ALS symptoms and types
ALS symptoms can vary from one person to another.
Early symptoms of ALS can include:
- Dropping things
- Difficulty with simple tasks such as buttoning a shirt or turning a key
- Slurred or “thick” speech
- Muscle cramping, weakening and twitching
Some people with one or more early symptoms may simply think they are experiencing normal signs of aging. However, as the disease progresses, muscles will continue to weaken and it will become more apparent by process of elimination that the cause is ALS.
Symptoms that begin in the arms and legs are called spinal or limb-onset ALS. Symptoms that begin in the muscles used for speaking, swallowing, or breathing are referred to as bulbar onset ALS.
Spinal/limb onset ALS
The most common type of ALS is spinal or limb onset ALS. As the name indicatess, the limbs (arms and legs) are usually the first areas to be affected. Usually, symptoms begin in either the arms or the legs, not both at once.
Symptoms of lower limb onset ALS
Symptoms of lower limb onset ALS include:
- Difficulty running properly
- Foot drop (foot slapping down on the ground when walking, rather than rolling smoothly)
Symptoms of upper limb onset ALS
Symptoms of upper limb onset ALS include:
- Less finger dexterity
- Cramping in the arm or the hand
- Weaker hands
- Stiffness in the arms or hands
Both types of limb onset ALS have other symptoms that indicate whether upper or lower motor neurons are being affected first.
- The upper motor neurons are located in the brain. They tell your lower motor neurons when and how to move your mucles.
- The lower motor neurons are located in the spinal cord. They send signals directly to your muscles to tell them to move, according to the directions of the upper motor neurons.
Signs of lower motor neuron degeneration
Signs of lower motor neuron degeneration include:
- Muscle weakness and atrophy
- Involuntary twitching of muscles (known as fasciculations)
- Muscle cramps
- Weakened reflexes
- Flaccidity (decreased muscle tone)
- Difficulty swallowing
- Inability to articulate speech
- Shortnesss of breath at rest
Signs of upper motor neuron degeneration
Signs of upper motor neuron degeneration include:
- Muscle stiffness or rigidity
- Increased or hyperactive reflexes
- Emotional lability (a decreased ability to control laughing or crying)
Bulbar onset ALS
In about 20 to 25 per cent of ALS cases, the muscles for speaking, swallowing and/or breathing are the first to be affected. This is known as bulbar onset ALS. The term “bulbar” refers to the motor neurons located in the corticobulbar area of the brainstem, which controls muscles of the face, head, and neck. Bulbar ALS often progresses faster than limb onset ALS.
In rare cases, the respiratory muscles are the first to be affected, and the disease may be classified separately as respiratory onset ALS. People with this type of ALS may be referred directly to acute respiratory care instead of to an ALS clinic or a neurologist.
If you have bulbar ALS, talk to your ALS clinic team about dietary changes, communication devices, speech therapy, and medications that can hep maintain your quality of life.
Symptoms of bulbar onset ALS
Symptoms of bulbar onset ALS include:
- Changes in voice and speech
- Harsh, hoarse or strained voice
- Whispery voice (as a result of the vocal cords not folding as they do normally, allowing more air to escape during speech)
- Poor articulation
- Less vocal range
- Quieter voice
- Spasms in muscles of the jaw, face, voice box, throat or tongue
- Involuntary excessive laughing and crying
- Brisk jaw jerks
- Involuntary twitching in muscles of the tongue
- Vocal cord spasms causing the sensation that air cannot be moved in or out
ALS and cognitive changes
Sometimes, ALS is accompanied by cognitive change. Cognitive change means the way a person thinks, knows, perceives, and understands the world around them becomes altered, which may or may not lead to differences in the way the person acts. What this might look like in real life is a person whose personality seems to change.
Almost half of people diagnosed with ALS will experience some level of cognitive change. Most commonly, people with ALS experience only mild to moderate cognitive changes. Mild cognitive changes are often recognized only through neurological testing, whereas moderate changes may be more noticeable.
Mild cognitive changes
Mild cognitive changes include:
- Alterations in planning, reasoning, organizing and managing life functions (or executive functions)
- Increased apathy
- Changes in language (written or oral expression) and comprehension, including decreased verbal fluency and speech impairment
- Inattention or forgetfulness
- Bouts of irritability, apathy and/or anxiety and depression.
Clinically relevant cognitive changes
Approximately 35 per cent of patients with ALS experience clinically relevant cognitive impairment, with an additional 15 per cent having frontotemporal dementia (ALS-FTD). There is greater complexity to the spectrum of cognitive changes detailed within the diagnostic criteria defined by Dr. Mike Strong and team.
Frontotemporal dementia (FTD)
Frontotemporal dementia is a progressive brain disease involving loss of neurons in the frontal and temporal lobes of the brain, which are areas of the brain responsible for rational thought, impulse control, and personality. Unlike Alzheimer’s disease, severe behavioural changes are usually the first signs to develop in FTD rather than memory loss, which may not occur at all.
Both FTD and ALS occurring at once is referred to as ALS frontotemporal spectrum disorder.
Signs and symptoms of frontotemporal dementia
Signs and symptoms of frontotemporal dementia include:
- Recurring sudden mood swings
- Loss of reasoning or problem-solving ability
- Repetitive behaviours
- Socially inappropriate behaviour
- Impulsiveness and loss of inhibitions
- Memory loss
- Balance and/or movement problems
- Problems with speech and/or language
Content adapted from the ALS Guide, which was published in 2019 as a joint project of ALS Societies across Canada.