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Upper Motor Neuron Disease

Motor Neuron Dis Upper


Presentation

  • Fasciculations are present in this motor neuron disease. Atrophy is present in this motor neuron disease. Hypotonic to flaccid tone in this motor neuron disease.[howtorelief.com]
  • Adrenal insufficiency may be present and may predate onset of neurological symptoms by several years. Adult female carriers may present with slowly progressive spastic paraparesis.[clinicalgate.com]
  • The stretching reflexes and muscle response to stretching is also present. If the upper motor neuron lesion is extensive, muscle rigidity in the leg extensors and arm flexor muscles can also be seen.[medicalfoster.com]
  • Cognitive Neuroscience section, we are now extending that work to include comparisons with FTD patients studied with the same methods, with a goal of identifying imaging measures that discriminate between patients with different diagnoses and clinical presentations[grantome.com]
  • Fronto - temporal dementia A lady presents with her husband.[brainscape.com]
Anemia
  • However, the complicated form may also include optic neuropathy, pigmentary retinopathy, deafness, ataxia, ichthyosis, amyotrophy, peripheral neuropathy, dementia, autoimmune hemolytic anemia/thrombocytopenia (Evans syndrome), extrapyramidal dysfunction[clinicalgate.com]
Formication
  • In addition to spastic paraparesis, sensory (including leg formications) and bladder dysfunction may occur. Occasionally there is a coarse tremor of the upper extremities.[clinicalgate.com]
Muscle Twitch
  • Lower motor neuron disease causes weakness, loss of muscle ("atrophy") and muscle twitching ("fasciculations"). ALS may begin with abnormalities of upper or lower motor neurons.[neurology.emory.edu]
  • Apraxia (Medical Encyclopedia) Hand or foot spasms (Medical Encyclopedia) Muscle atrophy (Medical Encyclopedia) Muscle function loss (Medical Encyclopedia) Muscle twitching (Medical Encyclopedia) Myotonia congenita (Medical Encyclopedia) Spasticity (Medical[icdlist.com]
  • These persistent muscle twitches are generally not painful, but can be annoying or interfere with sleep. They are the result of the ongoing disruption of signals from the nerves to the muscles that occurs in ALS.[mda.org]
Muscle Spasticity
  • In upper motor neuron (UMN) disease, patients often experience resistance to movement and stiffness (muscle spasticity), over-reactive reflexes, and the Babinski sign, a specific reflex indicating damage to the neural pathways between the brain and spinal[newhealthadvisor.com]
  • Involvement of UMN results in stiffness of muscles (spasticity) and slowed movements. People may have only LMN or UMN signs early in the illness but typically develop a combination of the two as the disease progresses.[mndnsw.asn.au]
  • Disease of the upper motor neurons causes stiffness of muscles (spasticity), muscle weakness and exaggerated tendon reflexes, such as knee jerks.[theconversation.com]
  • The small muscles of the hands and feet are usually first affected, but muscle spasticity is absent. Primary lateral sclerosis - this is another rare type of MND. It mainly causes weakness in the leg muscles.[patient.info]
Frozen Shoulder
  • You Might Also Like: Rotator Cuff vs Frozen Shoulder Lower Motor Neuron Disease Reflexes are diminished or absent in this motor neuron disease. Fasciculations are present in this motor neuron disease. Atrophy is present in this motor neuron disease.[howtorelief.com]
Hyperreflexia
  • Upper Motor Neuron Disease Symptoms The weakness of involved muscles, hyperreflexia, hypertonicity, mild disuse atrophy, and abnormal reflexes; damaged tracts are in the lateral white column of the spinal cord.[howtorelief.com]
  • No spasticity or hyperreflexia.[brainscape.com]
  • Examination reveals UMN signs in the legs (weakness, spasticity, pathological reflexes, hyperreflexia), although reflexes may also be brisk in the arms.[clinicalgate.com]
  • Neuron Lesion findings Muscle group weakness (not complete paralysis) Minimal muscle atrophy "Clasp-knife" spasticity Initial resistance to motion is strongest Once initial resistance gives-way, resistance to motion is less for remaining range of motion Hyperreflexia[fpnotebook.com]
  • […] motor neurons are neurons from the cerebral cortex to the anterior horn Lower motor neurons are neurons from the anterior horn to the peripheral muscle Motor Neuron Signs Type Clinical Features Upper motor neuron signs Weakness Increased muscle tone Hyperreflexia[medbullets.com]
Spastic Gait
  • gait in upper motor neurone hemiplegia scissor gait clasp knife rigidity clonus pronator drift spastic dysarthria Hoffmann's sign[gpnotebook.co.uk]
Spastic Paralysis
  • There are also rare progressive, autosomal recessive upper motor neuron degenerations (Infantile –Onset Ascending Hereditary Spastic Paralysis, Juvenile Primary Lateral Sclerosis, and Juvenile ALS) which begin during childhood and present with spasticity[neuropathology-web.org]
Cognitive Deficit
  • Neuropsychological test batteries may define subtle cognitive deficits due to frontal cortical involvement, but dementia is not a prominent feature. A few patients may exhibit abnormal voluntary eye movements.[clinicalgate.com]

Treatment

  • No treatments are currently available to prevent, stop, or reverse HSP. Please help us raise some money for the Spastic Paraplegia Foundation (SPF) to support HSP research.[gofundme.com]
  • The treatment of upper motor neuron syndrome depends on the underlying condition.[medicalfoster.com]
  • Treatment Options to Slow Down the Progression 1. Treatment to Slow Down Upper Neuron Motor Disease Progression Riluzole is currently the only medication specifically developed to treat MND.[newhealthadvisor.com]
  • Management and treatment There is no cure for MA. Treatment is conservative and with early detection aims to slow the progression of muscle wasting.[orpha.net]
  • […] for depression. [21] The support for exercise in treating these psychological dysfunctions is overwhelming, and should be a major consideration as an all-encompassing treatment strategy for MND/ALS.[en.wikibooks.org]

Prognosis

  • […] hyperintensity in the corticospinal tracts (specificity 6 GRE/SWI: hypointensity in the precentral gyrus bilaterally, known as the " motor band sign " 8,9 MR spectroscopy 2 decreased NAA decreased glutamate increased choline increased myo-inositol Treatment and prognosis[radiopaedia.org]
  • Prognosis of Motor Neuron Disease Prognosis of Motor Neuron Disease varies depending on the type of MND and the age of onset. Some MNDs for example PLS or Kennedy’s disease, are not fatal and progress slowly.[naturalherbsclinic.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]
  • 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 MA is not life threatening but can cause a social disability in those with a complete loss of hand function. Early intervention can minimize progression of disease.[orpha.net]

Etiology

  • Etiology The exact etiology is unknown.[orpha.net]
  • 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]
  • As more is learned about the molecular etiology of HAM/TSP, future therapies will likely target the pathogenic effect of HTLV-1–reactive T cells.[clinicalgate.com]
  • Introduction to the disease Motor neuron disease (MND) or amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease of unknown etiology.[explainmedicine.com]

Epidemiology

  • Summary Epidemiology The prevalence is unknown. It is seen mainly in Asian countries (particularly in Japan and India) with only a very few cases reported in Europe and the United States.[orpha.net]
  • There may be several causes for such oxidative damage to motor neurons and the disease may just represent an end-stage phenotypic expression of these abnormalities. [ 4 ] Epidemiology MND is relatively uncommon with an annual incidence of about 2 cases[patient.info]
Sex distribution
Age distribution

Pathophysiology

  • Within this category there are some specific pathophysiological diseases. For example, spinal muscular atrophy is a genetic disease that results in the death of the lower motor neuron.[theness.com]
  • Advances in treating amyotrophic lateral sclerosis: insights from pathophysiological studies . Trends Neurosci 2014;37:433-42. Brooks BR, Miller RG, Swash M, Munsat TL.[acnr.co.uk]
  • 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

  • No treatments are currently available to prevent, stop, or reverse HSP. Please help us raise some money for the Spastic Paraplegia Foundation (SPF) to support HSP research.[gofundme.com]
  • Hawking is philosophical about having ALS, saying that he tries to have a normal life, and that his condition doesn’t prevent him from achieving great things.[newhealthadvisor.com]
  • A cervical collar worn in the early stages of disease has been shown to halt progress of the disease in some cases, as it prevents neck flexion. Muscle strengthening exercises and hand coordination training can also be helpful.[orpha.net]
  • The drug aims to prevent damage of neurons , and the company that developed it reports it can slow the physical decline of MND patients by 33% .[theconversation.com]

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