OBJECTIVE: To study whether clinical characteristics can differentiate sporadic presentations of hereditary spastic paraparesis (HSP) from primary lateral sclerosis (PLS). [ncbi.nlm.nih.gov]

Presents a consistent chapter organization that delivers all the content you need in a logical, practical manner. Presents a new co-editor, Thomas D. [books.google.com]

Although researchers do not understand how this gene causes the disease, they know that the ALS2 gene is responsible for giving instructions for creating the alsin protein, which is present in motor neuron cells. [humanitas.net]

• Falling

  Weaker muscles may cause you to fall more, which may result in injuries. [humanitas.net]

  Be sure you stay safe, keeping in mind that your muscle weakness puts you at higher risk of tripping and falling. Eat a healthy diet. [drugs.com]

  Weaker muscles may cause you to fall more, which may result in injuries. Oct. 19, 2016 [mayoclinic.org]

  In the present study, people with pure PLS were stronger than regular ALS subjects in muscle tests, with the UMN-focused ALS patients falling somewhere in between. [alzforum.org]

• Weight Gain

  Because PLS can cause your activity level to slow down, be sure you're eating a nutritious diet to avoid excessive weight gain and added pressure on your joints. [drugs.com]

  Activity is vital not only because it will prevent weight gain but also the development of contractions. [ic.steadyhealth.com]

  Discussion In the discussion to their study, Wills et al stated “Patients with amyotrophic lateral sclerosis who received a high-carbohydrate hypercaloric tube-feeding formula with a goal of weight gain were less likely to have serious adverse events, [raredr.com]

  Amitriptyline, in a dosage of 5 to 100 mg at bedtime, can provide antidepressant and antisialorrheic actions as well as nocturnal sedation, potentiation of analgesia and possible weight gain. [aafp.org]

• Hoarseness

  ; Hoarseness, reduced rate of speaking, slurred speech and drooling as the facial muscles weaken; Drooling and difficulties with swallowing and breathing late in the disease Occasionally, some patients suffer from speaking and swallowing difficulties [humanitas.net]

  They include: Stiffness, weakness and spasticity in your legs Tripping, difficulty with balance and clumsiness as the leg muscles weaken Weakness and stiffness progressing to your trunk, then your arms, hands, tongue and jaw Hoarseness, reduced rate of [mayoclinic.org]

  The symptoms of PLS usually progress over a protracted period of time and include: stiffness in the lower limbs lack of muscle strength weakness in the upper limbs and chest (the legs are usually affected first) slurred speech and hoarseness weakness [medic8.com]

  Other symptoms include hoarseness, dysphagia, and slurred speech; because swallowing is difficult, salivation appears to increase, and patients tend to choke on liquids. [msdmanuals.com]

• Sialorrhea

  Some antihistamines, such as diphenhydramine (Benadryl), in a dosage of 25 to 50 mg three times daily, may also be helpful in suppressing sialorrhea. [aafp.org]

  Anterior horn cell loss produces weakness, atrophy, and fasciculations. 10 Involvement of the corticobulbar tract produces UMN-type orofacial weakness and associated signs and symptoms, such as sialorrhea, dysphagia, and dysarthria. [clinicalgate.com]

  Radiotherapy reduces sialorrhea in amyotrophic lateral sclerosis. Eur. J. Neurol. 14, 1373–1377 (2007). 109. Limousin, N. et al. Malnutrition at the time of diagnosis is associated with a shorter disease duration in ALS. J. Neurol. [nature.com]

• Impulsivity

  These tests use a low amount of electrical current to test and measure your nerves' ability to send impulses to muscles in different areas of your body. This test can determine if you have nerve damage. Spinal tap (lumbar puncture). [drugs.com]

  When the motor neurons can no longer send impulses to the muscles, the muscles begin to waste away (atrophy), causing increased muscle weakness. [my.clevelandclinic.org]

  The damage disrupts the normal transmission of nerve impulses within the brain and between the brain and other parts of the body, causing impairment in muscle coordination, strength, balance, sensation, and vision. [ssa.gov]

• Urinary Urgency

  No differences were found in the features used by previous studies to distinguish HSP from PLS, including evidence of mild dorsal column impairment (decreased vibratory sense or abnormal leg somatosensory evoked potentials), symptoms of urinary urgency [ncbi.nlm.nih.gov]

  He experienced the onset of right-hand weakness at 39 years of age, and during the next decade, developed right-leg weakness, dysarthria, and chronic urinary urgency. [ajnr.org]

  The common symptoms of PLS include leg weakness, spasticity, spastic bulbar weakness, dysarthria, dysphagia, urinary urgency, and incontinence. [physio-pedia.com]

• Clumsiness

  This loss causes movement problems, such as slow movements, balance problems and clumsiness. Adult primary lateral sclerosis The cause of adult primary lateral sclerosis is unknown. [mayoclinic.org]

  Symptoms include weakness, muscle stiffness and spasticity, clumsiness, slowing of movement, and problems with balance and speech. PLS is more common in men than in women, with a varied gradual onset that generally occurs between ages 40 and 60. [malacards.org]

  Generally, symptoms consist of stiffness, clumsiness, and awkward movements, usually affecting first the mouth, throat, or both, then spreading to the limbs. [msdmanuals.com]

• Hyperreflexia

  His neurological examination showed only mild facial asymmetry and asymmetric spasticity as well as hyperreflexia affecting the right upper limb. [karger.com]

  Hyperreflexia and cognitive changes which are seen on neuropsychological testing can all be seen in the later stages of the disease. The experts have still not come up with any specific test that will properly diagnose primary lateral sclerosis. [ic.steadyhealth.com]

  His examination was significant for hyperreflexia and spasticity in all 4 limbs, minimal voluntary movement of his upper extremities, lower extremity paresis, and dysarthria. [ajnr.org]

  The diagnostic process consists of a history and physical examination, repeated at regular intervals, to document progressive hyperreflexia, fasciculations, and upper and lower motor neuron involvement. [aafp.org]

  […] sheath -Causing the axon to break into pieces -The axon debris is cleaned up by macrophages ALS causes degneration of the corticospinal upper motor neurons that reside in the cerebral cortex specifically the precentral gyrus 1. spastic paralysis 2. hyperreflexia [quizlet.com]

• Slurred Speech

  ; Hoarseness, reduced rate of speaking, slurred speech and drooling as the facial muscles weaken; Drooling and difficulties with swallowing and breathing late in the disease Occasionally, some patients suffer from speaking and swallowing difficulties [humanitas.net]

  speech and drooling as the facial muscles weaken Difficulties with swallowing and breathing late in the disease Less commonly, PLS begins in your tongue or hands and then progresses down your spinal cord to your legs. [mayoclinic.org]

  Both upper and lower motor neurones are effected and symptoms include slurred speech, choking on certain food textures and coughing when having a drink. [southtees.nhs.uk]

  The symptoms of PLS usually progress over a protracted period of time and include: stiffness in the lower limbs lack of muscle strength weakness in the upper limbs and chest (the legs are usually affected first) slurred speech and hoarseness weakness [medic8.com]

• Babinski Sign

  Affiliated tissues include brain, testes and cortex, and related phenotypes are dysphagia and babinski sign OMIM : 58 Although primary lateral sclerosis (PLS) is similar to amyotrophic lateral sclerosis (ALS; 105400), they are considered to be clinically [malacards.org]

  There are no standard laboratory tests for upper motor neuron disease, but spasticity (a specific type of stiffness), abnormally brisk tendon reflexes, Babinski’s sign and diminished fine motor coordination are seen as diagnostic signs on examination. [memory.ucsf.edu]

  sign. [4] Some people present with emotional lability and bladder urgency, [4] and occasionally people with PLS experience mild cognitive changes detectable on neuropsychological testing, particularly on measures of executive function. [5] PLS is not [en.wikipedia.org]

  sign positive) Flexor ( normal; Babinski's sign negative) Extraneous muscle activity No fasciculations/fibrillations Fasciculations and fibrillations amyotrophic lateral sclerosis (ā·mī· ·trō·fik laˑ·t ·r l skl ·rōˑ·sis), n a fatal neurological condition [medical-dictionary.thefreedictionary.com]

  Lower motor neuron loss causes initially increased electrical excitability leading to fasciculations, and later muscle weakness and atrophy; upper motor neuron involvement causes spasticity, clonus, hyperactive tendon reflexes, and Babinski signs. [neuropathology-web.org]

• Spastic Gait

  Treatment is based on the signs and symptoms present in each person. 0002015 EMG: chronic denervation signs 0003444 Loss of speech 0002371 Progressive spastic paraparesis 0007199 Pseudobulbar signs 0002200 Spastic dysarthria 0002464 Spastic gait Spastic [rarediseases.info.nih.gov]

  He then developed a severe right flexion contracture of the right hand with progressive right lower limb spasticity and a spastic gait. [karger.com]

Mixed UMN and LMN or mainly UMN disorders resembling ALS are uncommon but are important to consider in the workup of patients with possible ALS. [clinicalgate.com]

• Gliosis

  The MSCs transplantation increases neuron survival and prevents gliosis, as microglia and strocytes are both the target and cause of neuroinflammation, MSCs can rescue neurons and oligodendrocytes from apoptosis through the release of trophic and anti-apoptotic [globalstemcells.com]

  Loss of motor neurons and hypercellularity due to gliosis. At one end of the spectrum, SMA 0, are cases that have a prenatal onset, paralysis of facial and extraocular muscles, and a very short survival. [neuropathology-web.org]

  Pathology Atrophy of the anterior horn cells and replacement of the large motor neurons by fibrous astrocytes (gliosis) causes the affected anterior and lateral columns of the spinal cord to become hard, hence the term “lateral sclerosis.” 4 Large neurons [aafp.org]

Offers practical, clinically relevant material for the diagnosis and treatment of musculoskeletal conditions. [books.google.com]

We combine internationally accredited hospitals, next generation treatments, unique products and services that are integrative and effective to ensure best possible treatment results. [globalstemcells.com]

Treatment Treatment for primary lateral sclerosis ( PLS ) focuses on relieving symptoms and preserving function. There are no treatments to prevent, stop or reverse PLS. Treatments include: Medication. [drugs.com]

[…] and support There is no cure for motor neurone disease, but treatment can help reduce the impact the symptoms have on your life. [nhs.uk]

PLS is a disease of the UMN, distinguished from ALS in prognosis and absence of LMN signs. [ncbi.nlm.nih.gov]

Pure primary lateral sclerosis and UMN-dominant ALS appear to have a more benign prognosis than typical ALS. Survival for patients with UMN-dominant ALS was intermediate between that of primary lateral sclerosis and classic ALS. [pallipedia.org]

This cautious approach is understandable, given the prognosis of the illness and the devastation that being given the diagnosis may cause to a person's life. [patient.info]

Prognosis A distinctive clinical feature of PLS is that it has a very slow progression, leading it to be considered to have a more benign prognosis in comparison to ALS [3]. [physio-pedia.com]

In 21 people with muscular atrophy in only specific parts of the arm, for more than four years, the prognosis was good: they were likely to survive for many years with little deterioration of muscle function. [alzforum.org]

[…] maintain a repository of clinically characterized patients with primary lateral sclerosis for future research protocols, to characterize the natural history of neurodegenerative disorders with corticospinal neuron degeneration, to investigate proposed etiologies [clinicaltrials.gov]

We present 5 cases of progressive hemiparetic corticospinal tract degeneration, identified by the clinical presentation and the exclusion of other etiologies using serological, imaging, and electrodiagnostic studies. 2015 S. [karger.com]

GEFs regulate the activity of members of the Ras superfamily of GTPases." [6] At least 10 deletion mutations and 1 missense mutation of the alsin gene have been shown to cause JPLS. [6] (See Etiology.) [emedicine.medscape.com]

Etiology ALS may have a multifactorial etiology or may result from a number of different neuronal insults. The major lines of investigation include genetic, viral, autoimmune 11 and neurotoxic hypotheses. [aafp.org]

Usually, etiology is unknown. Nomenclature and symptoms vary according to the part of the motor system most affected. Myopathies have similar features but are disorders of the muscle membrane, contractile apparatus, or organelles. [msdmanuals.com]

Disease: Amyotrophic Lateral Sclerosis (ALS), Familial ALS, Sporadic ALS, Primary Lateral Sclerosis (PLS), Study Type: Observational Study Study Category: Epidemiology Study Status: Enrolling Phase : Not Applicable Study Chair(s)/Principal Investigator [neals.org]

We now have a fair amount of research in vitro and epidemiologically associating low levels of vitamin D with neurodegenerative disease,” said James Leverenz, MD, director of the Center for Brain Health at the Cleveland Clinic. [neurologyadvisor.com]

Epidemiological aspects of ALS are summarized in Table 66-1. [clinicalgate.com]

Epidemiology of ALS in Italy: a 10-year prospective population-based study. Neurology 72, 725–731 (2009). 10. O'Toole, O. et al. Epidemiology and clinical features of amyotrophic lateral sclerosis in Ireland between 1995 and 2004. J. Neurol. [nature.com]

Clinical epidemiology of amyotrophic lateral sclerosis. Neurol Clin. 1996;14:399–420. 10. Swash M, Schwartz MS. What do we really know about amyotrophic lateral sclerosis? J Neurol Sci. 1992;113:4–16. 11. Drachman DB, Kundel RW. [aafp.org]

Progress has been made in understanding the genetic defects and the pathophysiology of this crippling motor neuron disease (commonly called Lou Gehrig’s disease). [nejm.org]

ALS shares similar pathophysiologic pathways with multiple sclerosis, Alzheimer’s disease, and Parkinson’s disease. [neurologyadvisor.com]

Pathophysiology This is a degenerative condition that affects motor neurons, namely the anterior horn cells of the spinal cord and the motor cranial nuclei. [patient.info]

The investigation of the role of cerebellar structure in larger PLS population using structural and functional MRI can be of value in understanding the pathophysiology of the disease and its clinical and cognitive manifestations. [journals.plos.org]

Pain is addressed according to the pathophysiology of the problem causing the pain. [the-medical-dictionary.com]

Discusses physical agents and therapeutic exercise in the prevention, diagnosis, treatment and rehabilitation of disorders that produce pain, impairment, and disability. [books.google.com]

The MSCs transplantation increases neuron survival and prevents gliosis, as microglia and strocytes are both the target and cause of neuroinflammation, MSCs can rescue neurons and oligodendrocytes from apoptosis through the release of trophic and anti-apoptotic [globalstemcells.com]

Stretching and strengthening exercises may help to maintain muscle strength, flexibility and range of motion, and to prevent joint immobility. Heating pads can help relieve your symptoms of muscle pain. Speech therapy. [drugs.com]

Spasticity -Prevent contracture of muscles Low impact aerobics: swimming, walking, stationary bike -Strengthen unaffected muscles -Cardiovascular health -Decrease depression -Decrease fatigue Emerging Medical Treatment 1. [quizlet.com]

However, there are treatments and therapies that help to ease and prevent symptoms and improve quality of life. The most commonly used treatments include: Medication: medicines may be prescribed to prevent muscle spasms. [medic8.com]