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Management of Primary Lateral Sclerosis

  • Neuromuscular Disorders (C Fournier, Section Editor)
  • Published:
Current Treatment Options in Neurology Aims and scope Submit manuscript

Abstract

Purpose of review

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.

Recent findings

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.

Summary

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.

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Authors and Affiliations

  1. Department of Neurology, Qilu Hospital (Qingdao), Cheeloo College of Medicine, Shandong University, Jinan, China

    Cuiping Zhao MD

  2. Department of Neurology, Hospital for Special Surgery, New York, NY, USA

    Dale J. Lange MD

  3. Department of Neurology, University of Florida, 1149 Newell Drive, Gainesville, FL, 32610, USA

    James P. Wymer MD, PhD

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  1. Cuiping Zhao MD
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  2. Dale J. Lange MD
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  3. James P. Wymer MD, PhD
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Correspondence to James P. Wymer MD, PhD.

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Conflict of Interest

JPW and DJL report support from Acorda pharmaceuticals with study medication only for a trial of dalfampridine in primary lateral sclerosis. No other potential conflicts of interest relevant to this article are reported.

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Zhao, C., Lange, D.J. & Wymer, J.P. Management of Primary Lateral Sclerosis. Curr Treat Options Neurol 22, 31 (2020). https://doi.org/10.1007/s11940-020-00640-6

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