Management of Primary Lateral Sclerosis
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Neuromuscular Disorders (C Fournier, Section Editor)
Management of Primary Lateral Sclerosis Cuiping Zhao, MD1 Dale J. Lange, MD2 James P. Wymer, MD, PhD3,* Address 1 Department of Neurology, Qilu Hospital (Qingdao), Cheeloo College of Medicine, Shandong University, Jinan, China 2 Department of Neurology, Hospital for Special Surgery, New York, NY, USA *,3 Department of Neurology, University of Florida, 1149 Newell Drive, Gainesville, FL, 32610, USA Email: [email protected]
* Springer Science+Business Media, LLC, part of Springer Nature 2020
This article is part of the Topical Collection on Neuromuscular Disorders Keywords Primary lateral sclerosis I Spasticity I Pseudobulbar affect I Sialorrhea I Biomarkers
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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Curr Treat Options Neurol
(2020) 22:31
Introduction Primary lateral sclerosis (PLS) is a rare slowly progressive neurodegenerative condition marked by progressive spasticity, weakness, and bulbar dysfunction without evidence for significant lower motor neuron disease [1–4]. This condition comprises less than 4% of the patients diagnosed with motor neuron disease [4]. All muscle groups can be affected and the pattern is variable. There is no typical patient with PLS. The decline is slow and progressive with involvement of the arms, legs, speaking, and/or swallowing. Death is typically from bulbar dysfunction and nutrition and/or breathing decline. A recent consensus statement on diagnostic criteria for the disease has been developed and defines PLS to be possible if there is at least 2 years of progressive signs and symptoms of upper motor neuron dysfunction in the absence of lower motor neuron dysfunction, sensory symptoms, and an alte
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