What are the alternative names for Amyotrophic Lateral Sclerosis (ALS)?

Amyotrophic Lateral Sclerosis (ALS) is also known as Motor Neuron Disease (MND). In the United States and Canada, it is also referred to as Lou Gehrig's disease (LGD), named after the famous baseball player who had the condition. Historically, it has also been called Charcot's disease.

What is the fundamental pathological process that defines Amyotrophic Lateral Sclerosis (ALS)?

ALS is characterized by the progressive degeneration and death of both upper motor neurons, located in the motor cortex of the brain, and lower motor neurons, found in the brainstem and spinal cord. This loss of motor neurons disrupts the signals sent from the brain to the muscles, leading to voluntary muscle contraction failure.

How does the term 'amyotrophic lateral sclerosis' describe the disease?

The name itself provides a description of the disease's effects. 'Amyotrophic' comes from Greek roots meaning 'no muscle nourishment,' referring to muscle wasting. 'Lateral' indicates the location of the nerve damage in the spinal cord, specifically in the lateral columns where the motor neurons reside. 'Sclerosis' refers to the hardening or scarring that occurs in the affected spinal cord areas due to the nerve damage.

What are the primary initial symptoms of ALS?

ALS often begins subtly, with early symptoms including gradual muscle stiffness, involuntary muscle twitches, increasing muscle weakness, and muscle wasting (atrophy). Depending on the location of the initial motor neuron damage, symptoms might first appear as awkwardness when walking, difficulty with fine motor tasks like buttoning a shirt, or problems with speech and swallowing.

How does ALS progress, and what are the ultimate consequences for the patient?

The progression of ALS involves the continuous death of motor neurons, leading to worsening muscle weakness and atrophy. Eventually, this impacts the muscles responsible for essential functions such as eating, speaking, moving, and breathing. The loss of respiratory muscle function is typically the cause of death.

What is the difference between limb-onset and bulbar-onset ALS?

ALS is classified based on the initial symptoms. Limb-onset ALS begins with weakness or wasting in the arms or legs, accounting for about two-thirds of classical ALS cases. Bulbar-onset ALS starts with difficulties in speaking (dysarthria) or swallowing (dysphagia) due to weakness in the muscles controlling these functions, representing about 25% of classical ALS cases.

What is the distinction between classical ALS, Primary Lateral Sclerosis (PLS), and Progressive Muscular Atrophy (PMA)?

Classical ALS involves the degeneration of both upper and lower motor neurons. Primary Lateral Sclerosis (PLS) specifically affects only the upper motor neurons, leading to spasticity, while Progressive Muscular Atrophy (PMA) affects only the lower motor neurons, causing weakness and atrophy. There is ongoing discussion about whether PLS and PMA are distinct diseases or variants of ALS.

What percentage of ALS cases are considered sporadic, and what is the cause of these cases?

The vast majority of ALS cases, approximately 90% to 95%, are classified as sporadic, meaning they have no known cause. While the exact cause remains unknown, it is believed that a combination of genetic predispositions and environmental factors likely contributes to the development of sporadic ALS.

What is the genetic basis for familial ALS?

Familial ALS, accounting for about 5% to 10% of cases, has a genetic cause, often indicated by a family history of the disease. Roughly half of these familial cases are linked to mutations in specific genes, with four genes (C9orf72, SOD1, FUS, and TARDBP) being responsible for nearly half of all familial ALS cases.

What are the typical age ranges for ALS onset?

ALS can affect individuals at any age, but it most commonly begins between the ages of 40 and 70, with an average age of onset around 55. Cases starting before age 45 are termed 'young-onset' ALS, and those starting before age 25 are called 'juvenile' ALS. Late-onset ALS (after age 65) is generally associated with faster progression.

Are sensory nerves and autonomic functions affected in ALS?

Generally, ALS does not affect sensory nerves or the autonomic nervous system. This means that most individuals with ALS typically retain their senses of hearing, sight, touch, smell, and taste, and their bladder and bowel functions are usually spared.

What are the common complications that can arise from ALS?

Complications of ALS include increased risk of falling due to muscle weakness, respiratory failure which is the usual cause of death, pneumonia (often aspiration pneumonia), and malnutrition due to difficulties with eating and swallowing.

How is ALS diagnosed?

There is no single definitive test for ALS. Diagnosis is primarily based on a physician's clinical assessment, including a thorough medical history and neurological examinations to track the progression of symptoms. Diagnostic tests are performed to rule out other conditions that might mimic ALS.

What diagnostic tests are commonly used to support an ALS diagnosis or rule out other conditions?

Electromyography (EMG) and nerve conduction velocity (NCV) tests can provide supportive evidence for an ALS diagnosis by detecting electrical activity in muscles and assessing nerve function. Magnetic resonance imaging (MRI) is often used to exclude other neurological issues like tumors, multiple sclerosis, or spinal cord compression.

What is the prognosis for individuals diagnosed with ALS?

The prognosis for ALS is generally poor, with a median survival time of two to four years after diagnosis. However, survival can vary significantly, with about 10% of individuals living for ten years or longer. Death is most commonly caused by respiratory failure.

What role does non-invasive ventilation (NIV) play in managing ALS?

Non-invasive ventilation (NIV) is a primary treatment for respiratory failure in ALS. It uses a mask connected to a ventilator to assist breathing, which can improve quality of life and prolong survival, particularly in patients with normal or moderately impaired bulbar function. It is typically used initially during sleep.

What are the FDA-approved medications that can slow the progression of ALS?

Two medications approved by the FDA that can modestly slow the progression of ALS are riluzole and edaravone. Riluzole may prolong survival by a few months, potentially by reducing glutamate release, while edaravone has shown to slow functional decline in some early-stage patients, possibly by protecting neurons from oxidative stress.

What is the significance of TDP-43 in ALS pathophysiology?

TDP-43 is a protein that is the main component of abnormal aggregations, known as inclusion bodies, found in the cytoplasm of motor neurons in about 97% of ALS cases. In ALS, TDP-43 appears to mislocalize from the cell nucleus to the cytoplasm, where it forms these toxic aggregates, contributing to neurodegeneration.

What is excitotoxicity, and how is it related to ALS?

Excitotoxicity is nerve cell damage caused by excessive stimulation from neurotransmitters, particularly glutamate. Motor neurons are particularly vulnerable in ALS due to lower calcium buffering and a specific type of glutamate receptor (AMPA receptor) that is more permeable to calcium. Reduced levels of the EAAT2 transporter, which clears glutamate from synapses, lead to increased glutamate levels and excitotoxicity.

What is the role of physical, occupational, and speech therapy in managing ALS?

Physical therapy aims to maintain strength, endurance, and range of motion to promote functional independence and prevent complications. Occupational therapy assists with daily living activities using adaptive equipment. Speech therapy helps individuals manage difficulties with speaking and swallowing, and can recommend augmentative communication devices.

Why is maintaining nutrition and hydration important for people with ALS?

Preventing weight loss and malnutrition is crucial for improving both survival and quality of life in individuals with ALS. Weight loss can occur due to muscle wasting and increased energy expenditure, as well as difficulties with swallowing (dysphagia), which affects a large percentage of patients.

When should a feeding tube be considered for someone with ALS?

A feeding tube, typically inserted via percutaneous endoscopic gastrostomy (PEG), should be considered if a person with ALS loses 5% or more of their body weight or if they can no longer swallow food and water safely. While there is weak evidence for survival benefit, PEG tubes are often used to improve quality of life by maintaining nutrition and hydration.

What is the role of palliative care in ALS management?

Palliative care should be initiated early after an ALS diagnosis to manage symptoms, improve quality of life, and provide psychosocial support for patients and caregivers. It also facilitates discussions about end-of-life preferences, helping patients create advance directives regarding medical interventions.

What are the typical epidemiological statistics for ALS worldwide?

ALS is the most common motor neuron disease in adults. Globally, about 1.9 new cases per 100,000 people occur annually, and approximately 4.5 people per 100,000 have ALS at any given time. The lifetime risk is estimated to be around 1 in 350 to 1 in 400.

Are there differences in ALS prevalence based on ethnicity or sex?

Yes, ALS is more common in white individuals compared to Black, Asian, or Hispanic populations. Men also have a higher risk than women, particularly for spinal-onset ALS, although this sex difference diminishes in individuals over age 70. The prevalence is also higher in certain geographic regions, though the reasons are often unknown.

Who were some key historical figures in the early understanding of ALS?

Descriptions of ALS date back to Charles Bell in 1824. François-Amilcar Aran first described progressive muscular atrophy (a form of ALS) in 1850. The French neurologist Jean-Martin Charcot was pivotal in connecting the symptoms to underlying pathology and coined the term 'amyotrophic lateral sclerosis' in 1874.

What is the significance of the 'Ice Bucket Challenge' in relation to ALS?

The 'Ice Bucket Challenge' became a viral social media phenomenon in August 2014, significantly raising awareness and funds for ALS research. Participants poured ice water over themselves and nominated others to do the same, donating to ALS organizations, which greatly boosted research funding and public understanding of the disease.

What are the main categories of genes associated with ALS?

Genes implicated in ALS can be broadly grouped by their normal function: those involved in protein degradation (like SOD1, VCP, OPTN), those maintaining the cytoskeleton and axonal transport (like DCTN1, PFN1, TUBA4A), and those related to RNA processing (like TDP-43, FUS, ANG, SETX).

What is the role of the C9orf72 gene in ALS?

The C9orf72 gene is the most common genetic mutation associated with ALS, found in about 40% of familial cases and 7% of sporadic cases. The mutation involves an expansion of a hexanucleotide repeat sequence, which can lead to motor neuron death through mechanisms involving RNA deposition, toxic dipeptide repeat proteins, and reduced levels of the normal C9orf72 protein.

What environmental factors have been linked to an increased risk of developing ALS?

While the exact cause of sporadic ALS is unknown, environmental factors thought to contribute include exposure to heavy metals (like lead and mercury), certain chemicals (pesticides, solvents), electric shock, physical injuries (including head injuries), and smoking, particularly in men. These factors likely interact with genetic predispositions over a lifetime.

How does ALS affect cognitive and behavioral functions?

Cognitive impairment or behavioral dysfunction occurs in 30-50% of individuals with ALS, often becoming more pronounced as the disease progresses. Common cognitive symptoms include language difficulties, executive dysfunction, and problems with social cognition and verbal memory. Emotional lability (pseudobulbar affect) is also common, characterized by inappropriate crying or laughing.

What are the key neuropathological findings in ALS?

Neuropathological examination in ALS typically reveals skeletal muscle atrophy, atrophy of the motor cortex, sclerosis (hardening) of the corticospinal and corticobulbar tracts, thinning of the hypoglossal nerves, and thinning of the anterior nerve roots in the spinal cord. The hallmark microscopic finding is the presence of abnormal protein aggregates, primarily TDP-43, within motor neurons.

What is the significance of the ALS Functional Rating Scale-Revised (ALSFRS-R)?

The ALSFRS-R is a 12-item survey used to measure the functional capacity of individuals with ALS. It produces a score from 0 to 48, with higher scores indicating better function. This scale is crucial for tracking disease progression in clinical trials and by physicians, as a loss of approximately one point per month is considered average.

What is the typical progression of symptoms in limb-onset ALS?

In limb-onset ALS, symptoms usually begin in one limb, often the legs or arms. If the legs are affected first, individuals might experience tripping or stumbling due to foot drop. If the arms are affected first, tasks requiring dexterity, such as writing or using keys, may become difficult. Symptoms typically spread to neighboring body regions over time.

How is respiratory failure managed in ALS patients?

Respiratory failure is the most common cause of death in ALS and is managed primarily through non-invasive ventilation (NIV). NIV uses a mask connected to a ventilator to support breathing, which can improve survival and quality of life. Invasive ventilation via tracheostomy is an option for advanced cases when NIV is insufficient.

What is the role of palliative care in the context of ALS?

Palliative care is essential from the time of diagnosis in ALS. It focuses on relieving symptoms, improving the patient's and family's quality of life, and facilitating discussions about end-of-life care preferences, including advance directives for medical interventions. Hospice care specifically addresses end-of-life symptom management to ensure a peaceful death.

What are the main differences in ALS terminology between the US/Canada and the UK/Australia?

In the United States and continental Europe, 'ALS' generally refers to all forms of motor neuron disease, including classical ALS, progressive bulbar palsy, and primary lateral sclerosis. However, in the UK and Australia, 'motor neuron disease' (MND) is the broader term encompassing all forms, while 'ALS' specifically refers only to the classical form involving both upper and lower motor neuron degeneration.

What is the relationship between ALS and Frontotemporal Dementia (FTD)?

ALS and Frontotemporal Dementia (FTD) are considered part of a common disease spectrum due to shared genetic, clinical, and pathological features. Approximately 10-15% of individuals with ALS show signs of FTD, and conditions like repeating phrases, apathy, and loss of inhibition are common behavioral symptoms. The C9orf72 gene mutation is a significant genetic link between ALS and FTD.

What are the potential roles of viruses and retrotransposons in ALS?

Some research suggests a link between ALS and increased expression of endogenous retroviruses (HERVs) and retrotransposon activation. These are genetic elements that can move within the genome. The protein TDP-43, which is often dysfunctional in ALS, plays a role in regulating these elements and may contribute to disease pathogenesis, with preliminary data suggesting potential benefits from antiviral therapies.

How do the King's and Milano-Torino (MiToS) staging systems help in ALS research?

The King's and MiToS staging systems are research tools used to categorize the progression of ALS. These systems, numbered from 1 to 4 (King's) or 0 to 5 (MiToS), help stratify patients for analysis in clinical trials by assessing functional decline across different domains, providing a standardized way to measure disease advancement.

What is the typical progression rate of ALS as measured by the ALSFRS-R?

While the rate of progression varies significantly among individuals, the ALS Functional Rating Scale-Revised (ALSFRS-R) is often used to track it. On average, people with ALS tend to lose about one ALSFRS-R point per month. However, brief periods of stabilization or minor improvements can occur, though significant or sustained reversals are rare.

What are the main challenges in diagnosing ALS?

Diagnosing ALS is challenging because its symptoms can overlap with those of other, more treatable neurological conditions. A definitive diagnosis relies on a process of elimination, requiring careful clinical evaluation, ruling out alternative diseases through various tests, and observing the progressive nature of the characteristic motor neuron degeneration.

What is the role of genetic testing in ALS diagnosis and management?

Genetic testing can help classify ALS as familial if a disease-causing mutation is identified, which can be important for family members. While historically limited to those with a clear family history, testing is increasingly considered for all ALS patients, especially as targeted therapies like tofersen become available for specific genetic mutations (e.g., SOD1-ALS).

What are the main proposed mechanisms of neurodegeneration in ALS?

Neurodegeneration in ALS is thought to involve multiple cellular and molecular processes. Key mechanisms include problems with protein degradation, dysfunction of the cytoskeleton and axonal transport, issues with RNA processing, excitotoxicity due to excess glutamate, mitochondrial dysfunction, and potentially prion-like propagation of misfolded proteins.

How does the disease affect a person's ability to breathe?

As ALS progresses, it weakens the respiratory muscles, including the diaphragm and intercostal muscles. This leads to shortness of breath, initially upon exertion and later at rest, and a reduced ability to cough effectively. Eventually, respiratory failure occurs, which is the most common cause of death in ALS patients.

What is the significance of 'locked-in syndrome' in the context of ALS?

Locked-in syndrome is a state where a person loses the ability to initiate and control all voluntary movement, except for eye muscles, due to severe muscle weakness. While the extraocular muscles are usually spared in ALS, allowing for eye-tracking communication, the progression towards locked-in syndrome represents an advanced stage of the disease.

What is the general approach to managing ALS symptoms?

Management of ALS focuses on alleviating symptoms and providing supportive care to enhance quality of life and prolong survival. This involves a multidisciplinary team approach, addressing issues like muscle cramps, spasticity, pain, fatigue, depression, anxiety, excessive saliva (sialorrhea), and nutritional support.

What is the role of palliative care in ALS?

Palliative care is crucial for managing the symptoms of ALS, such as pain and breathing difficulties, and improving the overall quality of life for patients and their families. It also involves discussing and planning for end-of-life care, ensuring that patient preferences are respected and unwanted interventions are avoided.

What is the typical survival rate for individuals with ALS?

The typical survival rate for individuals diagnosed with ALS is generally two to four years from the onset of symptoms. However, there is considerable variability, with some patients surviving for five to ten years or even longer, while others may pass away sooner, often due to respiratory failure.

What are the main differences between sporadic and familial ALS?

Sporadic ALS accounts for the majority of cases (90-95%) and has no known cause, though genetic and environmental factors are suspected. Familial ALS (5-10% of cases) has a clear genetic basis, often identified through family history or specific gene mutations, and tends to have an earlier age of onset compared to sporadic ALS.

How do genetic factors contribute to ALS risk?

Genetic factors play a significant role, particularly in familial ALS, where mutations in genes like C9orf72, SOD1, FUS, and TARDBP are common causes. Even in sporadic ALS, genetic predispositions are thought to interact with environmental exposures over a lifetime, contributing to the cumulative cellular damage that leads to motor neuron death.

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What is ALS?

A Progressive Neurodegenerative Disorder

Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND) or Lou Gehrig's disease, is a rare, terminal neurodegenerative disorder. It results in the progressive loss of motor neurons, the nerve cells responsible for controlling voluntary muscle contraction.

Global Health Challenge

ALS is the most common form of motor neuron disease. While its exact cause remains unknown in most cases, it affects individuals worldwide, leading to increasing muscle weakness, atrophy, and eventual respiratory failure. The disease significantly impacts quality of life and has a profound global health burden.

Impact on Motor Neurons

ALS specifically targets both upper motor neurons (in the brain and spinal cord) and lower motor neurons (connecting the spinal cord to muscles). This dual impact leads to a cascade of symptoms, including muscle stiffness, twitches, progressive weakness, and muscle wasting, ultimately affecting the ability to move, speak, swallow, and breathe.

Classification

By Motor Neuron Involvement

ALS is classified based on which motor neurons are affected:

Classical ALS: Affects both upper and lower motor neurons (approx. 70% of cases).
Primary Lateral Sclerosis (PLS): Affects only upper motor neurons (approx. 5% of cases).
Progressive Muscular Atrophy (PMA): Affects only lower motor neurons (approx. 5% of cases).

The distinction between these can sometimes be blurred as conditions can progress or evolve.

By Onset Location

Symptoms can manifest differently depending on the initial site of motor neuron degeneration:

Limb-Onset ALS: Begins with weakness in the arms or legs (most common).
Bulbar-Onset ALS: Starts with difficulty speaking, swallowing, or chewing.
Respiratory-Onset ALS: Less common, begins with breathing difficulties.

By Age of Onset

ALS can occur at any age, but typically starts between 40-75 years, with peak incidence around 60.

Young-Onset ALS: Before age 45 (approx. 10% of cases).
Juvenile ALS: Before age 25 (approx. 1% of cases), often genetic.
Late-Onset ALS: After age 65, often associated with more rapid progression.

Symptoms

Early Signs

Initial symptoms can be subtle and may include muscle stiffness, involuntary twitches (fasciculations), gradual muscle weakness, and muscle wasting (atrophy). These often begin in one part of the body, such as a limb or the bulbar region.

Progression of Symptoms

As the disease progresses, weakness spreads, impacting the ability to perform daily tasks. Symptoms can include:

Difficulty speaking (dysarthria) and swallowing (dysphagia).
Shortness of breath (dyspnea) due to respiratory muscle weakness.
Muscle cramps and spasticity.

Cognitive and Behavioral Changes

While ALS primarily affects motor neurons, approximately 30-50% of individuals experience cognitive or behavioral changes. This can range from mild executive dysfunction to frontotemporal dementia (FTD) in 10-15% of cases, affecting personality, behavior, and language.

Causes & Risk Factors

Unknown Origin (Sporadic ALS)

About 90-95% of ALS cases are classified as sporadic, meaning they have no known cause. While genetic and environmental factors are believed to play a role, specific triggers remain elusive.

Genetic Factors (Familial ALS)

Approximately 5-10% of ALS cases are familial, linked to inherited genetic mutations. Several genes have been identified, including C9orf72, SOD1, FUS, and TARDBP, which contribute to motor neuron degeneration.

Environmental and Lifestyle Factors

Research suggests potential links between ALS onset and certain environmental exposures and lifestyle choices, including:

Exposure to heavy metals (lead, mercury) and chemicals (pesticides, solvents).
Smoking.
History of electric shock or head injury.
Intense physical activity (though evidence is mixed).

Pathology

Motor Neuron Degeneration

The hallmark of ALS is the progressive death of upper and lower motor neurons. This leads to denervation of skeletal muscles, causing atrophy and weakness.

Protein Aggregation

In most ALS cases, abnormal protein aggregates, primarily TDP-43, are found within neurons. In familial forms, mutations in genes like SOD1 or FUS lead to the accumulation of misfolded proteins, contributing to cellular dysfunction and death.

Biochemical Pathways

Several cellular mechanisms are implicated, including excitotoxicity (due to excess glutamate), oxidative stress, mitochondrial dysfunction, impaired protein degradation, and disruptions in axonal transport. These pathways collectively contribute to the vulnerability and eventual death of motor neurons.

Diagnosis

Clinical Assessment

There is no single definitive test for ALS. Diagnosis relies on a physician's comprehensive clinical evaluation, including a detailed medical history and neurological examinations to identify signs of upper and lower motor neuron degeneration.

Diagnostic Tools

Tests are performed to exclude other conditions that mimic ALS symptoms:

Electromyography (EMG) & Nerve Conduction Velocity (NCV): Assess muscle and nerve electrical activity.
MRI Scans: Rule out structural issues like tumors or spinal cord compression.
Blood and Urine Tests: Exclude metabolic or infectious causes.
Genetic Testing: May be used to identify familial ALS mutations.

Differential Diagnosis

Key conditions that must be ruled out include multifocal motor neuropathy, Kennedy's disease, spinal muscular atrophy, multiple sclerosis, and various myopathies, due to overlapping symptoms.

Management

Disease-Modifying Treatments

While there is no cure, treatments aim to slow progression and manage symptoms:

Riluzole: May modestly prolong survival.
Edaravone: May slow functional decline in early stages.
Tofersen: Approved for SOD1-ALS patients.

Note: AMX0035 (Relyvrio) was withdrawn from the market in April 2024.

Supportive Care

Crucial for maintaining quality of life:

Respiratory Support: Non-invasive ventilation (NIV) can improve survival and quality of life. Invasive ventilation is an option in advanced stages.
Nutritional Support: Feeding tubes (e.g., PEG) help maintain weight and hydration.
Therapies: Physical, occupational, and speech therapy aid mobility, daily activities, and communication.

Palliative and End-of-Life Care

Palliative care should be integrated early to manage symptoms like pain, anxiety, and dysphagia. Open discussion about end-of-life preferences and advance directives is vital for respecting patient autonomy.

Epidemiology

Global Prevalence

ALS is considered a rare disease, with incidence rates varying globally. Worldwide, approximately 1.9 new cases per 100,000 people occur annually. Prevalence is higher in Europe and North America, particularly among white populations.

Age and Sex

The risk of ALS increases with age, with most diagnoses occurring between 50 and 75 years. Men are slightly more likely to develop ALS than women, especially at younger ages.

Future Trends

The number of ALS cases is projected to increase globally, largely due to aging populations. Research into geographic clusters and potential environmental factors continues.

History

Early Descriptions

The disease was first described in the 1820s by Charles Bell. In 1869, Jean-Martin Charcot provided the first comprehensive description and coined the term "amyotrophic lateral sclerosis" in 1874.

Lou Gehrig's Disease

In the United States and Canada, ALS is often referred to as Lou Gehrig's disease, named after the famous baseball player diagnosed with the condition in 1939, bringing significant public awareness.

Scientific Advancements

Key milestones include the development of diagnostic criteria (El Escorial criteria), identification of genetic mutations (like SOD1, C9orf72), and the introduction of the first disease-modifying drugs like Riluzole.

Society & Culture

Notable Figures

Individuals like physicist Stephen Hawking, who lived with ALS for decades, and baseball player Lou Gehrig have significantly raised public awareness. Their resilience and contributions have inspired many.

The Ice Bucket Challenge

In 2014, the viral "Ice Bucket Challenge" phenomenon dramatically increased global awareness and funding for ALS research, demonstrating the power of social media in driving scientific progress and patient advocacy.

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References

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ALS Unveiled

💡 Dive in with Flashcard Learning! 💡

What is ALS? ℹ️

⚡ A Progressive Neurodegenerative Disorder

🌐 Global Health Challenge

🔬 Impact on Motor Neurons

Classification 🧬

📍 By Motor Neuron Involvement

🚶 By Onset Location

📅 By Age of Onset

Symptoms 🩺

⚠️ Early Signs

🗣️ Progression of Symptoms

🧠 Cognitive and Behavioral Changes

Causes & Risk Factors 🛡️

❓ Unknown Origin (Sporadic ALS)

👪 Genetic Factors (Familial ALS)

🌍 Environmental and Lifestyle Factors

Pathology 🔬

💥 Motor Neuron Degeneration

🧩 Protein Aggregation

⚡ Biochemical Pathways

Diagnosis 🩺

🤔 Clinical Assessment

🔬 Diagnostic Tools

⚖️ Differential Diagnosis

Management 🧑‍⚕️

💊 Disease-Modifying Treatments

🌬️ Supportive Care

❤️ Palliative and End-of-Life Care

Epidemiology 📈

🌍 Global Prevalence

👴 Age and Sex

📈 Future Trends

History 📜

📜 Early Descriptions

⚾ Lou Gehrig's Disease

🔬 Scientific Advancements

Society & Culture 🎭

🌟 Notable Figures

💧 The Ice Bucket Challenge

Teacher's Corner 🧑‍🏫

Edit and Print this course in the Wiki2Web Teacher Studio

True or False? 🤔

❓ Test Your Knowledge! ❓

Gamer's Corner 🎮

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Explore More Topics

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References

📜 References

Feedback & Support 👍

To report an issue with this page, or to find out ways to support the mission, please click here.

Disclaimer ⚠️

📜 Important Notice

Dive in with Flashcard Learning!

What is ALS?

A Progressive Neurodegenerative Disorder

Global Health Challenge

Impact on Motor Neurons

Classification

By Motor Neuron Involvement

By Onset Location

By Age of Onset

Symptoms

Early Signs

Progression of Symptoms

Cognitive and Behavioral Changes

Causes & Risk Factors

Unknown Origin (Sporadic ALS)

Genetic Factors (Familial ALS)

Environmental and Lifestyle Factors

Pathology

Motor Neuron Degeneration

Protein Aggregation

Biochemical Pathways

Diagnosis

Clinical Assessment

Diagnostic Tools

Differential Diagnosis

Management

Disease-Modifying Treatments

Supportive Care

Palliative and End-of-Life Care

Epidemiology

Global Prevalence

Age and Sex

Future Trends

History

Early Descriptions

Lou Gehrig's Disease

Scientific Advancements

Society & Culture

Notable Figures

The Ice Bucket Challenge

Teacher's Corner

True or False?

Test Your Knowledge!

Gamer's Corner

Discover other topics to study!

References

Feedback & Support

Disclaimer

Important Notice