Materiały źródłowe

• #1 Primary lateral sclerosis (PLS) – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-lateral-sclerosis/diagnosis-treatment/drc-20353972

  There are no treatments to prevent, stop or reverse primary lateral sclerosis. Treatment focuses on relieving symptoms and preserving function. […] You might take medicine to relieve these symptoms: […] Muscle spasms may be relieved by baclofen (Fleqsuvy, Ozobax, Lyvispah), tizanidine (Zanaflex) or clonazepam (Klonopin). These medicines are taken by mouth. […] If muscle spasms aren’t controlled with oral medicine, your health care professional might recommend surgery to implant a medicine pump. The pump delivers baclofen directly to the spinal fluid. […] Stretching and strengthening exercises can help maintain muscle strength, flexibility and range of motion. The exercises also may help prevent joint immobility. […] Speech therapy might help with communication and swallowing if your facial muscles are affected by PLS.

• #1 Primary lateral sclerosis (PLS) – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-lateral-sclerosis/diagnosis-treatment/drc-20353972

  If you have trouble chewing and swallowing, a dietitian can offer diet tips, nutritional supplements or special feeding methods. This can help you maintain your body weight when symptoms make it hard to eat. […] As PLS symptoms get worse, you may need an assistive device. Physical or occupational therapists may evaluate you regularly to determine whether you need a brace, cane, walker or wheelchair. Assistive technology devices also may help with communication.

• #2 Primary Lateral Sclerosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK609096/

  Currently, no cure or disease-modifying treatment for PLS has been identified. Treatment is centered on symptom management and improving the quality of life in patients with PLS. Given the numerous symptoms seen in PLS, an interprofessional approach should be taken to improve mobility, reduce spasticity, and facilitate activities of daily living. Physical and occupational therapy are vital in improving gait and balance and can help determine the need for additional durable medical equipment. Speech therapy can be helpful in those with bulbar symptoms who have dysphagia or speech difficulties. Spasticity is often the most disabling symptom, and first-line oral agents include baclofen, benzodiazepines, and tizanidine. Patients who have sedating adverse events from these agents but have benefits may trial intrathecal baclofen and subsequent baclofen pump placement. Botulinum toxin type A can also be used to treat spasticity. […] No cure or disease-modifying therapy for PLS has been established; treatment is supportive and centered around improving functional mobility and quality of life.

• #3 Clinical Care and Therapeutic Trials in PLS

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7899088/

  PLS is an extremely rare central nervous system degenerative disorder characterized by slowly progressive upper motor neuron loss leading to severe limb and bulbar dysfunction and disability. […] improved symptomatic treatments are a major unmet need, especially for the often refractory spasticity that is a core feature of the syndrome. […] Treatment in PLS is aimed at minimizing symptoms and maximizing quality of life. […] The ideal approach should be individualized for each patient with PLS to reach their goals for maintaining mobility, communication, and independence. […] The ideal treatment regimen includes medications, physical activity, psychosocial support, and when needed, durable equipment. […] Because a diagnosis of PLS often cannot be made without the passage of time, those patients must exist in a period of diagnostic uncertainty.

• #3 Clinical Care and Therapeutic Trials in PLS

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7899088/

  A person-centered approach to treating spasticity takes into account the individuals goals of maintaining mobility and reducing discomfort from muscle stiffness. […] Medications are useful for reducing symptoms of spasticity. […] Patients need better options, pharmacologic and nonpharmacologic, to improve mobility by reducing spasticity. […] Currently, one symptomatic clinical trial for spasticity in patients with PLS is on-going. […] Dalfampridine is hypothesized to enhance neuronal transmission in damaged axons. […] Most therapies currently employed to ameliorate symptoms for patients with PLS are extrapolated from evidence and experience from treating patients with ALS and other diseases with UMN dysfunction, such as multiple sclerosis. […] There is sufficient scientific rationale to support clinical trials using activity-based and non-invasive stimulation interventions in PLS to promote neuroplasticity.

• #3 Clinical Care and Therapeutic Trials in PLS

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7899088/

  Better treatment for spasticity would greatly improve quality of life for those living with PLS. […] At the same time, there is an urgent need to develop clinical trials that test disease-modifying treatments. […] The recently established new diagnostic criteria that allow us to capture early PLS cases and the development of the PLSFRS to assess patients disease progression more sensitively than the ALSFRS-R represent important steps towards clinical trial readiness in PLS.

• #4 Primary Lateral Sclerosis Treatment & Management: Approach Considerations, Treatments for Spasticity, Physical Therapy, Assistive Devices, and Ventilatory Support

  https://emedicine.medscape.com/article/1171782-treatment

  Patients late in the course of primary lateral sclerosis (PLS) may develop ventilatory failure and may require noninvasive ventilatory support. […] Patients with primary lateral sclerosis (PLS) may benefit from evaluation and follow-up at multidisciplinary clinics, such as those available for the more common ALS. These multidisciplinary clinics may provide, in a single location, physical and occupational therapy, speech and swallowing evaluation and therapy, nutritional assessment and counseling, and respiratory assessment. […] A balanced diet based on the patient’s physical activity and other needs is recommended to avoid excessive weight gain or inanition. […] Frequency of outpatient follow-up in patients with primary lateral sclerosis (PLS) depends on the patient’s need for symptom control. It may range from monthly initially to every 4-6 months once optimal treatment is established, provided that no new symptoms appear.

• #4 Primary Lateral Sclerosis Treatment & Management: Approach Considerations, Treatments for Spasticity, Physical Therapy, Assistive Devices, and Ventilatory Support

  https://emedicine.medscape.com/article/1171782-treatment

  Mechanism-specific treatments directed at the pathologic process that underlies primary lateral sclerosis (PLS) have not been identified. Consequently, treatments are directed at alleviating symptoms. […] Treatments for spasticity include baclofen (Lioresal), tizanidine (Zanaflex), and the benzodiazepines, such as diazepam (Valium) and clonazepam (Klonopin). Patients in whom oral treatment does not provide adequate relief may wish to consider intrathecal baclofen (ie, infusion of medication directly into the CSF via a surgically placed continuous infusion pump). However, patients must be selected appropriately to ensure that those who receive this treatment are likely to benefit. […] Stretching exercises, usually used in combination with pharmacologic treatment, may help to alleviate spasticity. A program of stretching/strengthening exercises, which may be done at home, may promote full range of joint motion and reduce the risk of contractures.

• #5 Primary Lateral Sclerosis: An Overview

  https://www.mdpi.com/2077-0383/13/2/578

  For spasticity, which is the most disabling symptom, the first-line oral agents include baclofen, tizanidine, benzodiazepines (e.g., clonazepam and diazepam), gabapentin, pregabalin, and dantrolene. These drugs can cause some side-effects, most commonly somnolence and the worsening of hyposthenia. For patients who achieve some benefit with anti-spasticity drugs, but are limited by these side-effects, a trial of intrathecal baclofen—and subsequent baclofen pump placement—may be useful. In recent years, cannabinoids have been increasingly used to treat spasticity with significant improvement and without serious adverse events, and delta-9-tetrahydrocannabinol and cannabidiol (THC/CBD: 50:50) spray (nabixomols) had a positive effect on spasticity symptoms in a placebo-controlled randomized phase 2 trial in patients with motor neuron disease. Treatment with botulinum toxin type A associated with physiotherapy has also proven beneficial in the short term and long term in patients with moderate-to-severe spasticity. Dalfampridine (4-aminopyridine) has also been shown to be effective in spasticity. Furthermore, there are data to support the effectiveness of some non-pharmacological therapies such as transcutaneous electrical nerve stimulation and acupuncture on spasticity, although more controlled studies are needed.

• #5 Primary Lateral Sclerosis: An Overview

  https://www.mdpi.com/2077-0383/13/2/578

  The management of excess oral secretions or drooling is similar to that used in ALS. Most patients have beneficial effects with oral anticholinergic medications—amitriptyline, scopolamine, glycopyrrolate, or atropine drops. For drooling unresponsive to oral therapies, botulism toxin injections into submandibular glands may be beneficial. […] For pseudobulbar affect, selective serotonin reuptake inhibitors can help patients with emotionality even in the absence of depression. Furthermore, the combination of dextromethorphan and quinidine (Neudexta) may prove beneficial. Tricyclic antidepressants may be useful in patients without significant beneficial effects from Neudexta. Urinary urgency can be relieved by drugs such as oxybutynin.

• #5 Primary Lateral Sclerosis: An Overview

  https://www.mdpi.com/2077-0383/13/2/578

  Currently, there are no disease-modifying treatments approved or tested in experimental trials for PLS, probably due to the rarity of the disease and lack of significant understanding of its underlying pathophysiology. Therefore, the approach to treatment remains essentially targeted to alleviate the symptoms and improve the quality of life of patients. Since clinical trials of symptomatic treatments are limited, clinical experience based on other neurological disorders with similar symptoms has been used to guide treatments. A multidisciplinary approach should be preferred in order to manage several disturbances that can be present along the disease course, such as spasticity, fatigue, pseudobulbar palsy, pain, depression, and sphincteric and sexual dysfunctions. Non-medication approaches including physical and occupational treatment are essential for gait and balance training, the reduction of the discomfort from muscle stiffness, and evaluation for assistive devices, along with speech therapy, pneumological assessment, and psychosocial support. Experimental data on physiotherapy in patients with PLS are lacking, but a strictly monitored exercise program has been proposed to potentially reduce motor deterioration in patients with ALS. Preliminary observations suggest that combining robot-aided and conventional rehabilitation could be a promising approach to mitigate the PLS disability burden.

• #6 Primary Lateral Sclerosis (PLS): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17986-primary-lateral-sclerosis

  Because theres no cure for PLS, treatment focuses on managing your symptoms and providing mobility support like using a cane or a walker. […] Treatment for PLS helps you manage your symptoms and could include: […] Medications to reduce muscle stiffness, spasms and difficulties swallowing. […] Physical therapy to decrease muscle weakness, and improve muscle flexibility and joint range of motion. […] Devices to assist with independence and mobility, like a cane, walker or wheelchair. […] Speech therapy or devices to assist with speaking. […] Common medications to treat symptoms of PLS include: […] Baclofen and tizanidine for muscle stiffness. […] Quinine for muscle cramps. […] Diazepam to relax your muscles. […] Each medication may come with side effects that vary based on the type. Talk to your healthcare provider before starting a medication to learn more about the side effects. […] Medications are effective in decreasing your symptoms and helping you go about your day without interruptions. For your safety, you may want to consider using a cane, walker or wheelchair to give you more independence and confidence when you move.

• #7 Primary lateral sclerosis – Wikipedia

  https://en.wikipedia.org/wiki/Primary_lateral_sclerosis

  Treatment for individuals with PLS is symptomatic. Baclofen and tizanidine may reduce spasticity. Quinine or phenytoin may decrease cramps. Some patients who do not receive adequate relief from oral treatment may consider intrathecal baclofen (i.e., infusion of medication directly into the cerebrospinal fluid via a surgically placed continuous infusion pump). However, patients are carefully selected for this type of procedure to ensure that they will likely benefit from this invasive procedure. […] Physical therapy often helps prevent joint immobility. Speech therapy may be useful for those with involvement of the facial muscles. Physiotherapy treatment focuses on reducing muscle tone, maintaining or improving range of motion, increasing strength and coordination, and improving functional mobility. In PLS, stretching is thought to improve flexibility and can also reduce muscle spasticity and cramps.

• #8 Decreased spasticity in primary lateral sclerosis after botulinum toxin injection: A case report | Neurología (English Edition)

  https://www.elsevier.es/en-revista-neurologia-english-edition–495-articulo-decreased-spasticity-in-primary-lateral-S2173580818300014

  Decreased spasticity in primary lateral sclerosis after botulinum toxin injection: A case report […] Treating spasticity can have a meaningful impact on patients quality of life, although few studies have addressed this topic. […] We began treatment with Lioresal in combination with stretches in the therapeutic gymnasium and hydrotherapy. […] We therefore proposed attempting to control spasticity with local BTX injections. […] One month later, there was a significant improvement in the patient’s spasticity, resulting also in a functional improvement. […] Numerous studies have demonstrated the effectiveness and safety of BTX in treating sialorrhoea in patients with ALS, with no cases describing a global worsening of the disease. […] In the light of the above, and given our patient’s poor response to medical treatment and physiotherapy, we decided to begin treatment with local injections of BTX, aiming to achieve functional results. […] The improvement in such an important function as gait, and being able to continue partaking in social activities, have afforded our patient a significantly higher quality of life, despite the progression of his condition, which is following the expected course for PLS.

• #9 Primary lateral sclerosis: Symptoms and treatment

  https://www.medicalnewstoday.com/articles/primary-lateral-sclerosis

  PLS has no cure, but medications can help manage specific symptoms. These include: baclofen and tizanidine for spasticity, quinine for cramps, diazepam for muscular contractions. […] Other therapies, such as physical and speech therapy, can help the person maintain the ability to move and communicate. […] Although no cure exists, medications and therapies can help manage symptoms. Various organizations provide information, resources, and emotional support to those living with PLS.

• #10

  https://www.painscale.com/article/conventional-medical-treatments-for-primary-lateral-sclerosis-pls

  Primary lateral sclerosis (PLS) is a rare neuromuscular disorder that involves the gradual degeneration of nerve cells more specifically, upper motor neurons in the brain. […] The main treatment goal of PLS is symptom management. Treatments include medications, physical therapy, occupational therapy, speech/swallowing therapy, and mobility devices. […] Medications that are commonly prescribed include the following: Muscle relaxants, such as tizanidine, clonazepam and baclofen, may be prescribed to relieve stiffness and spasticity. […] Antidepressants may be prescribed for pain or depression. […] Anticholinergics, such as glycopyrrolate and scopolamine, can help with drooling problems. […] Analgesics can help decrease pain from spasticity, cramps or neuropathy. Pain medications commonly prescribed are NSAIDS, such as ibuprofen or naproxen; opioids, such as oxycodone or morphine; and anesthetics, such as lidocaine.

• #10

  https://www.painscale.com/article/conventional-medical-treatments-for-primary-lateral-sclerosis-pls

  In addition to medications, various other treatments can help with symptoms, including physical and occupational therapy, speech/swallowing therapy, and mobility devices. […] Physical therapists can provide stretching and strengthening exercises to maintain or improve muscle strength, muscle flexibility, and range of motion. […] Occupational therapists can help individuals maintain independence by teaching them ways to modify everyday activities that they can no longer do or have difficulty doing on their own. […] Speech and swallowing therapy can help maintain facial muscle strength and speech clarity. […] Assistive mobility devices may be recommended by occupational or physical therapists as the disease progresses. Such devices may include a cane, walker or wheelchair.

• #11 Primary Lateral Sclerosis – PLEXUS

  https://plexusnc.com/primary-lateral-sclerosis/

  Today, Cell therapy is regarded as one of the most effective treatments for MND. […] Some of the benefits of Cell therapy for PLS include: Enhanced everyday functioning, Improved quality of life, Immune system modulation and reduction of inflammation, Prevention of further nerve damage, Speedy recovery post-procedure, Non-surgical procedure, Zero complications and side-effects. […] Plexus rehabilitation for MND also offers: Strengthening of muscles in the shoulder, neck, upper and lower limbs, and oral structures, Activities for daily living training, Endurance training and fitness management, Diets and nutrition plans, Hand function training, Functional splinting, Functional stretching for relief from muscle stiffness, Energy conservation and work simplification training, Counseling and caregiver support.

• #12 Primary Lateral Sclerosis

  https://www.sfn.org/sitecore/content/home/brainfacts2/diseases-and-disorders/neurological-disorders-az/diseases-a-to-z-from-ninds/primary-lateral-sclerosis

  Treatment for individuals with PLS is symptomatic. Muscle relaxants such as baclofen, tizanidine, and the benzodiazepines may reduce spasticity. Other drugs may relieve pain and antidepressants can help treat depression. Physical therapy, occupational therapy, and rehabilitation may prevent joint immobility and slow muscle weakness and atrophy. Assistive devices such as supports or braces, speech synthesizers, and wheelchairs may help some people retain independence. […] Speech therapy may be useful for those with involvement of the facial muscles.

• #13

  https://www.alsforums.com/community/threads/speech-therapy-for-pls.43039/

  My father was diagnosed with PLS a few months ago and has been experienced symptoms for more than 2 years. I wanted to ask you all about experiences with speech therapy. […] However, I thought it would be good to have some preventive speech therapy to strengthen muscles while he is fine. […] Any experience with preventive speech therapy? […] I’m not really sure there is preventative therapy in ALS/ PLS, and the speech therapist at your fathers clinic will advise him. […] I recommend he start banking his voice now with ModelTalker before his voice changes.

• #14 Primary lateral sclerosis (PLS) | Altru Health System

  https://www.altru.org/health-library/conditions/primary-lateral-sclerosis-pls

  There are no treatments to prevent, stop or reverse primary lateral sclerosis. Treatment focuses on relieving symptoms and preserving function. […] You might take medicine to relieve these symptoms: […] Muscle spasms may be relieved by baclofen (Fleqsuvy, Ozobax, Lyvispah), tizanidine (Zanaflex) or clonazepam (Klonopin). These medicines are taken by mouth. […] If muscle spasms aren’t controlled with oral medicine, your health care professional might recommend surgery to implant a medicine pump. The pump delivers baclofen directly to the spinal fluid. […] Speech therapy might help with communication and swallowing if your facial muscles are affected by PLS. […] If you have trouble chewing and swallowing, a dietitian can offer diet tips, nutritional supplements or special feeding methods. This can help you maintain your body weight when symptoms make it hard to eat. […] As PLS symptoms get worse, you may need an assistive device. Physical or occupational therapists may evaluate you regularly to determine whether you need a brace, cane, walker or wheelchair. Assistive technology devices also may help with communication.

• #15 Primary Lateral Sclerosis (PLS) – Motor Neurone Disease

  https://www.mndcentre.org.uk/primary-lateral-sclerosis-pls/

  Managing PLS is mostly done by managing the individual symptoms as they manifest. This can include medication in order to alleviate muscle cramping and tightness and physical therapy and exercise in order to maintain the patients flexibility and range of motion. Equipment to assist with day to day activities and communication aids may also be used. […] Light, low impact exercise such as walking or water aerobics can help strengthen muscles to help compensate for those which are weakened by the condition. Stretching exercises such as yoga or pilates could also lessen the severity of muscle cramps and help to maintain motion. The doctor may also prescribe medication to relieve your cramps and stiffness. […] There are many aids that could be useful to continue communication. Your doctor will discuss these with you to see what might be appropriate for you. […] If feeding gets difficult and tiring because of the throat muscles being impaired, it may be the time to consider PEG feeding.

• #16 Primary Lateral Sclerosis: Symptoms, Causes, Progression

  https://www.healthline.com/health/primary-lateral-sclerosis

  Primary lateral sclerosis (PLS) is a rare neurological disorder. It may take several years for a doctor to diagnose PLS. While there’s no cure, treatments can help improve quality of life. […] Treatment focuses on the symptoms a person is experiencing: Various therapies: Certain therapies may be used to help with muscle weakness, joint mobility, and everyday tasks. They may include physical therapy, occupational therapy, speech therapy, and rehabilitation. […] Medications: For example, muscle relaxants, such as baclofen, tizanidine, and benzodiazepines, can help with muscle spasticity. Pain medications, over-the-counter (OTC) or prescription, may help with discomfort. Antidepressants may help with depression. […] Medical devices: Medical devices like canes, wheelchairs, braces, and speech synthesizers can help people manage mobility issues and regain some independence with everyday activities. […] Theres no cure for PLS. While the progression of symptoms can vary from person to person, it’s not always fatal, and people may have a normal life expectancy.

• #17 Primary lateral sclerosis symptoms, treatments & forums | PatientsLikeMe

  https://www.patientslikeme.com/conditions/pls

  Primary lateral sclerosis (PLS) is a rare neuromuscular disease characterized by progressive muscle weakness in the voluntary muscles. PLS belongs to a group of disorders known as motor neuron diseases. […] What people are taking for it: Walker, Walking Stick/Cane, Wheelchair (powered). […] What people are taking for it: Baclofen, Tizanidine, Baclofen intrathecal pump. […] Treatment: Riluzole (Rilutek). Type: Prescription Drug. How many have tried: 107. Tried for: primary lateral sclerosis (2). […] Treatment: Baclofen (Mylan-Baclofen). Type: Prescription Drug. How many have tried: 451. Tried for: primary lateral sclerosis (1). […] Perceived effectiveness: 0 evaluations from primary lateral sclerosis patients report major effectiveness of Baclofen for primary lateral sclerosis (0%).

• #18 PLS Treatment – Find The Best Stem Cells Therapy | GSC

  https://globalstemcells.com/treatment/pls/

  Unique Access provides access to an extensive treatment protocol for Primary Lateral Sclerosis (PLS) which utilises higher quantities of stem cells, innovative stem cell growth factors, extensive rehabilitation, and many supportive therapies and supplements. This effective combination of the most advanced medical technologies with functional medicine has helped patients achieve significant improvements. […] Stem cell therapy is considered to be an alternative method for treating Primary Lateral Sclerosis (PLS) and other neurodegenerative disorders. Stem cell transplantation is a potential therapeutic strategy not only via cell replacement but also by modification of the extracellular motor neuronal environment, through a trophic and neuroprotective effect. […] Unique Access provides access to the very best stem cell treatment packages using higher doses of Mesenchymal Stem Cells (MSC) to treat Primary Lateral Sclerosis (PLS). Many of the treated patients showed major improvements in motor function, muscle tone and strength, speech and swallowing, balance and coordination, and fine and gross motor skills. Moreover, the disease progression has been reported to be significantly slower after stem cell transplantation.

• #19 Primary Lateral Sclerosis (PLS) with adaptive compensatory functional changes | American Journal of Neuroradiology

  https://www.ajnr.org/ajnr-case-collections-diagnosis/primary-lateral-sclerosis-pls-adaptive-compensatory-functional

  Treatment options include symptomatic therapy for spasticity (Baclofen, Tizanidine), Quinine for muscle cramps, and Diazepam for muscular contractions. […] Additional treatment may include physical, occupational, and speech therapy as well as psychosocial support. Current research groups are studying the role of nerve growth factors, axonal transport, and androgen receptors as future treatment targets.

• #20

  https://journals.lww.com/nrronline/fulltext/2024/09000/primary_lateral_sclerosis__more_than_just_an_upper.11.aspx

  Advances in clinical care and recent research achievements: While no disease-modifying treatment has been developed, considerable advances have taken place in the management of common, PLS-associated symptoms such as pseudobulbar affect. […] The introduction of the new diagnostic criteria and the development of the PLS-specific functional rating scale have important practical ramifications for an earlier diagnosis and more accurate clinical monitoring. […] Therapy development efforts in motor neuron diseases have been dominated by ALS and SMA studies and restricted phenotypes, such as PLS or SBMA, have attracted considerably less attention. […] On the basis of the considerable disability associated with PLS, and the compelling radiological evidence of the progressive nature of the condition, it is clear that translational research focusing on the development of disease-modifying therapies is an urgent priority.

• #20

  https://journals.lww.com/nrronline/fulltext/2024/09000/primary_lateral_sclerosis__more_than_just_an_upper.11.aspx

  In addition to disease-modifying therapy initiatives, research efforts to develop effective symptom control for PLS-associated clinical manifestations, such as spasticity, pseudobulbar affect, and spastic dysarthria need to be multiplied. […] The publication of the new diagnostic criteria and the validation of new clinical instruments have important practical ramifications to expedite the diagnosis and monitor disease progression clinically. […] The sample size limitations of single-center initiatives are increasingly overcome by international collaborations and research efforts are increasingly spearheaded by disease-specific consortia paving the way to the first clinical trials in PLS.

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