Materiały źródłowe

• #1 Health Information Library | Rumah Sakit Pusat Pertamina

  https://rspp.co.id/dcontent.html?id=CON-20197155&n=Primary%20lateral%20sclerosis%20(PLS)

  Primary lateral sclerosis (PLS) is a type of motor neuron disease. A motor neuron disease affects the nerve cells in the brain that control movement. In PLS, the breakdown of nerve cells causes weakness in the muscles that control the legs, arms and tongue. […] Symptoms typically begin with balance trouble. People with PLS may move slowly and are clumsy. Over time, symptoms evolve to include having trouble with their hands and arms, followed by problems with chewing, swallowing and speaking. Less commonly, the condition may begin with problems with speaking and swallowing. […] Symptoms of primary lateral sclerosis usually take years to develop and get worse. Symptoms generally begin in the legs. Rarely, PLS begins with weakness in the tongue or hands. Then weakness gradually moves down the spinal cord to the legs. However, symptoms vary from person to person. Symptoms may include: Stiffness, weakness and muscle spasms in the legs. This is known as spasticity. Rarely, spasticity starts in only one leg and moves to the arms, hands, tongue and jaw. Slowed movement. Tripping, being clumsy and having trouble with balance. Hand clumsiness. A hoarse voice, as well as slowed, slurred speech and drooling. Trouble chewing and swallowing. Sometimes, frequent, rapid and intense shifts in emotions. Rarely, breathing problems and bladder problems late in the disease.

• #2 Primary lateral sclerosis (PLS)

  https://www.mymlc.com/health-information/diseases-and-conditions/p/primary-lateral-sclerosis-pls/

  Primary lateral sclerosis (PLS) is a type of motor neuron disease that causes the nerve cells in the brain that control movement to fail over time. PLS causes weakness in your voluntary muscles, such as those you use to control your legs, arms and tongue. You may experience movement problems, such as difficulty with balance, slow movements and clumsiness. You may eventually experience problems with chewing, swallowing and speaking. […] Signs and symptoms of primary lateral sclerosis usually take years to progress and generally begin in the legs. Rarely, PLS begins in your tongue or hands and then progresses down your spinal cord to your legs. However, the disease has highly variable effects from person to person. Signs and symptoms may include: Stiffness, weakness and muscle spasms (spasticity) in your legs, rarely starting in one leg, and eventually progressing to your arms, hands, tongue and jaw. Slowed movement. Tripping, clumsiness and difficulty with balance. Hand clumsiness. Hoarseness, as well as slowed, slurred speech and drooling. Difficulties with chewing and swallowing. Sometimes, frequent, rapid and intense shifts in emotions (mood lability). Rarely, breathing problems and bladder problems late in the disease.

• #2 Upper Motor Neuron Disorders: Primary Lateral Sclerosis, Upper Motor Neuron Dominant Amyotrophic Lateral Sclerosis, and Hereditary Spastic Paraplegia

  https://www.mdpi.com/2076-3425/11/5/611

  Following the exclusion of potentially reversible causes, the differential for those patients presenting with a predominant upper motor neuron syndrome includes primary lateral sclerosis (PLS), hereditary spastic paraplegia (HSP), or upper motor neuron dominant ALS (UMNdALS). […] The core clinical features of a distinct clinical entity, primary lateral sclerosis (PLS), were first described in 1945 and included insidious onset, slow progression without plateau or remission, and examination findings limited to the pyramidal tracts without evidence of involvement of additional parts of the central nervous system. […] The majority of cases present after age 20, with the only exception being a rare form of juvenile hereditary PLS associated with the alsin gene. […] Average symptom onset is earlier than ALS, occurring in the 5th–6th decade as opposed to an average of 65 years for ALS, with a slight male predominance, similar to ALS.

• #3 PLS vs. ALS: Causes, Symptoms, Treatment, and OutlookHealthline

  https://www.healthline.com/health/pls-vs-als

  PLS and ALS are two types of motor neuron disease. PLS affects only upper motor neurons and progresses slowly. ALS affects both upper and lower motor neurons and progresses more quickly. […] The symptoms of PLS may include: muscle weakness, muscle spasticity, slow movement, clumsiness and trouble with balance, difficulties with speech. […] PLS typically progresses more slowly than ALS. It initially affects the legs before progressing to other areas like the torso, arms, and face. […] Because PLS progresses more slowly, it’s typically not fatal. Depending on your age and overall health, you can live with PLS for at least a decade and often longer, with the average duration of the disease ranging between 7.2 to 14.5 years. […] PLS only affects the upper motor neurons and typically starts in the legs before progressing to the torso, arms, and face. Overall, it progresses much more slowly than ALS and is usually not fatal.

• #4 PLS vs. ALS: Symptoms, causes, and treatments

  https://www.medicalnewstoday.com/articles/pls-vs-als

  PLS is a motor neuron disease that primarily affects the upper motor neurons to cause progressive weakness in the legs. This weakness can spread to the arms and other muscles as the disease progresses. The disease typically occurs during adulthood and progresses slowly. […] PLS progresses slowly and may take years to affect mobility. However, ALS rapidly progresses to cause severe disability and death within 35 years of symptom onset. […] However, the symptoms of PLS typically affect the lower body first, before affecting the upper body, and people with PLS typically have trouble walking. […] These symptoms will occur much quicker in people with ALS than in PLS.

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

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

  Primary lateral sclerosis (PLS) is a rare neuromuscular condition. You might first notice muscle spasms, stiffness and weakness in your legs. The condition slowly progresses to other body areas. Theres no cure for PLS. Treatment helps manage your symptoms. […] Symptoms usually begin in your legs. Over time, weakness and stiffness spread to other muscles throughout your body. […] Because theres no cure for PLS, treatment focuses on managing your symptoms and providing mobility support like using a cane or a walker. […] Symptoms of PLS develop slowly. Some of the first symptoms you may notice are: Muscle stiffness in your legs. Muscle weakness in your legs. Difficulty walking or maintaining your balance. Muscle spasms or painful cramps. […] As the condition progresses, other symptoms may include: Muscle stiffness and weakness in your fingers, hands and arms. Difficulty controlling your bladder (urinary urgency and incontinence). Pain in your lower back and neck.

• #6 Upper Motor Neuron Disorders: Primary Lateral Sclerosis, Upper Motor Neuron Dominant Amyotrophic Lateral Sclerosis, and Hereditary Spastic Paraplegia

  https://www.mdpi.com/2076-3425/11/5/611

  The majority of patients who do not present with spastic paraparesis will present with corticobulbar dysfunction including speech changes (rate change prior to dysarthria), dysphagia (milder as compared to ALS with a lower rate of gastrostomy requirements), as well as pseudobulbar affect. […] Other less common presentations include a hemiparetic (Mill’s variant) and upper extremity onset, although the majority of upper limb onset variants include some lower extremity or bulbar dysfunction with pure upper limb onset being more consistent with ALS. […] While the paraparetic form of PLS has recently been shown to be associated with an earlier age of onset as compared the hemiparetic or bulbar onset forms, site of onset does not appear to predict a difference in survival. […] Spread typically occurs from side to side, region to region, and ultimately results in spastic quadriparesis and bulbar dysfunction.

• #7 PLS : HSP & PLS : Spastic Paraplegia Foundation

  https://sp-foundation.org/understanding-pls-hsp/pls.html

  When upper motor neurons degenerate, impulses cannot adequately reach the lower motor neurons and the lower motor neurons cannot deliver the proper message to the corresponding muscle. The result is muscle weakness and spasticity. As the degeneration progresses, symptoms increase. […] In PLS, the degenerative process impacts the length of the spinal cord, thus affecting the legs, arms and speech and swallowing muscles. For most patients, the process begins in the legs and then travels up the spinal cord. In some people, the process begins in the brainstem, affecting the speech and swallowing muscles first, and then descends down the spinal cord.

• #8 Progression in Primary Lateral Sclerosis: a prospective analysis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3434688/

  To determine whether rates and patterns of progression differ among Primary Lateral Sclerosis (PLS) patients. […] Forty-seven patients continued to fulfill criteria for PLS over a mean follow-up of 6.6 years, with a mean disease duration 14 years. PLS-A patients had more predictable progression to additional body regions. Severity progressed faster in newly affected regions followed by stabilization in PLS-A or PLS-M subtypes. […] Clinical progression in PLS does not occur steadily, but has periods of faster decline upon spreading to a newly affected region. Classification of PLS patients by subtype is more relevant to predicting the spread of disease, but not progression of severity. […] Patients with the ascending subtype gave a history of steady progression of symptoms from legs to upper extremities to cranial regions.

• #9

  https://www.painscale.com/article/progression-and-potential-complications-of-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. […] There are two main patterns of PLS progression: ascending and multifocal. A key feature of PLS is relatively slow progression. […] The ascending subtype of PLS generally involves a steady and predictable progression from one region to the next. Symptoms of this subtype typically begin with stiffness and weakness in one leg before spreading to the other leg. Eventually, stiffness and weakness progress to the trunk, then the arms, hands, tongue and jaw. On average, advancement of symptoms from the legs to upper extremities takes about three and a half years. Meanwhile, progressive motor impairment occurs in the areas that were affected first. The median interval when cranial nerves are affected is a year and a half after the upper extremities.

• #10 Progression in Primary Lateral Sclerosis: a prospective analysis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3434688/

  The median interval between the onset of leg symptoms and upper extremity symptoms was 3.5 years and the median interval between the onset of leg symptoms and changes in speech was 5.0 years (range 1-15 years). […] During the prospective follow-up, only 4 of 26 patients exhibited progression of UMN signs to a previously unaffected region. However, within affected regions, the severity of motor impairment increased over time. […] The multifocal subtype of PLS had an asymmetric or patchy pattern of symptom spread by history. […] The time course of the spread of symptoms reported prior to enrollment was variable, although several patients had reported long periods of stability between progression of symptoms from one region or limb to another. […] In the absence of differences in age of onset, duration, survival or risk factors, there is little rationale at this time to search for different etiologies between PLS subtypes.

• #11

  https://www.painscale.com/article/progression-and-potential-complications-of-primary-lateral-sclerosis-pls

  The spread of multifocal PLS has a less predictable pattern than ascending PLS. Progression from one area to another is asymmetric and patchy. For instance, initial progressive symptoms may ascend on only one side of the body, or onset may start in one limb and then spread to the face before affecting the corresponding limb on the other side of the body. About 50% of people with this subtype report periods where progression seems to stop. Like the ascending subtype, once symptoms reach the upper extremities, advancement to cranial nerves soon follows.

• #12 PLS : HSP & PLS : Spastic Paraplegia Foundation

  https://sp-foundation.org/understanding-pls-hsp/pls.html

  Wherever symptoms originate, the legs, arms, hands, and speech and swallowing muscles will all eventually be affected. In time, assistive devices are needed, such as canes, walkers, or wheelchairs. Speech therapy and communication aids may become necessary as well. Many people report painful muscle spasms and other pain. Other common symptoms are hyperactive reflexes and Babinkskis sign. Emotional lability is often reported as well. […] The rate at which symptoms will progress and how severe they will become is impossible to predict. In general, the course is a slowly progressive one. Some individuals report periods of acceleration, while others have long plateaus where progression appears to stop. […] Researchers believe it can take several years for all parts of the body to become affected. An individuals genes, environment, nutrition, general health, and other factors may influence the rate of progression and severity of the disease.

• #13 Upper Motor Neuron Disorders: Primary Lateral Sclerosis, Upper Motor Neuron Dominant Amyotrophic Lateral Sclerosis, and Hereditary Spastic Paraplegia

  https://www.mdpi.com/2076-3425/11/5/611

  Average symptom duration varies from 7.2–14.5 years based on prior case series, longer than the typical average duration of 3–5 years quoted for ALS. […] Clinical examination in PLS often demonstrates spasticity, hyperreflexia, and only mild weakness as previously noted which is symmetric in most cases with a lack of sensory findings. […] While pseudobulbar affect is well described in PLS, its presence at initial presentation is atypical and may predict future development of LMN signs. […] Urinary symptoms (frequency) can be a complaint in one-third to one-half of patients later in the disease course. […] While dementia is generally considered less common in PLS as compared to ALS, early studies had small sample sizes due to the rarity of PLS and most only utilized neuropsychological screening instruments. […] More recent dedicated studies on the neuropsychological profiles of patients with PLS have recognized similar changes as compared to ALS cohorts in the domains of executive, language, memory, and fluency.

• #14 Health Information Library | Rumah Sakit Pusat Pertamina

  https://rspp.co.id/dcontent.html?id=CON-20197155&n=Primary%20lateral%20sclerosis%20(PLS)

  It can take as long as 20 years for primary lateral sclerosis to progress and become worse. Symptoms vary from person to person. Some people continue to walk, but others eventually need to use wheelchairs or other assistive devices. […] For most people, adult-onset PLS isn’t thought to shorten life expectancy. But it can gradually affect the quality of your life as more muscles become disabled. Weaker muscles can cause you to fall, which can result in injuries. Problems with chewing and swallowing may result in poor nutrition. Performing activities of daily living, such as bathing and dressing, may become hard. […] People with PLS may develop problems with their thinking, known as cognitive decline. Or they may have changes in their behavior. For some people, their symptoms overlap with symptoms of frontotemporal dementia.

• #15

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

  Clinical experience suggests that the diagnostic journey of patients with PLS is particularly circuitous. […] The uncertainty while awaiting the confirmation of PLS and reassuringly demonstrating that the clinical trajectory is not consistent with ALS is a source of distress for the patients and their caregivers. […] Recent case-series suggest that the most affected neuropsychological domains in PLS are language, social cognition, and executive function. […] Extra-pyramidal manifestations are another understudied facet of the disease. […] 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. […] 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.

• #16 Primary Lateral Sclerosis – StatPearls – NCBI Bookshelf

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

  Primary lateral sclerosis (PLS) is a rare neurodegenerative disorder that primarily affects the upper motor neurons, leading to progressive muscle stiffness, poor coordination, balance difficulties, and sometimes speech and swallowing challenges. […] The disorder typically begins in the lower limbs and progresses slowly over years, with symptoms often persisting for 7 to 14 years. […] Clinical symptoms include stiffness, bulbar symptoms (eg, difficulty speaking and swallowing), labile affect (ie, pseudobulbar affect), mild weakness, and balance and coordination difficulties. […] The progression of symptoms is variable but generally slow, with the average symptom duration ranging from 7.2 to 14.5 years. […] PLS differs from ALS in that the need for a feeding tube or permanent assisted ventilation is low at 7% and less than 1%, respectively.

• #17 Upper Motor Neuron Disorders: Primary Lateral Sclerosis, Upper Motor Neuron Dominant Amyotrophic Lateral Sclerosis, and Hereditary Spastic Paraplegia

  https://www.mdpi.com/2076-3425/11/5/611

  Onset of symptoms is often insidious, with patients having a more difficult time discerning the exact onset of symptoms as compared to those with ALS. […] At the time of initial evaluation, patients typically report stiffness of the extremities (especially the legs), clumsiness leading to falls, or bulbar symptoms, such as dysarthria, dysphagia, or emotional lability. […] Patients may report periods of gradual decline followed by apparent stabilization that may last several months. […] With the more typical lower extremity onset (90% of cases), patients often seek medical attention once spasticity has progressed to a point where it is significantly impairing locomotion and perhaps increasing falls. […] While patients may describe weakness, this is more typically secondary to incoordination/imbalance due to spasticity.

• #18

  https://www.alsforums.com/community/threads/pls-progression-timeline.30658/

  I have Bulbar PLS (starts with speech). PLS progression can very wildly. However, I have heard of at least 4 people where Bulbar PLS has progressed at a similar rate. Year 1 – Bulbar symptoms start Year 2 – Speech problems progress and you become hard to understand Year 3 – Speech is worse but progress more or less stabilizes Year 5 – Balance and leg spasticity begin. Year 6 – Need cane or walker to walk. Year 7 – Need scooter or PWC to get around […] Swift decline starting in Dec 2012 after spinal fusion surgery. Now, (almost) Dec 2015, 99.9 % use scooter. Can still stand and transfer, but balance is gone, clonus and spasms increased, speech slowed, fingers curled, PBA, swallowing problems. […] I have Bulbar PLS. Usually it takes 4-5 years to get a PLS diagnosis since certain types of ALS can appear as PLS in the early years. I was given a PLS diagnosis after 2 years because Bulbar ALS progresses rapidly.

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

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

  PLS slowly breaks down the UMN (degeneration), so your muscles dont get the messages they need to move as expected. […] As PLS progresses, you may have trouble walking without assistance. You might need to use a cane, a walker or a wheelchair. In addition, muscle weakness may make you more at risk of falls or accidents where you could injure your body if you lose balance. […] How fast your symptoms appear and worsen varies from person to person. On average, the progression of primary lateral sclerosis is slow and happens over many years or decades. If you notice a sudden worsening of symptoms, contact your healthcare provider. […] 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.

• #20 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

  The patient completed the 10-Metre Walk Test in 20 seconds, taking 21 steps. […] The patient’s current ALSFRS-R score is 36 due to the appearance of bulbar symptoms. During this time, the patient has continued walking, although he currently requires 2 canes to walk both indoors and outdoors. […] 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.

• #21 Primary lateral sclerosis (PLS)

  https://www.mymlc.com/health-information/diseases-and-conditions/p/primary-lateral-sclerosis-pls/

  Average progression of primary lateral sclerosis can take as long as 20 years and has highly variable effects from person to person. Some people continue to walk, but others eventually need to use wheelchairs or other assistive devices. In most cases, adult-onset PLS isn’t thought to shorten life expectancy, but it can gradually affect the quality of your life as more muscles become disabled. Weaker muscles can cause you to fall, which can result in injuries. Problems with chewing and swallowing may result in poor nutrition. Performing activities of daily living, such as bathing and dressing, may become difficult.

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

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

  As PLS progresses, mobility and communication may become more difficult, which can also limit a person’s independence. That said, not everyone who has PLS will experience all the possible symptoms. And symptoms that do develop may not happen all at the same time. […] There’s 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.

• #23 Primary Lateral Sclerosis – StatPearls – NCBI Bookshelf

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

  The most frequent complications seen in PLS include movement issues and falls secondary to gait impairment, balance difficulties, and weakness. […] Given the progressive and irreversible nature of the disease, mood symptoms like depression are frequently seen in patients with PLS. […] Patients with PLS often have a benign clinical course with a more prolonged survival compared to patients with ALS. […] Patients with selective UMN involvement who do not develop LMN involvement after 4 years typically remain with pure UMN involvement and have a normal lifespan.

• #24 PLS vs ALS: Difference, Symptoms, Causes, Treatments

  https://www.verywellhealth.com/pls-vs-als-6828402

  Symptoms of PLS take several years to progress, while symptoms of ALS come on more rapidly. […] PLS progresses gradually over several years or even decades. […] Common symptoms of PLS include: Weakness in the legs, Muscle twitching and spasticity, Difficulty walking, Balance problems, Clumsiness, Slowing of movement, Speech or swallowing problems, Stiffness and weakness start in the legs and slowly spread to the torso area. […] Symptoms of PLS usually go on to live a normal life span, and those diagnosed with ALS typically have a life expectancy of three to five years.

• #25 Primary Lateral Sclerosis: Background, Etiology, Epidemiology

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

  Primary lateral sclerosis (PLS) is a progressive, degenerative disease of upper motor neurons characterized by progressive spasticity (ie, stiffness). It affects the lower extremities, trunk, upper extremities, and bulbar muscles (usually in that order). […] Patients with PLS occasionally have mild, nonspecific, and nonprogressive findings of denervation on electrodiagnostic testing. The severity of the denervation and reinnervation does not resemble that seen in ALS and does not justify these patients’ being classified as having ALS. These patients may be concerned that their PLS eventually could evolve into ALS. Although absolute guarantees cannot be given, some measure of reassurance may be derived from the overall slow progression in these patients. […] Dysfunction and disability accrue slowly as primary lateral sclerosis (PLS) progresses. […] PLS has not been considered to shorten life expectancy. However, inspection of reported survival data from 36 patients with PLS now suggests that the median survival is approximately 20 years.

• #26 Primary lateral sclerosis: consensus diagnostic criteria | Journal of Neurology, Neurosurgery & Psychiatry

  https://jnnp.bmj.com/content/91/4/373

  Spasticity with pathological hyperreflexia are invariable examination findings. […] Bladder instability resulting in frequency and varying degrees of retention is a common accompaniment to PLS. […] The presence of wider brain involvement in PLS is increasingly recognised clinically but is typically minor in relation to the core clinical syndrome. […] The diagnosis of PLS requires the presence of symptoms of progressive upper motor neuron (UMN) dysfunction for at least 2 years. […] Probable PLS is defined by the absence of significant active LMN degeneration 2 years from symptom onset. […] Definite PLS is defined by the absence of significant active LMN degeneration 4 or more years from symptom onset. […] The late development of LMN involvement in some cases of UMN-predominant ALS has the potential to lead to misclassification of PLS. […] The development of an international registry that includes all those with probable PLS will allow a more precise delineation of the pathogenesis from ALS, and accelerate therapeutic developments.

• #27 Primary lateral sclerosis – UMC Utrecht

  https://www.umcutrecht.nl/en/primary-lateral-sclerosis

  Primary lateral sclerosis (PLS) is a rare motor neuron disease where symptoms progress slowly over a number of years. PLS affects the nerve cells that send messages from the brain down the spinal cord and on to the muscles. This causes weakness, especially in the legs, and stiffness in the muscles and joints (known as spasticity). Some people develop weakness and stiffness in the muscles used for speech and swallowing too. Not all symptoms necessarily happen to everyone and they do not usually develop at the same time. […] Life span could essentially be normal, although it may be life-limiting, depending on whether it remains as pure PLS or develops into ALS. Within the first four years from developing symptoms, people thought to have PLS can additionally develop lower motor neuron symptoms leading to the diagnosis ALS. […] PLS typically affects people aged 35-65 years. There is no cure or treatment for PLS, but symptoms can be managed to improve quality of life.

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

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

  There is no single test that confirms a diagnosis of primary lateral sclerosis (PLS). PLS can have symptoms similar to other neurological diseases such as multiple sclerosis and ALS. […] Sometimes it takes 3 to 4 years before a diagnosis can be made. This is because early ALS can look like PLS until other symptoms surface a few years later. You might be asked to return for repeat EMGs over 3 to 4 years before a PLS diagnosis is confirmed. […] There are no treatments to prevent, stop or reverse primary lateral sclerosis. Treatment focuses on relieving symptoms and preserving function. […] Muscle spasms may be relieved by baclofen (Fleqsuvy, Ozobax, Lyvispah), tizanidine (Zanaflex) or clonazepam (Klonopin). These medicines are taken by mouth. […] 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.

• #29

  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. […] In addition to medications, various other treatments can help with symptoms, including physical and occupational therapy, speech/swallowing therapy, and mobility devices. […] Assistive mobility devices may be recommended by occupational or physical therapists as the disease progresses.

• #30

  https://www.als.net/news/als-and-pls-two-similar-neurodegenerative-diseases-with-important-differences/

  They can often be confused for one another in their early stages, and some even refer to PLS as a benign variant of ALS although it can still cause disabling complications. […] The most obvious distinction between the two diseases is the speed at which they generally progress. […] The death of these neurons causes progressively worsening weakness and spasticity of the muscles. It can also lead, like ALS, to problems with speech and swallowing. […] Therefore, PLS does not lead to muscle wasting, as in ALS, even though it does weaken them. […] It usually begins with weakness in the legs, which can lead to tripping and difficulty walking, and progresses to the body, arms, hands, and the face and mouth, although some cases begin in the upper body or face and travel downwards. […] Similar to ALS, there are no effective treatments for PLS other than treating the symptoms with drugs like muscle relaxers and pain relievers, as well as physical and occupational therapy.

• #31 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

  Primary lateral sclerosis (PLS) is a rare neuromuscular disease with slowly progressive weakness in voluntary muscle movement. PLS affects the upper motor neurons (also called corticospinal neurons) in the arms, legs, and face. Symptoms include weakness, muscle stiffness and spasticity, clumsiness, slowing of movement, and problems with balance and speech. PLS progresses gradually over a number of years, or even decades. PLS is not fatal. There is no cure and the progression of symptoms varies. Some people may retain the ability to walk without assistance, but others eventually require wheelchairs, canes, or other assistive devices. […] When symptoms begin, PLS may be mistaken for amyotrophic lateral sclerosis (ALS) or spastic paraplegia.

• #32 Primary Lateral Sclerosis: An Overview

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

  The clinical distinction between these two entities could be practically impossible in some circumstances, and genetic testing remains essential for ruling out HSP as the etiology for an apparently sporadic adult-onset UMN syndrome with leg onset. […] The most accurate diagnosis possible will, in turn, allow us to study more homogeneous patient samples with a view to obtaining a better pathophysiological interpretation of the disease and, last but not least, to identify possible disease-modifying treatments.

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