Amyotrophic lateral sclerosis 1

Symptoms

What are the main symptoms of Amyotrophic Lateral Sclerosis?

Most people with amyotrophic lateral sclerosis (ALS) initially notice weakness in their hands and legs, slurred or unclear speech, trouble swallowing or chewing and muscle problems (twitching, cramping, stiffness or weakness). Late symptoms of amyotrophic lateral sclerosis (ALS) include generalized muscle weakness and atrophy (muscle wasting), increasing problems with moving, swallowing, and speaking, exaggerated reflexes (hyperreflexia), and an overactive gag reflex. People with ALS eventually lose the ability to stand/walk, get in/out of bed on their own, and use their hands/arms. A small percentage of people may have trouble with memory or decision making. About 5% of individuals with ALS, regardless of family history, have frontotemporal dementia (FTD). The average duration of disease is three years, but it can vary significantly. Most people with ALS die because the muscles that control their breathing are compromised.

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Are there differences in signs and symptoms between sporadic and familial Amyotrophic Lateral Sclerosis?

Are there any areas of the body that are not affected by Amyotrophic Lateral Sclerosis?

Are there earlier onset, later onset, or variant forms of Amyotrophic Lateral Sclerosis?

What health problems should I look for in Amyotrophic Lateral Sclerosis?

Any other diseases that look a lot like Amyotrophic Lateral Sclerosis?

Is there one or two characteristic “odd” or “unusual” symptom or clinical feature of Amyotrophic Lateral Sclerosis?

Is there variable expression or incomplete penetrance in amyotrophic lateral sclerosis?

Do males and females with amyotrophic lateral sclerosis show different symptoms?

Are there differences in signs and symptoms between sporadic and familial Amyotrophic Lateral Sclerosis?

The signs and symptoms of sporadic and familial amyotrophic lateral sclerosis (ALS) are the same. However, people with familial, or inherited, ALS often have signs and symptoms at an earlier age than people with sporadic ALS. The mean age of onset is 56 years in individuals with no known family history (sporadic ALS) and 46 years in individuals with more than one affected family member (familial ALS or FALS).

Most people with amyotrophic lateral sclerosis (ALS) initially notice weakness in their hands and legs, slurred or unclear speech, trouble swallowing or chewing and muscle problems (twitching, cramping, stiffness or weakness). Late symptoms of amyotrophic lateral sclerosis (ALS) include generalized muscle weakness and atrophy (muscle wasting), increasing problems with moving, swallowing, and speaking, exaggerated reflexes (hyperreflexia), and an overactive gag reflex. People with ALS eventually lose the ability to stand/walk, get in/out of bed on their own, and use their hands/arms. A small percentage of people may have trouble with memory or decision making. About 5% of individuals with ALS, regardless of family history, have frontotemporal dementia (FTD). The average duration of disease is three years, but it can vary significantly. Most people with ALS die because the muscles that control their breathing are compromised.

References
Are there any areas of the body that are not affected by Amyotrophic Lateral Sclerosis?

Amyotrophic lateral sclerosis usually does not affect bowel or bladder control, senses, mood, and eye movement.

Are there earlier onset, later onset, or variant forms of Amyotrophic Lateral Sclerosis?

Amyotrophic lateral sclerosis (ALS) can be seen as a juvenile-onset disorder. Juvenile-onset ALS is a term that is given to individuals with ALS who are diagnosed before age 25 and generally have a family history. Individuals who have variants in the SETX gene have a slowly progressive disease with onset in adolescence.

About 5% of individuals with ALS, regardless of family history, also have have frontotemporal dementia (FTD). Features of FTD include cognitive deficits in attention, abstraction, planning, and problem solving. Individuals with variants in the C9orf72 and CHCHD10 genes have been reported with ALS/FTD.

References
What health problems should I look for in Amyotrophic Lateral Sclerosis?

Most people with amyotrophic lateral sclerosis (ALS) initially notice weakness in their hands and legs, slurred or unclear speech, trouble swallowing or chewing and muscle problems (twitching, cramping, stiffness or weakness). Late symptoms of amyotrophic lateral sclerosis (ALS) include generalized muscle weakness and atrophy (muscle wasting), increasing problems with moving, swallowing, and speaking, exaggerated reflexes (hyperreflexia), and an overactive gag reflex. People with ALS eventually lose the ability to stand/walk, get in/out of bed on their own, and use their hands/arms. A small percentage of people may have trouble with memory or decision making. About 5% of individuals with ALS, regardless of family history, have frontotemporal dementia (FTD). The average duration of disease is three years, but it can vary significantly. Most people with ALS die because the muscles that control their breathing are compromised. It is important to see specialists when you have ALS.

Any other diseases that look a lot like Amyotrophic Lateral Sclerosis?

Many neurodegenerative disorders seem similar in their earlier stages. Studies that can rule out conditions that are similar to amyotrophic lateral sclerosis (ALS) other similar conditions include imaging of brain and/or spinal cord (MRI), blood studies (e.g. CBC, serum concentration of vitamin B12, lead, and TSH), studies of the cerebral spinal fluid (the fluid in the spinal cord that surrounds the nerves) to rule out infection or multiple sclerosis and muscle and/or nerve biopsy.

There are specific hereditary and acquired disorders that are similar to ALS.

Hereditary disorders include:

Spinal and bulbar muscular atrophy (SBMA, Kennedy disease)

ALS8 (also known as SMAIV or Finkel type SMA)

Distal hereditary motor neuronopathy type VIIB

Primary lateral sclerosis (PLS)

Hereditary spastic paraplegia (HSP)

Hexosaminadase A deficiency

Adult polyglucosan body disease

BSCL2-related neurologic disorders

Acquired disorders include:

cervical spine disease

brain stem or spinal cord tumors

thyroid disorders

lead poisoning

vitamin B12 deficiency

multiple sclerosis

paraneoplastic syndrome with occult cancer

motor neuropathies

myasthenia gravis

myasthenic syndrome

inclusion body myositis

Is there one or two characteristic “odd” or “unusual” symptom or clinical feature of Amyotrophic Lateral Sclerosis?

Muscle disorders that affect the lower motor neurons usually present with fasciculation (muscle twitching), atrophy/weakness, and hyporeflexia (decreased reflexes). Muscle disorders that affect the upper motor neurons usually present with spasticity, weakness, and hyperreflexia (exaggerated reflexes). Amyotrophic lateral sclerosis is unique in that it involves the lower motor neurons and presents with hyperreflexia. Patients with amyotrophic lateral sclerosis are also still able to move their eyes.

References
Is there variable expression or incomplete penetrance in amyotrophic lateral sclerosis?

If a genetic condition has reduced penetrance, not everyone that has a significant change in these genes will develop the disease. If a genetic condition has variable expression, different individuals may develop different symptoms at different ages of onset, even if they are part of the same family or have the same genetic change. Many familial forms of amyotrophic lateral sclerosis (ALS) have high penetrance close to 100%. Patients with ALS can have different ages of onset and symptoms may progress at different rates and in somewhat different orders. However, regardless of initial symptoms, muscle breakdown and weakness will affect other muscles eventually.

References
Do males and females with amyotrophic lateral sclerosis show different symptoms?

Males with X-linked amyotrophic lateral sclerosis (ALS), most often due to variants in the UBQLN2 gene have a much earlier age of onset and more rapid progression than females with X-linked ALS.

References

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