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What causes Amyotrophic Lateral Sclerosis (ALS)?

Differentiation Between Primary Lateral Sclerosis and Amyotrophic Lateral Sclerosis: Examination of Symptoms and Signs at Disease Onset and During Follow-up

Objective To determine which clinical features at onset and during follow-up could help differentiate between PLS and ALS. Design Retrospective study comparing patients with a diagnosis of PLS or ALS for differences in symptoms or signs at disease onset and during follow-up. Results At presentation, stiffness was the only symptom that was significantly different between patients with PLS and patients with ALS. During follow-up, limb wasting was rare in patients with PLS. Disease duration was significantly longer in patients with PLS compared with patients with ALS. 

During the 16 years of follow-up, the mortality rate was significantly lower in patients with PLS compared with patients with ALS. Conclusion Our findings suggest that a patient presenting with spasticity who does not develop wasting within 3 years most likely has PLS. Motor neuron diseases comprise a heterogeneous group of disorders that affect the upper motor neuron and/or the lower motor neuron. Although there may be similarities between patients with ALS and patients with PLS, and PLS could be an ALS variant restricted to the UMN, there are obvious differences between these 2 entities. In this study, we have compared patients with PLS with patients with ALS to identify differences in symptoms or signs at disease onset and during follow-up to determine features that differentiate these 2 entities. 

Primary lateral sclerosis was diagnosed in patients only if they had no evidence of acute or chronic denervation on EMG/NCS. We evaluated both symptoms and signs at the time of onset in addition to symptoms and signs at follow-up for significant differences between the 2 patient populations. Survival data were available in 36 patients with PLS and 597 patients with ALS. The mean SD of disease duration was significantly longer in patients with PLS compared with patients with ALS. The mortality rate was also significantly different between the 2 groups, with an overall mortality rate of 33% in the PLS group and 89% in the ALS group. 

Limb wasting was rare in patients with PLS compared with patients with ALS. Bulbar symptoms were significantly less common in patients with PLS than in patients with ALS. Cortical signs were slightly more common in PLS compared with ALS. Clinically overt dementia was more prevalent in patients with ALS than in patients with PLS. Parkinsonism, and cerebellar pyramidal, and sensory occurred very rarely in both patient groups and thus were not significantly different. 

An analysis of presenting symptoms and signs in patients with ALS revealed that those who presented with spasticity had significantly better survival rates than patients who presented with other signs or symptoms. There was no statistically significant difference in disease duration between the 2 groups, though the patients who presented with spasticity had a longer disease duration than those who presented with other signs or symptoms. There was a trend for patients presenting with spasticity to be younger at disease onset than those who presented with other symptoms or signs. Patients with ALS who presented with spasticity had a significantly longer survival than patients who presented with other signs or symptoms. Disease duration could be determined in 83% of patients with PLS and 90% of patients with ALS. 

Given the dramatically different disease duration and rate of mortality between the 2 groups, we do not feel that the patients who were lost to follow-up would have had a great impact on our results. 

Keywords: [“patient”,”PLS”,”ALS”]

Amyotrophic Lateral Sclerosis

Amyotrophic lateral sclerosis is a progressive nervous system disorder. It gradually destroys the nerves responsible for muscle movement. Over time, ALS leads to almost total paralysis of muscle movement, including breathing. Eventually, the disorder leads to respiratory failure. Symptoms of ALS include: Progressive weakness in arms and legs. 

Trouble chewing and swallowing, resulting in frequent choking and gagging. While no cure exists for ALS, we can work with you to reduce or manage symptoms for a time. Physical therapy – To reduce pain associated with muscle cramping and spasticity. Nutritional care – Providing dietary guidelines and help with tube feeding, if needed. UVA’s Richard R. 

Dart ALS Clinic has been recognized as the region’s first Certified Treatment Center of Excellence by the ALS Association. Content was created using EBSCO’s Health Library. This information is not a substitute for professional medical advice. 

Keywords: [“ALS”,”muscle”,”need”]

Department of Neurology |SUNY Upstate Medical University

We provide patients with comprehensive care involving multiple specialties, so that patients can receive many services at one visit. We are the largest ALS Center in New York outside of New York City, and are a referral center for central and western New York State. The Center is one of only a handful of centers that have received certification by both the Muscular Dystrophy Association and the ALS Association. The ALS Clinic at Upstate is part of the ALS Association’s national network of Certified Treatment Centers of Excellence. We provide evidence-based, multidisciplinary ALS care and services in a supportive atmosphere with an emphasis on hope and quality of life. 

The Center is nationally recognized for its research into possible treatments for ALS, and patients are given the opportunity to participate in a variety of clinical trials. 

Keywords: [“Center”,”ALS”,”Association”]