Management of Primary Lateral Sclerosis

Abstract

Primary lateral sclerosis is a progressive neurodegenerative process that primarily affects upper motor neuron and presents with progressive stiffness and spasticity in all muscle groups. Given its rarity, diagnosis can be difficult and there has been limited research into what the best treatment is. This review will work to summarize the current available knowledge on the management of primary lateral sclerosis (PLS) including medications, nonpharmacologic interventions, diet, and exercise. There have been no controlled studies of therapies in PLS. Management decisions are based on expert opinion from trials of other motor neuron diseases. The disease, while not curable, is treatable. A treatment plan will involve a mixture of medications, nonpharmacologic interventions, and exercise. The plan should be specific for each individual’s disabilities and not a one size fits all plan. Multidisciplinary centers with specialists trained in PLS can help to develop a plan. Research into the best practices and new treatments can have the potential to improve function. While there is no cure for PLS, there are treatments for PLS. Therapy should involve an individualized care plan involving a mixture of treatment options developed and maintained through a multidisciplinary service and science hub model.