What Are the Stages of ALS?

There are a few staging systems

Medically reviewed by Smita Patel, DO

After being diagnosed with amyotrophic lateral sclerosis (ALS), the stage may be determined by two staging systems. The stages describe the progression of the condition. In ALS, weakness gradually worsens as the motor neurons in the spinal cord degenerate.

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.

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 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.

King’s Staging System

King’s staging system includes five disease stages that define the physical effects of ALS. This system includes a count of central nervous system (CNS) regions affected by weakness, atrophy (loss or shrinkage), spasticity (abnormal increase in muscle tone or stiffness), dysphagia (difficulty swallowing), or dysarthria (difficulty speaking).

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

The five stages are:

• Stage 1: One CNS area involved

• Stage 2: Two CNS areas invoked

• Stage 3: Three CNS areas involved

• Stage 4: Nutritional or respiratory failure that requires a feeding tube or artificial respiration

• Stage 5: Death

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:

• Stage 0: No functional impairment

• Stage 1: Loss of one type of function

• Stage 2: Loss of two types of function

• Stage 3: Loss of three types of function

• Stage 4: Loss of four types of function

• Stage 5: Death

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.

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 causes rigidity, spasms, and brisk motor reflexes. Lower motor neuron disease causes floppy muscles and muscle 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:

• A baseline of normal motor function and progressively worsening motor function symptoms and physical examination

• The presence of upper motor neuron signs and lower motor neuron signs in the same area of the body or lower motor neuron signs in two areas of the body

• A ruling out of other causes of the symptoms and physical examination changes

Some people are diagnosed with ALS at 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.

At What Stage Is ALS Medication Recommended?

The four medications approved by the Food and Drug Administration (FDA) for the treatment of ALS are Rilutek (riluzole), Radicava (edaravone), Relvyrio (sodium phenylbutyrate/taururosdiol), and Qalsody (tofersen). Each of these medications is approved for any stage of ALS. They are expected to have the most benefit when they are started as soon as the condition is diagnosed.

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

At the very early stages of ALS, your main focus for coping would consist of getting started on medical treatment that is available to you. You may also need some accommodations so that you can safely get around. But the early stages are characterized by relatively preserved mobility and independence.

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 use feeding and breathing devices. You may also need to move to a location where you can have 24-hour availability of nursing care.

Related: 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 Questions

How quickly does ALS progress?

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.

Can you tell when ALS progresses?

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.

Are there different types of ALS?

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).

Read the original article on Verywell Health.