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]

Mimics can be grouped into those presenting with LMN- or UMN-only signs and those with mixed signs. LMN presentations represent the greatest diagnostic challenge. [pn.bmj.com]

• Eye Movements Abnormal

  A variety of eye movement abnormalities, including supranuclear gaze palsy, have also been described in MND, 64 but these are rarely prominent. [pn.bmj.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 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]

  Oral antispastic agents are usually indicated in patients with diffuse or regional muscle spasticity rather than localized muscle spasticity. [cambridge.org]

  Disease of the upper motor neurons causes stiffness of muscles (spasticity), muscle weakness and exaggerated tendon reflexes, such as knee jerks. [theconversation.com]

  Bohannon RW, Smith MB (1987) Interrater reliability of a modified Ashworth scale of muscle spasticity. Phys Ther 67: 206–207. View Article Google Scholar 15. [journals.plos.org]

• Back Pain

  Again the level of the problem can generally be inferred from accompanying symptoms and signs, for example: Back pain and sciatica suggests a root problem Weakness of the biceps with absence of the biceps reflex, with upper motor neurone signs in the [ole.bris.ac.uk]

  Female carriers may manifest more subtle symptoms such as cramps, back pain, or arthralgias. [clinicalgate.com]

• Loss of Attention

  Progressive sensorimotor deficits in the lower extremities with a history of memory loss or “attention deficit disorder” should also prompt testing for adrenomyeloneuropathy, as should a history of idiopathic childhood epilepsy or primary adrenal failure [clinicalgate.com]

• Hyperreflexia

  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]

  […] brain injury, spinal cord injury Polio, Guillain Barre, Peripheral nerve injury, Peripheral neuropathy, Radiculopathy Tone Increased: Hypertonia, velocity dependent Decreased or absent: hypotonia, flaccidity, not velocity dependent Reflexes Increased: hyperreflexia [physiotherapy-treatment.com]

• 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]

• Bilateral Babinski's Reflex

  Concurrently, hyperreflexia of the jaw jerk and bilateral upper limb reflexes were normalized, and the bilateral Babinski reflexes became negative. Gaze paretic nystagmus and finger-to-nose incoordination were also improved. [bmcneurol.biomedcentral.com]

• Forgetful

  Patients may experience cognitive changes, for example trouble learning new things, or forgetting facts. They may also have problems with concentration and language. A small number of MND patients also develop dementia. [newhealthadvisor.com]

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]

Clinical trials test new treatments on patients and compare the results with standard treatments. Your medical team can discuss any clinical trial options with you. [brainandspine.org.uk]

They are working towards finding new treatments. [medicalnewstoday.com]

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]

[…] 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]

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]

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]

It is wise to get prepared for all possible scenarios when the patient has a poor prognosis, as the patient’s quality of life should still be honored. [healthguidance.org]

Etiology The exact etiology is unknown. [orpha.net]

Introduction to the disease Motor neuron disease (MND) or amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease of unknown etiology. [explainmedicine.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]

Primary lateral sclerosis The etiology of primary lateral sclerosis (PLS) is unknown, but it may be similar to that proposed for ALS. [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]

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]

Epidemiologic data suggest that smoking may be an established risk factor for sporadic ALS. [8] The peak age of onset is between 55 and 75 years. [emedicine.medscape.com]

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]

The present review will examine LMN syndromes from a clinical perspective as well as providing an overview of current understanding of pathophysiological mechanisms. [jnnp.bmj.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]

[…] range of functional problems. 2 - Neurophysiology of spasticity pp 9-63 By Geoff Sheean, Professor Department of Neurosciences, University of California – San Diego Medical Centre San Diego, California, USA Export citation View abstract Summary The pathophysiology [cambridge.org]

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]

Your best chance to prevent the condition is by removing as much as toxins in your environment as possible, improving hygiene and adopting a healthy lifestyle. [healthguidance.org]

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]

Physical, speech and occupational therapy may help improve posture, prevent joint immobility, slow muscle weakness and atrophy, and cope with swallowing difficulties. [health24.com]