Muscle Twitches: Is It a Sign of ALS? - Rare Disease

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Muscle twitching


Hello Dr.
Chen: I experienced muscle twitching between April and May of last year.
It started in the web of my hand, and then I noticed that muscles all over my body would twitch intermittently and at various locations.
This has been going on for almost a year, and I am very concerned that it might be ALS.
I have searched online for your published papers related to ALS and learned that muscle twitching is one of the early symptoms of ALS.
However, muscle twitching does not necessarily mean ALS, so I have a few questions that I hope you can help clarify:
1.
Is muscle twitching in ALS always caused by denervated muscle? If so, would we also see atrophy in the twitching muscles? (I have not noticed any atrophy or weakness in the twitching muscles.)

2.
I have noticed indentations on the edges of my tongue, which are not serrated, and sometimes I find it more difficult to pronounce words that start with the sounds "d," "t," and "n," although at other times my speech is completely normal.
Could you please explain the specific symptoms of bulbar onset?
3.
I have previously undergone an EEG, which was normal.
Is it possible for the EEG to be normal in ALS?
Thank you for your assistance, Dr.
Chen.

cck, 20~29 year old female. Ask Date: 2005/02/07

Dr. Chen Shunsheng reply Rare Disease


Your questions are answered as follows:
1.
Is muscle twitching in ALS necessarily caused by dead muscle? If so, will the twitching muscles also show atrophy? (I have not noticed atrophy or weakness in the twitching muscles.)
Answer: Typically, muscle atrophy should be present.
2.
I see indentations on the edges of my tongue, which are not serrated, and sometimes I find it difficult to pronounce words starting with the sounds "d," "t," and "n," but at other times, my speech is completely normal.
I would like to ask Dr.
Chen about the specific symptoms of bulbar onset.

Answer: Weakness and atrophy of the tongue muscles with twitching.
3.
I previously had an EEG that was normal.
Is it possible for the EEG to be normal in ALS?
Answer: The EEG in ALS is typically normal.
For ALS diagnosis reference, you still need to consult a physician for confirmation of the diagnosis:
Table 1: Diagnostic Criteria for ALS
The diagnosis of ALS must include:
1.
Lower Motor Neuron (LMN) signs (including normal muscle appearance on electromyography)
2.
Upper Motor Neuron (UMN) signs
3.
Symptoms that are progressive
* LMN: Lower Motor Neuron
* UMN: Upper Motor Neuron
Table 2: Subclassification of Diagnostic Criteria
Definite ALS: UMN + LMN signs found in three regions, typical Charcot type ALS.
Probable ALS: UMN + LMN signs found in two regions, with UMN signs predominating over LMN signs.
Possible ALS: UMN + LMN signs found in one region or UMN signs appearing in two or three regions, such as limb ALS, progressive bulbar palsy, and suspected primary lateral sclerosis.
Suspected ALS: LMN signs appearing in two or three regions along with progressive muscle atrophy and other motor syndromes (see Table 3).
* Definition of regions: brainstem (glossopharyngeal), upper limbs, thorax and trunk, lower limbs.
Table 3: Clinical Manifestations to Exclude or Support ALS Diagnosis
A.
The diagnosis of ALS must not include the following clinical manifestations:
1.
Sensory signs
2.
Sphincter dysfunction
3.
Visual disturbances
4.
Autonomic dysfunction
5.
Parkinson's disease
6.
Alzheimer-type dementia
7.
Certain "mimicking" syndromes (see Table 4).
Table 4: Syndromes Similar to ALS
1.
Monoclonal gammopathy with motor neuron disease
2.
Other immune-mediated LMN syndromes
3.
Non-tumor endocrine syndromes
4.
Lymphoma
5.
Acute infections
6.
Post-infectious syndromes
7.
Genetic enzyme deficiencies
8.
Exogenous toxin-induced diseases
9.
Physical trauma
10.
Vascular diseases
11.
Spinal cord diseases
12.
Radiation-induced neurological abnormalities
13.
Creutzfeldt-Jakob disease and other conditions.

Reply Date: 2005/02/07

More Info


Muscle twitches, also known as fasciculations, can be a source of concern for many individuals, especially when they occur frequently or in various parts of the body. Your inquiry about whether these twitches could be a sign of Amyotrophic Lateral Sclerosis (ALS) is a valid one, given the complexity of the disease and its symptoms.
Firstly, it is essential to understand that while muscle twitches can be associated with ALS, they are not exclusive to this condition. Fasciculations can occur in healthy individuals and can be caused by a variety of factors, including stress, fatigue, caffeine intake, and electrolyte imbalances. In ALS, muscle twitches are typically a result of lower motor neuron (LMN) degeneration, which leads to muscle denervation and subsequent muscle atrophy. However, not all muscle twitches indicate that there is muscle death or atrophy present.
To address your specific questions:
1. Are muscle twitches in ALS always associated with dead muscle? In ALS, muscle twitches are often accompanied by muscle weakness and atrophy, but this is not always the case at the onset. It is possible to experience fasciculations without noticeable muscle weakness or atrophy initially. The absence of muscle wasting or weakness in your case makes it less likely that ALS is the cause, especially since ALS typically presents with both LMN and upper motor neuron (UMN) signs.

2. Bulbar onset symptoms: Bulbar onset ALS affects the muscles responsible for speech and swallowing. Symptoms may include slurred speech, difficulty swallowing, and changes in voice. The presence of bite marks on the tongue and difficulty pronouncing certain sounds can indicate bulbar involvement, but these symptoms must be evaluated in conjunction with other clinical signs. If you are experiencing difficulty with specific sounds intermittently, it may not necessarily indicate bulbar onset ALS, especially if other symptoms are absent.

3. Electroencephalogram (EEG) results: An EEG measures electrical activity in the brain and is not typically used to diagnose ALS. In fact, EEG results are often normal in individuals with ALS. The diagnosis of ALS is primarily based on clinical examination and specific tests such as electromyography (EMG) and nerve conduction studies, which assess the electrical activity of muscles and the integrity of motor neurons.

In summary, while muscle twitches can be a symptom of ALS, they are not definitive on their own. The absence of muscle weakness or atrophy, normal EEG results, and the presence of other symptoms are crucial factors in determining the underlying cause of your symptoms. It is advisable to consult a neurologist who specializes in neuromuscular disorders for a thorough evaluation. They may recommend additional tests, such as an EMG, to assess muscle and nerve function more accurately.

In conclusion, while your concerns are understandable, it is essential to approach the situation with a comprehensive understanding of the symptoms and their potential causes. Many individuals experience muscle twitches without any serious underlying condition, and a thorough medical evaluation can provide clarity and peace of mind.

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