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]

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

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

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]

Fronto - temporal dementia A lady presents with her husband. [brainscape.com]

• Weakness

  […] due to weakness of ventilatory muscles.[5]. [explainmedicine.com]

  […] and tongue; and weak palatal movement. [msdmanuals.com]

  Symptoms include pharyngeal muscle weakness (muscles that are involved in swallowing), weak facial muscles, progressive loss of speech, and tongue muscle atrophy. [advancedpsy.com]

  Are characterised by increased muscle tone (spasticity), weakness (generally flexors weaker than extensors in the legs and the reverse in the arms - pyramidal pattern of weakness), increased reflexes, an up-going plantar response and sustained clonus [ole.bris.ac.uk]

• Pain

  Also pain due to nerve affection may occur in some patients with ALS. 4, 5, 6, 7 Fasciculations are a common symptom of ALS. These persistent muscle twitches are generally not painful but can interfere with sleep. [mda.org]

  A comparative trial of botulinum toxin type A and methylprednisolone for the treatment of myofascial pain syndrome and pain from chronic muscle spasm. Pain 2000;85: 101-105. ‎ [books.google.de]

  Pain A non-steroidal anti-inflammatory drug (NSAID), such as ibuprofen, will help with mild to moderate pain from muscle cramping as spasms. Drugs such as morphine can help relieve severe joint and muscle pain in the advanced stages. [medicalnewstoday.com]

  In the initial stage, besides the previously mentioned signs, there is also: Clumsiness Fatigue Pain in the muscles Cramps Twitching Slurred speech Weak grip In advanced stages of disease, there is: Muscle weakness Pain in muscles and joints Worsening [medicalfoster.com]

  Symptoms Initial stage: Clumsiness Tiredness Muscular pain Cramps A weakening of grip, so that picking up or holding objects may become hard Twitching Slurred or difficult to understand speech Limb weakness Advanced stage: Weakness in the muscles, in [newhealthadvisor.com]

• Rigor

  Improved understanding of the multiple features of the upper motor neuron syndrome supports more rigorous assessment, and improved treatment planning. [en.wikipedia.org]

  Nevertheless, our aim is to faithfully present in scope and rigor a medical school caliber course experience. [coursera.org]

  Because of the loss of muscle bulk through atrophy, rigor mortis is usually absent in advanced stages of ALS disease. [emedicine.medscape.com]

• Pneumonia

  Affected individuals have an increased risk of choking, and aspiration pneumonia. They are also affected by emotional lability (laughing or crying outbursts). Most ALS-affected persons show progressive bulbar palsy symptoms. [can.ubc.ca]

  Pneumonia due to infection or aspiration. Urinary tract infections. Constipation. Spasticity and cramping of muscles. Depression Loss of speech as a means of communication. Immobility and attendant disability. [patient.info]

  They eventually become unable to eat or speak and are at increased risk for choking and aspiration pneumonia. c. [advancedpsy.com]

  Following recovery from pneumonia, he started antiretroviral therapy with zidovudine, lamivudine, lopinavir and ritonavir [Figure - 1]. In December 2001, he returned to work full-time. [annalsofian.org]

  […] sensory nerves are not typically affected. 5 Bulbar-onset disease may be evident in 20-25% of patients, characterised by progressive dysarthria, dysphagia, hoarseness, tongue wasting, weakness and fasciculations as well as emotional lability. 2 Aspiration pneumonia [acnr.co.uk]

• Dyspnea

  Respiratory dysfunction Progressive respiratory dysfunction may resulted due to respiratory muscle weakness due to loss of motor neurons in spinal cord.It may also causes decreased voluntary and reflexive coughing as well.[4] Dyspnea Not a prominent symptom.Occurs [explainmedicine.com]

  Dyspnea-fasciculation syndrome: early respiratory failure in ALS with minimal motor signs. Amyotroph Lateral Scler Other Motor Neuron Disord 2002;3:239-43. Czaplinski A, Strobel W, Gobbi C, Steck AJ, Fuhr P, Leppert D. [acnr.co.uk]

• Dysphagia

  Dysarthria and dysphagia are found in various neurological diseases. Case presentation We report a 51-year-old man with chronic alcoholism and malnutrition who progressively developed dysarthria and dysphagia. [bmcneurol.biomedcentral.com]

  Patients usually present with facial weakness, dysphagia, and dysarthria. [msdmanuals.com]

  Dysphagia A manifestation of bulbar palsy mainly affecting the lower cranial nerve nuclei.[2]. LMN dysfunction causes a weakened tongue.It interferes with the formation of a bolus or a gutter for liquids. [explainmedicine.com]

  For dysphagia (difficulties swallowing): Many MND patients have problems eating and drinking, particularly in the later stages of the disease. [newhealthadvisor.com]

  A brain stem lesion may also cause dysarthria, dysphagia, Horner's syndrome, cerebellar signs, spinothalamic sensory loss. [ole.bris.ac.uk]

• Fasciculation of the Tongue

  Fasciculations of the tongue, which are a prominent feature of SMA, are helpful in making this distinction. [neuropathology-web.org]

  Bulbar onset: The first sign is usually slurring of the speech (impaired tongue movement). Wasting and fasciculation of the tongue. Dysphagia (usually a late feature with significant speech difficulties). [patient.info]

  In the presence of a progressive pure motor disorder, signs such as florid fasciculations, bilateral tongue wasting, the ‘split hand’, head drop, emotionality, and cognitive or behavioural impairment carry high positive predictive value. [pn.bmj.com]

• Tongue Atrophy

  Neither tongue atrophy nor fasciculation were found. The tongue could be protruded from the mouth and remained midline, and moved adequately from side to side. [bmcneurol.biomedcentral.com]

  When first seen at our Center in June 2002, he had moderate dysarthria and mild dysphagia, tongue atrophy and fasciculations and a positive jaw jerk and pout reflex. [annalsofian.org]

• Muscle Weakness

  Progressive muscle weakness in vouluntary muscles A rare neuromuscular disorder that causes progressive weakening and wasting of the muscles in the arms and legs in particular. [brainscape.com]

  […] due to weakness of ventilatory muscles.[5]. [explainmedicine.com]

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

  In the initial stage, besides the previously mentioned signs, there is also: Clumsiness Fatigue Pain in the muscles Cramps Twitching Slurred speech Weak grip In advanced stages of disease, there is: Muscle weakness Pain in muscles and joints Worsening [medicalfoster.com]

• Muscle Cramp

  However unlike muscle cramps, fatigue is not a prominent early symptom.[5]. [explainmedicine.com]

  Symptoms may include muscle wasting, clumsy hand movements, fasciculations and muscle cramps. The trunk muscles and respiration may be affected and the disease develops into ALS in many patients. f. [advancedpsy.com]

  For muscle cramps and stiffness: MND patients suffering from muscles cramps and stiffness can be treated with physical therapy or medication. [newhealthadvisor.com]

  Muscle cramps and stiffness Muscle cramps and stiffness can be treated with physical therapy and medications, such botulinum toxin (BTA) injections. BTA blocks the signals from the brain to the stiff muscles for about 3 months. [medicalnewstoday.com]

  Motor neuron disease has different symptoms, however it can be different based on the type: • Emotional changes • Difficulty breathing, swallowing or speaking • Fasciculation (muscle twitching) • Muscles cramping or stiffness • Fatigue • Poor muscle coordination [healthguidance.org]

• 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]

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

  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]

• Myalgia

  Myalgias and muscle spasm are often prominent prior to onset of weakness. Bulbar and respiratory involvement may occur. PCR of the virus from cerebrospinal fluid is the gold standard for confirming the diagnosis. [jnnp.bmj.com]

  Postpolio syndrome Most patients with postpolio syndrome (PPS) present with new, slowly progressive muscle weakness, frequently accompanied by muscle pain (myalgias) and fatigue, which can occur in both previously affected and unaffected muscles. [emedicine.medscape.com]

• Stroke

  Normally, in response to stroking of the sole of the foot, the big toe (often also other toes) will flex. If the descending pathways are damaged, the stroke of the foot will make the big toe extend and the other toes to flex. [medicalfoster.com]

  Snapshot A 62-year-old man is in the stroke unit after experiencing a severe right-sided middle cerebral artery stroke. On physical exam there is left-sided upper extremity muscle weakness. [medbullets.com]

  UMNL neurological lesion involving any part of an upper motor neurone, e.g. stroke, cerebral palsy; characterized by clonus, exaggerated tendon reflexes, extensor plantar response, increased voluntary muscle tone and soft-tissue contracture [medical-dictionary.thefreedictionary.com]

  A possible diagnosis is a lesion affecting the right cerebral cortex or cerebrospinal tract, such as a stroke. Perfect revision for medical student finals, OSCES and PACES [oxfordmedicaleducation.com]

  Neuron Lesion Location of lesion; Structures involved Central nervous system- cortex, brainstem, corticospinal tracts, spinal cord Cranial nerve nuclei/nerves and Spinal cord: anterior horn cell, spinal roots, peripheral nerve Diagnosis or Pathology Stroke [physiotherapy-treatment.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]

  The lower motor neuron involvement in disease can cause LMN manifestations such as fasciculation, atrophy, weakness, and hyporeflexia, Upper motor neurone involvement will cause UMN symptoms and signs such as spasticity, weakness and hyperreflexia where [explainmedicine.com]

• Dysarthria

  Dysarthria and dysphagia are found in various neurological diseases. Case presentation We report a 51-year-old man with chronic alcoholism and malnutrition who progressively developed dysarthria and dysphagia. [bmcneurol.biomedcentral.com]

  Dysarthria Progressive bulbar palsy may manifest as progressive disturbance of speech lead in to dysarthria .[2]. The mechanism of dysarthria is a flaccid or spastic paresis of the musculature of the face, tongue, lips, palate, pharynx and larynx. [explainmedicine.com]

  A brain stem lesion may also cause dysarthria, dysphagia, Horner's syndrome, cerebellar signs, spinothalamic sensory loss. [ole.bris.ac.uk]

  Symptoms include weakness of the facial and tongue muscles, hand tremor, muscle cramps, dysphagia, dysarthria and gynecomastia. Weakness usually begins in the pelvis before spreading to the legs. [advancedpsy.com]

  Symptoms for diagnosis include dysarthria (Speech problems from effect on tongue muscles), dysphagia (difficulty swallowing due to effect on responsible muscles) References: Blackstone, C., O’Kane, C. and Reid, E. (2011). [seriouslysciatic.wordpress.com]

• Tremor

  For example, the thumb and forefingers will move back-and-forth against each other in a characteristic tremor called “pill-rolling tremor.” The tremor stops when the body part engages in active movement. [nba.uth.tmc.edu]

  Tremors which are severe on sustained postures (such as with the hands outstretched), but which may worsen slightly with action are characteristic of essential tremor (this is also seen in “senile” tremor or familial tremor). [dartmouth.edu]

  Symptoms may include hypotonia, diminished limb movements, lack of tendon reflexes, fasciculations, tremors, swallowing and feeding difficulties and impaired breathing. [advancedpsy.com]

  Additional rare manifestations include worsening of symptoms with exposure to cold (cold paresis), muscle cramps, cold hands, irregular coarse tremor and/or contraction fasciculations. [orpha.net]

  Symptoms may include hypotonia (severely reduced muscle tone), diminished limb movements, lack of tendon reflexes, fasciculations, tremors, swallowing and feeding difficulties, and impaired breathing. [ninds.nih.gov]

• Paresis

  In summary she had a spastic paresis of the left leg with increased reflexes. These signs are consistent with a right-sided upper motor lesion. [oxfordmedicaleducation.com]

  Additional rare manifestations include worsening of symptoms with exposure to cold (cold paresis), muscle cramps, cold hands, irregular coarse tremor and/or contraction fasciculations. [orpha.net]

  雑誌 Dev Med Child Neurol 52:27-32 (2010) DOI: 10.1111/j.1469-8749.2009.03475.x 文献 PMID: 3955865 著者 Patton MA, Baraitser M, Brett EM タイトル A family with congenital suprabulbar paresis (Worster-Drought syndrome). [kegg.jp]

  The mechanism of dysarthria is a flaccid or spastic paresis of the musculature of the face, tongue, lips, palate, pharynx and larynx. At the early stage of ALS patient may experience a mild change in his voice or speech. [explainmedicine.com]

  Hemiparesis/plegia is paralysis or paresis (weakness) of one side of the body. [dartmouth.edu]

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]

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]

[…] 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]

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]