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Adult Primary Lateral Sclerosis

PLS


Presentation

  • 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]
  • 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]
  • Case Reports The 2 patients presented herein were referred from the neurology department with a diagnosis of PLS.[ajnr.org]
  • To study whether clinical characteristics can differentiate sporadic presentations of hereditary spastic paraparesis (HSP) from primary lateral sclerosis (PLS).[ncbi.nlm.nih.gov]
  • Clinical presentation ALS may present initially with signs of only upper or lower motor neuron involvement.[emedicine.medscape.com]
Dutch
  • One hundred four Dutch patients with an adult-onset, sporadic upper motor neuron syndrome of at least 3 years' duration. Hereditary spastic paraparesis was genetically confirmed in 14 patients (7 with SPG4 and 7 with SPG7 mutations).[ncbi.nlm.nih.gov]
  • In the Dutch study, in fact, three patients initially diagnosed with non-ALS conditions were eventually reclassified as having true ALS.[alzforum.org]
  • To investigate whether these two seipin/BSCL2 mutations are present in patients with sporadic HSP and PLS, we screened exon 3 of the seipin/BSCL2 gene in 86 Dutch patients with a sporadic adult-onset UMN syndrome.[link.springer.com]
Hyperreflexia
  • His neurological examination showed only mild facial asymmetry and asymmetric spasticity as well as hyperreflexia affecting the right upper limb.[karger.com]
  • 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]
  • Treatment is based on the signs and symptoms present in each person. 0002015 Hyperreflexia Increased reflexes 0001347 Slow progression Signs and symptoms worsen slowly with time 0003677 Spastic dysarthria 0002464 Spastic gait Spastic walk 0002064 Spastic[rarediseases.info.nih.gov]
Babinski Sign
  • 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]
  • 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]
  • All deep tendon reflexes were exaggerated with bilateral ankle clonus and Babinski sign. There was no sensory or cerebellar dysfunction. Routine hematological and biochemical investigations including chest X-ray were normal.[neurologyindia.com]
  • 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]
  • 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]
Spastic Gait
  • Treatment is based on the signs and symptoms present in each person. 0002015 Hyperreflexia Increased reflexes 0001347 Slow progression Signs and symptoms worsen slowly with time 0003677 Spastic dysarthria 0002464 Spastic gait Spastic walk 0002064 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]
  • 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]
Poor Coordination
  • It causes weakness and stiffness in the arms and legs, a slowed walk, and poor coordination and balance. Speech also becomes slow and slurred. Like ALS, it usually starts in people 40 to 60 years old. The muscles get stiffer and weaker over time.[webmd.com]

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]
  • You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.[rarediseases.info.nih.gov]
  • 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]
  • Treatment [ edit ] Treatment for individuals with PLS is symptomatic. Baclofen and tizanidine may reduce spasticity. Quinine or phenytoin may decrease cramps.[en.wikipedia.org]

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]
  • 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]
  • (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]
  • Case Report Case Report American Journal of Neuroradiology April 2011, 32 (4) E61-E64; DOI: Abstract SUMMARY: PLS is a disease of the UMN, distinguished from ALS in prognosis and absence of LMN signs.[ajnr.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]
  • 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]
  • 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]

Epidemiology

  • 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]
  • Clinically Relevant Anatomy Epidemiology [3] Approximately 2-5% of adults in neuromuscular clinics are diagnosed with PLS. The age of onset is approximately 50 years and older, though a juvenile-onset form of PLS has been identified as well.[physio-pedia.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]
  • Familial ALS is genetically determined, is inherited in an autosomal dominant fashion, and does not follow the epidemiology described above for sporadic ALS.[medicine.yale.edu]
  • 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]
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 The weakness and atrophy of ALS results from progressive degeneration of motor neurons in the spinal cord, brain stem, and motor cortex.[medicine.yale.edu]
  • 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]
  • 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]

Prevention

  • 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]
  • 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]
  • Frequent repositioning of the body, padding to prevent uneven pressure, and prevention of venous stasis in the legs are crucial. Pain management continues to be important. "Head drop" from weak neck extensor muscles may become a major problem.[the-medical-dictionary.com]

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