Primary Lateral Sclerosis (PLS)

Characteristics

Primary Lateral Sclerosis (PLS) is a neuromuscular disorder affecting the central motor neurons. It manifests as stiffness and spasticity caused by increased muscle tone and heightened reflexes.

Prevalence

In the Netherlands, approximately 100 to 200 people are diagnosed with PLS. The disease typically begins between the ages of 35 and 65, though onset can range from 18 to 76 years. PLS occurs equally in men and women and is rarely hereditary.

Disease Progression

PLS progresses relatively slowly. Symptoms usually begin with stiffness and spasticity in both legs, causing patients to stumble, struggle with running, and experience reduced leg strength. In some cases, the disease starts in the muscles responsible for speech (bulbar region) or in the arms.

As the disease advances, weakness may also affect the hands and arms. Speech becomes slower and less articulate due to difficulties with articulation. Over time, chewing and swallowing problems can develop. A spastic bladder often emerges in later stages, leading to frequent urination and potential incontinence.

A small minority of PLS patients may experience cognitive and behavioral changes after prolonged illness. PLS generally does not shorten life expectancy. However, in some cases, the disease may progress to ALS within four years of symptom onset.

Treatment

Neurologists refer patients to specialized multidisciplinary teams experienced in managing PLS, ALS, and PSMA. These teams, usually located in rehabilitation centers, include:

• A rehabilitation physician
• An occupational therapist
• A physical therapist
• A speech therapist
• A dietitian
• A social worker

This comprehensive approach helps address the various symptoms and challenges associated with PLS, improving quality of life and maintaining functionality as much as possible.