Amyotrophic lateral sclerosis: A 40-year personal perspective

Eisen, Andrew

doi:10.1016/j.jocn.2008.07.072

Cited by 88 publications

(63 citation statements)

References 83 publications

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“…The symptoms occur during or after physical activity in early stages but as the disease progresses they also occur in the sitting position. Shortness of breath, shorter speech, the use of respiratory muscles and increase in the number of respiratory symptoms such as weak coughing are important evidences that show decrease in the respiratory muscles function [2].…”

Section: Case Reportmentioning

confidence: 99%

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From Respiratory Distress to Diagnosis of Amyotrophic Lateral Sclerosis

Naldan¹,

Aydin²,

Ahıskalıoğlu³

et al. 2016

Med-Science

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Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease of motor neurons, which results in weakness and atrophy of voluntary skeletal muscles. ALS is the most common form of motor neuron disease (MND). It is currently incurable and treatment is largely limited to supportive care. Family history is associated with an increased risk of ALS. The disease usually does not affect cognitive abilities, but causes muscle weakness. Respiratory function in the early stages of the disease is generally normal, shortness of breath occurs as the disease progresses. This paper has been submitted about ALS patient who wasdiagnosed and treated in intensive care unit followed by severe respiratory failure.

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Section: Case Reportmentioning

confidence: 99%

“…The disease is fatal within nearly three years after diagnosis has been made. Death usually occurs due to respiratory failure [2]. The patients are usually diagnosed with muscle weakness.…”

Section: Introductionmentioning

confidence: 99%

From Respiratory Distress to Diagnosis of Amyotrophic Lateral Sclerosis

Naldan¹,

Aydin²,

Ahıskalıoğlu³

et al. 2016

Med-Science

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“…Amyotrophic lateral sclerosis; associated with the common upper and lower motor neuron degeneration, generally resulting in death due to respiratory failure an average of three years after the beginning of symptoms [46]. Microglial activation, astrocytosis, lymphocyte infiltration and dendritic cells are accompanied with progressive degeneration of motor neurons [47].…”

Section: Amyotrophic Lateral Sclerosismentioning

confidence: 99%

Stem Cell Therapy in the Treatment of Neurological Diseases

Kocaoğlu¹,

Korucu²,

Civlan³

et al. 2014

rain Disord Ther

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Before a half century, regeneration of neurons has been admitted as an impossible event. Thus, neurodegenerative disorders (e.g. Parkinson's disease, Alzheimer's disease, multiple sclerosis), vascular events (e.g. stroke) and traumatic diseases (e.g. spinal cord injury) have been admitted as incurable diseases. Afterward, tissue reparative and regenerative potential of stem cell researches for these disorders drew attention of the scientist to replacement therapy. Now, there are hundreds of current experimental and clinical regenerative treatment studies. One of the most popular treatment methods is cell transplantation. For this purpose many types of stem cells such as mononuclear stem cells, mesenchymal stem cells, and olfactory ensheathing cells can be used. As a result, cell transplantation has become a promising therapeutic option for these neurologic disorders. In this article, we reviewed stem cell treatment modalities for neurological disorders in the light of current literature.

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“…More than 5,000 people in the USA are diagnosed with ALS each year (http://www.alsa.org), and the global incidence of ALS is about 2 per 100,000 persons [3]. The average age of disease onset is 55-65 years of age, and it occurs more frequently in men than in women (http://www.…”

Section: Introductionmentioning

confidence: 99%

“…The cause of the spasticity, paralysis, and death is believed to be progressive degeneration and elimination of upper motor neurons in cerebral cortex, and lower motor neurons in brainstem and spinal cord [1,4,5]. Other than life support management, no effective treatments exist for ALS [2,3,6]. Most individuals (~90 %) who develop classical ALS are identified as sporadic, with few known genetic contributions [1].…”

Section: Introductionmentioning

confidence: 99%

Aberrant Regulation of DNA Methylation in Amyotrophic Lateral Sclerosis: A New Target of Disease Mechanisms

Martin

Wong

2013

Neurotherapeutics

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Amyotrophic lateral sclerosis (ALS) is the third most common adult-onset neurodegenerative disease. A diagnosis is fatal owing to degeneration of motor neurons in brain and spinal cord that control swallowing, breathing, and movement. ALS can be inherited, but most cases are not associated with a family history of the disease. The mechanisms causing motor neuron death in ALS are still unknown. Given the suspected complex interplay between multiple genes, the environment, metabolism, and lifestyle in the pathogenesis of ALS, we have hypothesized that the mechanisms of disease in ALS involve epigenetic contributions that can drive motor neuron degeneration. DNA methylation is an epigenetic mechanism for gene regulation engaged by DNA methyltransferase (Dnmt)-catalyzed methyl group transfer to carbon-5 in cytosine residues in gene regulatory promoter and nonpromoter regions. Recent genome-wide analyses have found differential gene methylation in human ALS. Neuropathologic assessments have revealed that motor neurons in human ALS show significant abnormalities in Dnmt1, Dnmt3a, and 5-methylcytosine. Similar changes are seen in mice with motor neuron degeneration, and Dnmt3a was found abundantly at synapses and in mitochondria. During apoptosis of cultured motor neuron-like cells, Dnmt1 and Dnmt3a protein levels increase, and 5-methylcytosine accumulates. Enforced expression of Dnmt3a, but not Dnmt1, induces degeneration of cultured neurons. Truncation mutation of the Dnmt3a catalytic domain and Dnmt3a RNAi blocks apoptosis of cultured neurons. Inhibition of Dnmt catalytic activity with small molecules RG108 and procainamide protects motor neurons from excessive DNA methylation and apoptosis in cell culture and in a mouse model of ALS. Thus, motor neurons can engage epigenetic mechanisms to cause their degeneration, involving Dnmts and increased DNA methylation. Aberrant DNA methylation in vulnerable cells is a new direction for discovering mechanisms of ALS pathogenesis that could be relevant to new disease target identification and therapies for ALS.

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Amyotrophic lateral sclerosis: A 40-year personal perspective

Cited by 88 publications

References 83 publications

From Respiratory Distress to Diagnosis of Amyotrophic Lateral Sclerosis

From Respiratory Distress to Diagnosis of Amyotrophic Lateral Sclerosis

Stem Cell Therapy in the Treatment of Neurological Diseases

Aberrant Regulation of DNA Methylation in Amyotrophic Lateral Sclerosis: A New Target of Disease Mechanisms

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