneration of upper and lower motor neurons. In the absence of any validated biological marker, the diagnosis of ALS depends upon recognition of characteristic symptoms and signs together with supportive electrophysiological findings. The diagnosis of ALS is easy to recognize in its fully developed form but during the early stages both false positive and false negative diagnoses are common. In clinical practice, diagnostic difficulties mostly arise with patients who present either with only upper motor neuron, or with only lower motor neuron signs. It may be difficult to distinguish ALS with clinically predominant lower motor neuron involvement from alternative diagnoses including spinal atrophies of adult onset, Kennedy's disease, inclusion body myositis and motor neuropathies with conduction blocks. The diagnosis of ALS related syndromes (progressive muscular atrophy, primary lateral sclerosis and progressive bulbar palsy) requires the elimination of alternate diagnoses. This paper reviews the main characteristics of diseases mimicking ALS and the atypical subsets of ALS.

Amyotrophic Lateral Sclerosis↗

PubMed1986

Failure of immunosuppression with a ten- to 14-day course of high-dose intravenous cyclophosphamide to alter the progression of amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a progressive disorder of the nervous system for which there is no known treatment. Because recent studies have suggested that there may be abnormalities of the immune function in patients with ALS and since we have found a beneficial effect from a short course of intensive immunosuppression with cyclophosphamide in progressive multiple sclerosis, we treated six patients with ALS with a ten- to 14-day course of intensive immunosuppression in a pilot study. At 18 months following therapy, all patients showed a continued progression of the disease; four of the six patients died. We conclude that this form of immunosuppression does not alter the course of ALS.

Aged↗

PubMed1994

Respiratory dysfunction in amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder of the voluntary motor system. Involvement of the respiratory system is inevitable and leads to the development of respiratory failure, the usual cause of death in this disorder. ALS at present is incurable, and only symptomatic treatment is available. This article presents guidelines for the recognition and management of respiratory failure.

Amyotrophic Lateral Sclerosis↗

PubMed1999

Estrogen replacement therapy in women with amyotrophic lateral sclerosis.

Amyotrophic Lateral Sclerosis (ALS) occurs more commonly in men than in women, and women get the disease later in life compared to men. This epidemiologic aspect of the disease raises the question as to whether estrogen may be neuroprotective in delaying or preventing ALS. Postmenopausal women with ALS were separated into two groups dependent upon whether or not they took estrogen replacement therapy. Women who used estrogen had onset of their disease at an earlier age compared to those not taking hormonal replacement. There was no difference in survival in those patients taking estrogen compared to those not on the medication. Women with ALS were more likely to take estrogen compared to a control group of patients with neurological diseases other than motor neuron disease. Therefore, no evidence for a neuroprotective role of estrogen in postmenopausal women with ALS was found.

Adult↗

PubMed1998

Comprehensive management of amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive motor neuron disease that poses a myriad of clinical problems. Patients with ALS are best treated in a multidisciplinary setting involving physicians, clinical nursing specialists, and physical, occupational, speech, and respiratory therapists, as well as psychologists and social workers. Palliative and rehabilitative strategies may ease suffering, while new treatments provide hope for effective treatment of this disease.

Algorithms↗

PubMed1996

[Clinical aspects of amyotrophic lateral sclerosis].

Amyotrophic lateral sclerosis is a progressive degenerative disease of upper and lower motor neurons with a prevalence of 4.3/100.000. The clinical symptoms include peripheral weakness and central spastic paresis and bulbar paralysis (weakness of mimic muscles, atrophy of the tongue, dysarthria). The prognosis leads to death within a few years. Pathogenetic factors are free O2-radicals, a disturbance of glutamate-metabolism, abnormal accumulation of neuronal proteins and autoimmunological mechanisms.

Amyotrophic Lateral Sclerosis↗

PubMed2006

[Management of lost independence and its social consequences in amyotrophic lateral sclerosis].

Amyotrophic lateral sclerosis is characterized by progressive unavoidable loss of independence but with preserved intellectual capacity. Care requires human and technical compensation taking into account the desires of the patient and his/her family. Care should be taken to avoid isolating from the social environment by implementing coherent material and human organizations aimed at maintaining patient comfort and acceptable quality of life "to the end" while respecting the rights and will of the care recipient.

Amyotrophic Lateral Sclerosis↗

PubMed2006

Does VEGF represent a potential treatment for amyotrophic lateral sclerosis?

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease. The pathogenesis of ALS is unclear and there is no effective treatment. Vascular endothelial growth factor (VEGF) is a cytokine that has a protective function via angiogenic, neurotrophic, gliotrophic and anti-apoptotic activity. Data indicate that VEGF can inhibit neurodegeneration in ALS and may have therapeutic potential in this disease. The use of gene therapy to deliver VEGF into the central nervous system is being evaluated.

Amyotrophic Lateral Sclerosis↗

PubMed1998

Foramen magnum meningioma presenting as amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a progressive, degenerative disease of the nervous system. It affects adults in all age groups and leads to death few years after the diagnosis. The etiology is unknown, and there no convincing medical or surgical therapy Some diseases can mimic ALS. If diagnosed early these may be treatable, but if misdiagnosed as ALS and left untreated they may be fatal. We present a case of foramen magnum meningioma presenting clinically as ALS. After surgery, the symptoms disappeared and the neurological deficits improved.

Amyotrophic Lateral Sclerosis↗

PubMed1989

Using phonation time to estimate vital capacity in amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a neuromuscular disease in which progressive respiratory weakness is usually the proximate cause of death. Since continued assessment of respiratory function is important in predicting progression, phonation time was measured in a group of 41 ALS patients and correlated with measured vital capacity. The findings show that the variance in phonation time accounts for nearly 50% of the variability of vital capacity, and therefore, successive measurements of phonation time are a useful means of estimating the loss of vital capacity.

Adult↗

PubMed2000

Symptomatic treatment of respiratory and nutritional failure in amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterised by death of upper and lower motor neurones. Nutritional and respiratory failure occurs in most patients with ALS. Nutritional failure occurs primarily as a result of dysphagia, although malnutrition may also develop in the absence of clinically apparent dysphagia. The optimal management of nutrition in early ALS has not been established. In later stages of the disease, parenteral nutritional support using percutaneous endoscopic gastrostomy confers a significant survival benefit in selected patients. Respiratory failure occurs as a result of bulbar, cervical and thoracic loss of motor neurones. Inspiratory muscles are preferentially affected. Management of respiratory failure includes the use of strategies that limit aspiration pneumonia, the reduction in secretions, and positioning of the patient to a maximal mechanical advantage. Use of non-invasive positive pressure ventilation in appropriate patients significantly enhances survival. The decision to undertake invasive mechanical ventilation should be made prior to the development of symptoms that might warrant this intervention. The progressive nature of the condition should be taken into account when such a decision is discussed with the patient and carer. Further studies are required to determine the optimal nutritional requirements of patients with ALS, and to elucidate the physiological changes involved in the decline in respiratory function.

Amyotrophic Lateral Sclerosis↗

PubMedDate not supplied

The otolaryngologic presentation of amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis is a progressive dengenerative neuromuscular disease of insidious onset. It involves upper and lower motor neurons and causes both spastic and atrophic muscular symptoms. More than one fourth of patients have complaints relating to the head and neck (bulbar palsy); thus, the otolaryngologist may be the first physician to see them. Predominant symptoms are slurred speech, hoarseness, dysphagia, and dyspnea. Muscular weakness, atrophy, and fasciculation are noted on examination. The course is relentless, and only 20% of patients survive five years after diagnosis.

Adult↗

PubMed2006

[Physical therapy in amyotrophic lateral sclerosis].

Amyotrophic lateral sclerosis is a neurodegenerative disease, without any curative treatment. Clinical expression is variable and related to loss of motor neurons in the cortex, brain stem and spinal cord. There is little scientific evidence demonstrating the usefulness of physical therapy in this disease. Only stretching exercises, proprioceptive neuromuscular facilitation techniques and functional mobility training seem to have a real benefit in terms of spasticity, quality-of-life and pain. The main objective of physical therapy appears to be the preservation of optimal quality-of-life throughout the course of this incurable degenerative disease.

Amyotrophic Lateral Sclerosis↗