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Stages

Delayed Diagnosis

Treatment

Frequently Asked Questions

Your perception of your symptoms describes the effects of the disease. The staging systems can help you and your healthcare providers consider objective measures as you work together to decide the next steps in your care.

This article will describe King’s staging system, Milano-Torino staging (MiToS) system, and Gold Coast diagnostic criteria for amyotrophic lateral sclerosis. It will also discuss treatment and coping for each stage.

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Neurologist tests a person’s hand reflexes

What Are the Stages of ALS?

The two staging systems used in ALS are the King’s staging system and Milano-Torino staging (MiToS) system. They are both considered useful for assessing results in ALS clinical trials. Each focuses on different aspects of ALS.

The two systems include gradually worsening progression, acknowledging that some aspects of ALS might occur in varied sequences. Each contains progressive stages that end in stage 5 (death).

Even with treatment, ALS stages progress in severity and do not improve.

Complexities of Defining an ALS TimelineALS always worsens and tends to worsen quickly over the course of months, leading to severe disability within one to two years. The order in which different symptoms appear is not always consistent.However, for most people with ALS, all of the symptoms will eventually develop. This makes staging somewhat difficult, and therefore the staging systems take into considerations features such as the number of regions of the body affected, the type of function that is lost, and the level of independence.

Complexities of Defining an ALS Timeline

ALS always worsens and tends to worsen quickly over the course of months, leading to severe disability within one to two years. The order in which different symptoms appear is not always consistent.However, for most people with ALS, all of the symptoms will eventually develop. This makes staging somewhat difficult, and therefore the staging systems take into considerations features such as the number of regions of the body affected, the type of function that is lost, and the level of independence.

ALS always worsens and tends to worsen quickly over the course of months, leading to severe disability within one to two years. The order in which different symptoms appear is not always consistent.

However, for most people with ALS, all of the symptoms will eventually develop. This makes staging somewhat difficult, and therefore the staging systems take into considerations features such as the number of regions of the body affected, the type of function that is lost, and the level of independence.

King’s Staging System

The regions are defined asbulbar(face, mouth, and throat muscles), upper body, lower body, and diaphragm (a large abdominal muscle that controls breathing).

The five stages are:

Milano–Torino Staging System

The Milano–Torino (MiToS) staging system begins at stage 0 and ends at stage 5. The six stages are based on loss of function. This system uses the revised ALS Functional Rating Scale (ALSFRS-R), which includes four key components: fine motor control, leg motor control, speaking and swallowing, and breathing.

The stages are:

Bulbar and Spinal ALSBulbar ALS is described as ALS that begins with symptoms involving the muscles of the face, mouth, and throat. Spinal ALS is described as ALS that begins with symptoms involving the arms, legs, and trunk.Usually, ALS involves both the bulbar and the spinal regions—although the sequence is not always consistent.

Bulbar and Spinal ALS

Bulbar ALS is described as ALS that begins with symptoms involving the muscles of the face, mouth, and throat. Spinal ALS is described as ALS that begins with symptoms involving the arms, legs, and trunk.Usually, ALS involves both the bulbar and the spinal regions—although the sequence is not always consistent.

Bulbar ALS is described as ALS that begins with symptoms involving the muscles of the face, mouth, and throat. Spinal ALS is described as ALS that begins with symptoms involving the arms, legs, and trunk.

Usually, ALS involves both the bulbar and the spinal regions—although the sequence is not always consistent.

Delayed Diagnosis and ALS Progression

A key feature of ALS is that it involves movement and not sensation. Additionally, this condition is one of only a few neurological disorders that cause both upper motor neuron and lower motor neuron involvement. Each of these effects leads to physical weakness.

Upper motor neuron disease involves the brain and spinal cord and causesrigidity,spasms, and brisk motor reflexes. Lower motor neuron disease causes floppy muscles andmuscle atrophy. This combination is unusual in other neurological disorders and is a characteristic of ALS.

The Gold Coast criteria is a diagnostic system that can be used to determine whether you have ALS.Using this system, three components are necessary to indicate ALS.

The Gold Coast criteria are:

Some people arediagnosed with ALSat a relatively late stage, while others may be diagnosed at a fairly early stage, when symptoms first begin. This often has to do with how soon you seek medical attention for your symptoms and the severity of your symptoms.

Treatment Based on ALS Symptoms

The treatment of ALS is based on symptoms, not stages. But having an idea of your disease stage can help you as you consider plans for the next stage in your disease. For example, you might be able to plan for transportation services or home modifications to make it more accessible.

Coping Through Each ALS Stage

The ALS stages are progressive and indicate worsening disease. However, the symptoms do not always occur in a particular order. Overall, late stages are associated with decreased mobility, decreased independence, and worsening ability to eat and breathe independently.

Early Stages

Mid Stages

In the mid stages of ALS, you will need assistance in getting around. This may first involve a walker, and then you might need to use a wheelchair. You will also need to consider factors that can limit your abilities, such as stairs, narrow hallways, and uneven pavement or floors.

Late Stages

In late stages of ALS, eating and independent breathing are severely impaired. Communication also becomes extremely limited. During this stage you will need substantial assistance from caregivers, and you will need to usefeedingandbreathing devices. You may also need to move to a location where you can have 24-hour availability of nursing care.

Caring for Someone With ALS

Summary

The Gold Coast criteria are used to determine whether a person has ALS. Your healthcare providers will use these criteria if you have signs and symptoms of ALS.

After a diagnosis of ALS, two staging systems are commonly used: the King’s staging system and the Milano-Torino staging (MiToS) system.These staging systems can help you, your family, and your healthcare providers consistently communicate with each other to define the severity and progression of your ALS.

Additionally, ALS staging systems are used in clinical trials to help measure the improvement and progression of the disease. You might decide to enroll in a clinical trial if you want to try new treatments for ALS that have not yet been formally approved.

A Word From Verywell

Getting a diagnosis of ALS can be overwhelming. You and your loved ones might want to know about diagnostic criteria and staging so that you can have a better understanding of what defines your condition.

While there are some variations in the exact disease course of ALS, there are general features that most people who have ALS have in common. Knowing your stage and what is expected next can help you plan and prepare for your needs with ALS.

Frequently Asked QuestionsALS progresses quickly. Generally, within a year or two of diagnosis, most people with ALS must use a wheelchair and require substantial assistance with daily functions such as eating.Learn MoreLife Expectancy and Treatment in ALSIf you have ALS, you will know if the disease is progressing because you will lose motor skills and the ability to control your muscles. You may also notice that you are having difficulty speaking, swallowing, and breathing.Sometimes ALS is described as spinal or bulbar based on the location of the initial symptoms. However, most of the time, ALS progresses to involve both the bulbar motor neurons (located in the brain stem) and the spinal motor neurons (located in the spinal cord).Learn MoreSpinal Cord and Motor Neuron Diseases

ALS progresses quickly. Generally, within a year or two of diagnosis, most people with ALS must use a wheelchair and require substantial assistance with daily functions such as eating.Learn MoreLife Expectancy and Treatment in ALS

ALS progresses quickly. Generally, within a year or two of diagnosis, most people with ALS must use a wheelchair and require substantial assistance with daily functions such as eating.

Learn MoreLife Expectancy and Treatment in ALS

If you have ALS, you will know if the disease is progressing because you will lose motor skills and the ability to control your muscles. You may also notice that you are having difficulty speaking, swallowing, and breathing.

Sometimes ALS is described as spinal or bulbar based on the location of the initial symptoms. However, most of the time, ALS progresses to involve both the bulbar motor neurons (located in the brain stem) and the spinal motor neurons (located in the spinal cord).Learn MoreSpinal Cord and Motor Neuron Diseases

Sometimes ALS is described as spinal or bulbar based on the location of the initial symptoms. However, most of the time, ALS progresses to involve both the bulbar motor neurons (located in the brain stem) and the spinal motor neurons (located in the spinal cord).

Learn MoreSpinal Cord and Motor Neuron Diseases

7 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Clinicaltrial.gov.Staging system in amyotrophic lateral sclerosisAl-Chalabi A, Chiò A, Merrill C, Oster G, Bornheimer R, Agnese W, Apple S.Clinical staging in amyotrophic lateral sclerosis: analysis of Edaravone Study 19.J Neurol Neurosurg Psychiatry. 2021;92(2):165-171. doi:10.1136/jnnp-2020-323271Balendra R, Al Khleifat A, Fang T, Al-Chalabi A.A standard operating procedure for King’s ALS clinical staging.Amyotroph Lateral Scler Frontotemporal Degener.2019;20(3-4):159-164. doi:10.1080/21678421.2018.1556696He R, Zheng M, Lian L, Yao X. Milano-TorinoStaging and long-term survival in Chinese patients with amyotrophic lateral sclerosis.Cells.2021;10(5):1220. doi:10.3390/cells10051220Hannaford A, Pavey N, van den Bos M, et al.Diagnostic utility of Gold Coast Criteria in amyotrophic lateral sclerosis.Ann Neurol.2021;89(5):979-986. doi:10.1002/ana.26045Turner MR; UK MND Clinical Studies Group.Diagnosing ALS: the Gold Coast criteria and the role of EMG.Pract Neurol.2022 Jun;22(3):176-178. doi:10.1136/practneurol-2021-003256National Institute of Neurological Disorders and Stroke.Amyotrophic lateral sclerosis (ALS) fact sheet.

7 Sources

Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Clinicaltrial.gov.Staging system in amyotrophic lateral sclerosisAl-Chalabi A, Chiò A, Merrill C, Oster G, Bornheimer R, Agnese W, Apple S.Clinical staging in amyotrophic lateral sclerosis: analysis of Edaravone Study 19.J Neurol Neurosurg Psychiatry. 2021;92(2):165-171. doi:10.1136/jnnp-2020-323271Balendra R, Al Khleifat A, Fang T, Al-Chalabi A.A standard operating procedure for King’s ALS clinical staging.Amyotroph Lateral Scler Frontotemporal Degener.2019;20(3-4):159-164. doi:10.1080/21678421.2018.1556696He R, Zheng M, Lian L, Yao X. Milano-TorinoStaging and long-term survival in Chinese patients with amyotrophic lateral sclerosis.Cells.2021;10(5):1220. doi:10.3390/cells10051220Hannaford A, Pavey N, van den Bos M, et al.Diagnostic utility of Gold Coast Criteria in amyotrophic lateral sclerosis.Ann Neurol.2021;89(5):979-986. doi:10.1002/ana.26045Turner MR; UK MND Clinical Studies Group.Diagnosing ALS: the Gold Coast criteria and the role of EMG.Pract Neurol.2022 Jun;22(3):176-178. doi:10.1136/practneurol-2021-003256National Institute of Neurological Disorders and Stroke.Amyotrophic lateral sclerosis (ALS) fact sheet.

Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.

Clinicaltrial.gov.Staging system in amyotrophic lateral sclerosisAl-Chalabi A, Chiò A, Merrill C, Oster G, Bornheimer R, Agnese W, Apple S.Clinical staging in amyotrophic lateral sclerosis: analysis of Edaravone Study 19.J Neurol Neurosurg Psychiatry. 2021;92(2):165-171. doi:10.1136/jnnp-2020-323271Balendra R, Al Khleifat A, Fang T, Al-Chalabi A.A standard operating procedure for King’s ALS clinical staging.Amyotroph Lateral Scler Frontotemporal Degener.2019;20(3-4):159-164. doi:10.1080/21678421.2018.1556696He R, Zheng M, Lian L, Yao X. Milano-TorinoStaging and long-term survival in Chinese patients with amyotrophic lateral sclerosis.Cells.2021;10(5):1220. doi:10.3390/cells10051220Hannaford A, Pavey N, van den Bos M, et al.Diagnostic utility of Gold Coast Criteria in amyotrophic lateral sclerosis.Ann Neurol.2021;89(5):979-986. doi:10.1002/ana.26045Turner MR; UK MND Clinical Studies Group.Diagnosing ALS: the Gold Coast criteria and the role of EMG.Pract Neurol.2022 Jun;22(3):176-178. doi:10.1136/practneurol-2021-003256National Institute of Neurological Disorders and Stroke.Amyotrophic lateral sclerosis (ALS) fact sheet.

Clinicaltrial.gov.Staging system in amyotrophic lateral sclerosis

Al-Chalabi A, Chiò A, Merrill C, Oster G, Bornheimer R, Agnese W, Apple S.Clinical staging in amyotrophic lateral sclerosis: analysis of Edaravone Study 19.J Neurol Neurosurg Psychiatry. 2021;92(2):165-171. doi:10.1136/jnnp-2020-323271

Balendra R, Al Khleifat A, Fang T, Al-Chalabi A.A standard operating procedure for King’s ALS clinical staging.Amyotroph Lateral Scler Frontotemporal Degener.2019;20(3-4):159-164. doi:10.1080/21678421.2018.1556696

He R, Zheng M, Lian L, Yao X. Milano-TorinoStaging and long-term survival in Chinese patients with amyotrophic lateral sclerosis.Cells.2021;10(5):1220. doi:10.3390/cells10051220

Hannaford A, Pavey N, van den Bos M, et al.Diagnostic utility of Gold Coast Criteria in amyotrophic lateral sclerosis.Ann Neurol.2021;89(5):979-986. doi:10.1002/ana.26045

Turner MR; UK MND Clinical Studies Group.Diagnosing ALS: the Gold Coast criteria and the role of EMG.Pract Neurol.2022 Jun;22(3):176-178. doi:10.1136/practneurol-2021-003256

National Institute of Neurological Disorders and Stroke.Amyotrophic lateral sclerosis (ALS) fact sheet.

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