From the Guidelines
Early symptoms of ALS (Amyotrophic Lateral Sclerosis) typically begin subtly and progress gradually, with the most common initial signs including muscle weakness, muscle twitching, cramping, or stiffness, especially in the hands, arms, or legs, as well as slurred speech or difficulty swallowing (dysphagia) 1, 2, 3, 4.
Common Initial Symptoms
- Muscle weakness, particularly in the limbs, which may manifest as difficulty with fine motor tasks like buttoning shirts or turning keys
- Muscle twitching (fasciculations), cramping, or stiffness, especially in the hands, arms, or legs
- Slurred speech or difficulty swallowing (dysphagia)
- Fatigue, tripping, or dropping things frequently ### Disease Progression The disease typically affects one region before spreading to others, so asymmetrical weakness is common. Importantly, ALS symptoms vary significantly between individuals, with some experiencing primarily upper motor neuron symptoms (spasticity, hyperreflexia) while others show lower motor neuron signs (weakness, atrophy, fasciculations) 2, 4.
Importance of Early Diagnosis
Early diagnosis is crucial for management, though challenging due to symptom overlap with other conditions. If these symptoms appear, especially in combination or with progressive worsening, immediate medical evaluation is essential as early intervention with medications like riluzole or edaravone may help slow disease progression 1, 4.
Diagnostic Evaluation
Clinical assessment of dysphagia should include evaluation of lip closure and evidence of saliva pooling, tongue strength, mobility and tone, chewing capacity, palatal movement in response to tactile stimulation, the quality and strength of the cough as well as phoniatric function 1. Instrumental assessment of dysphagia, such as videofluoroscopy (VFS) or fiberoptic endoscopic evaluation of swallowing (FEES), may also be performed according to local protocols, in patients with symptoms of dysphagia 1, 3.
From the Research
Early ALS Symptoms
- The early symptoms of Amyotrophic Lateral Sclerosis (ALS) are not explicitly stated in the provided studies, but it can be inferred that the disease is characterized by progressive muscle weakness, amyotrophy, fasciculations, and signs of corticospinal tract deficits 5.
- ALS often has a focal onset but subsequently spreads to different body regions, where failure of respiratory muscles typically limits survival to 2-5 years after disease onset 6.
- The disease can also have extra-motor manifestations such as changes in behavior, executive dysfunction, and language problems, which can be severe enough to meet the clinical criteria of frontotemporal dementia (FTD) in up to 50% of cases 6.
Disease Progression
- ALS is a progressive, neurodegenerative, and inevitably fatal disease with no cure, and life expectancy is typically 2-5 years after symptom onset 7.
- The disease is considered "treatable" and rehabilitation is integral to optimal, comprehensive care, with physical therapy playing a critical role in the overall management of individuals with ALS 7.
- Riluzole, a benzothiazole derivative, is the only approved treatment for ALS, which blocks glutamatergic neurotransmission in the CNS and has a modest effect on prolonging tracheostomy-free survival by only 2-3 months 8.
Treatment and Management
- Multidisciplinary care, including nutritional and respiratory support and symptom management, remains the cornerstone of treatment for ALS 6.
- Riluzole has been used as the only approved treatment for ALS since 1995, but its mechanism of action in slowing the progression of the disease remains obscure 9.
- Physical therapy tailored to the individual's needs and goals can enable people with ALS to live their lives to the fullest and with quality 7.