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]

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]

Mild UMN signs are often present in the lower limbs. [jnnp.bmj.com]

Respiratory onset can present with: Dyspnoea and orthopnoea. [patient.info]

• 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]

  […] wasting and weakness, absent reflexes, loss of weight and muscle twitching. [mndnsw.asn.au]

  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]

  Upper motor neuron degeneration generally causes spasticity (tightness in a muscle), slowness of movement, poor balance and incoordination, while lower motor neuron degeneration causes muscle weakness, muscle atrophy (shrinkage of muscles) and twitching [mda.org]

• Muscle Spasticity

  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]

  […] particular the limbs, where movement becomes more and more difficult The limb muscles begin to shrink and some develop spasticity, where they become stiff Pain in the muscles and joints Any twinges or spasms in the muscles worsen The patient has problems [newhealthadvisor.com]

  Muscles become spastic, spasm and display uncontrollable twitches. [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]

• 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]

  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]

  Examination reveals UMN signs in the legs (weakness, spasticity, pathological reflexes, hyperreflexia), although reflexes may also be brisk in the arms. [clinicalgate.com]

• Bilateral Babinski's Reflex

  The jaw jerk reflex was brisk and the snout reflex was positive. Neither tongue atrophy nor fasciculation were found. Bilateral upper and lower limb weakness with increased bilateral upper limb reflexes and Babinski reflexes were found. [bmcneurol.biomedcentral.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]

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]

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]

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]

[…] 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]

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]

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]

A viral etiology of MND has been hypothesized for over four decades. [annalsofian.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]

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]

The epidemiology of neurologic disease (Chapter 66). In : Joynt RJ, Griggs RC, editors. Clinical Neurology. Lippincott William Wilkins: Philadelphia; 1998. p. 71-7. 24. Scelsa SN, MacGowan DJ, Mitsumoto H, Imperato T, LeValley AJ, Liu MH, et al. [annalsofian.org]

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]

Neurophysiology of spasticity pp 9-63 By Geoff Sheean, Professor Department of Neurosciences, University of California – San Diego Medical Centre San Diego, California, USA https://doi.org/10.1017/CBO9780511544866.003 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]

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]

Physical therapy, occupational therapy, and rehabilitation may help to improve posture, prevent joint immobility, and slow muscle weakness and atrophy. [ninds.nih.gov]