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Adult Primary Lateral Sclerosis

PLS


Presentation

  • 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]
  • Such syndromes may be present in as many as 60% of patients with ALS. Conversely, the occurrence of motor neuron dysfunction in patients with clinically pure frontotemporal dementia is increasingly recognized.[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]
  • Although researchers don't understand how this gene causes the disease, they know that the ALS 2 gene is responsible for giving instructions for creating a protein called alsin, which is present in motor neuron cells.[mayoclinic.org]
Overeating
  • Strong OUP Oxford , ١١‏/١٠‏/٢٠١٢ - 432 من الصفحات Over the past ten years, there has been an increasing recognition that syndromes of frontotemporal dysfunction (FTD) are a common occurrence in patients with amyotrophic lateral sclerosis (ALS).[books.google.com]
  • His care taker is also his wife, who has been a saint over the last 15 years providing 24 hour care. She is having joint problems, probably as a result of the care taking over the years.[alsforums.com]
  • Causes In primary lateral sclerosis ( PLS ), the nerve cells in the brain that control movement fail over time. This loss causes movement problems, such as slow movements, balance problems and clumsiness.[mayoclinic.org]
  • Over time, your muscles waste away. You may hear a doctor or nurse call this “atrophy.” When this happens, you lose control over movements. It gets harder to walk, talk, swallow, and breathe.[webmd.com]
Drooling
  • . ; 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]
  • […] 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 speaking, slurred speech and drooling[mayoclinic.org]
  • Amitriptyline and other drugs also can help drooling problems. Physical therapy. Stretching and strengthening exercises may help to maintain muscle strength, flexibility and range of motion, and to prevent joint immobility.[drugs.com]
  • He drools, as he has little facial muscle control. He takes 2-3 pills a day of various medications. His care taker is also his wife, who has been a saint over the last 15 years providing 24 hour care.[alsforums.com]
  • Other symptoms are difficulty eating, drooling, dysarthria, dysphonia, choking events with meals, nasal regurgitation of fluids or pulmonary aspiration. Respiratory onset can present with: Dyspnoea and orthopnoea.[patient.info]
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.[hopkinsmedicine.org]
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]
Hyperreflexia
  • Affiliated tissues include spinal cord , and related phenotypes are spastic tetraparesis and hyperreflexia[malacards.org]
  • 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]
  • […] 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]
  • Breathing may also become compromised in the later stages of the disease, causing those patients who develop ventilatory failure to require noninvasive ventilatory support. [3] Hyperreflexia is another key feature of PLS as seen in patients presenting[en.wikipedia.org]
Babinski Sign
  • 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[en.wikipedia.org]
  • 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]
  • Ankle clonus and Babinsky signs were elicited bilaterally. Lumbar puncture was negative for oligoclonal bands. Results MR mages were acquired on a Magnetom Sonata 1.5T scanner (Siemens, Erlangen, Germany).[ajnr.org]
  • Signs of upper motor neuron dysfunction may include limb and trunk spasticity, pathologic spread of deep tendon reflexes, clonus, pathologic reflexes (such as Babinski's sign), and spastic dysarthria.[dynamicchiropractic.com]
Spastic Gait
  • He slowly developed a progressive spastic gait, to the point that a recent fall last year required percutaneous pinning and plating for a right ankle fracture. He takes no medications.[dynamicchiropractic.com]
Agitation
  • Specific medicines have been found to be helpful to treat the agitation, apathy and depression commonly seen in FTLD.[memory.ucsf.edu]

Workup

  • 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]

Treatment

  • 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]

Prognosis

  • 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]
  • 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]
  • 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]
  • (See Prognosis and Treatment.) The slow rate of progression of PLS provides most patients and families with time to adapt to the changes and identify resources for support.[emedicine.medscape.com]
  • These multidisciplinary clinics may provide patients with the necessary treatment that they require by having an occupational therapist, physical therapist, speech language pathologist, dietician and nutritionist, all in one site. [3] Prognosis [ edit[en.wikipedia.org]

Etiology

  • […] 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]
  • 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.[merckmanuals.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 and Epidemiology The cause of PLS is unknown. Clinical neurophysiologic studies confirm upper motor neuron dysfunction in PLS; motor-evoked potentials are absent or delayed, and peripheral conduction is normal.[dynamicchiropractic.com]

Epidemiology

  • 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]
  • Etiology and Epidemiology The cause of PLS is unknown. Clinical neurophysiologic studies confirm upper motor neuron dysfunction in PLS; motor-evoked potentials are absent or delayed, and peripheral conduction is normal.[dynamicchiropractic.com]
Sex distribution
Age distribution

Pathophysiology

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

Prevention

  • 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]

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