The nervous system
Laurie K. McCorry, Martin M. Zdanowicz, Cynthia Y. Gonnella in Essentials of Human Physiology and Pathophysiology for Pharmacy and Allied Health, 2019
ALS is also known as Lou Gehrig’s disease after the hall of fame New York Yankees’ baseball player who was afflicted with this condition. ALS is a progressive neurologic condition that primarily affects motor neurons in the anterior (motor) horns of the spinal cord. The term “amyotrophy” refers to the degeneration and shrinkage of motor neurons and subsequent wasting of skeletal muscle that occurs as a result of nervous innervation. The “sclerosis” in the name refers to the hardened or scarred appearance of the neurons at autopsy. ALS affects approximately 5,600 individuals each year in the United States. The average age of onset is 55 and the disease is more prevalent in men than women. Although the exact cause of ALS is uncertain, 5%–10% of cases are inherited. The remaining percentage of non-inherited cases may be possibly related to inappropriate immune response, excess levels of excitatory neurotransmitters such as glutamate, or the accumulation of abnormal proteins in nerve cells. There is currently no cure for ALS, and the average life expectancy after diagnosis is generally 3–5 years. The most common cause of death for patients with ALS is respiratory failure.
Cletus
Walter J. Hendelman, Peter Humphreys, Christopher R. Skinner in The Integrated Nervous System, 2017
ALS is a progressive degenerative disorder affecting both the upper and lower motor neurons in combination and at varying intensities. It is important to understand the term ALS. Amyotrophy (A) refers to muscle weakness not due to muscle disease. This represents the lower motor neuron component of the damage caused by ALS. Fasciculations are involuntary muscle movements that resemble a ‘bag of worms’ in multiple muscle groups. The presence of fasciculations is a cardinal sign that muscles have undergone denervation and reinervation secondary to progressive damage to lower motor neurons in the spinal cord or brainstem. The observation of fasciculations in the tongue is very significant in that it shows lower motor neuron damage located above the level of the cervical spine. The lateral sclerosis (LS) part of the name refers to the lateral columns or lateral funiculi in which the corticospinal tracts run. Therefore, the term ALS refers to both upper and lower motor neuron damage all in one acronym. The finding of an exaggerated jaw jerk is often present in ALS indicating bilateral upper motor neuron damage to corticobulbar tract affecting control of motor neurons in the trigeminal motor nucleus of the pons.
Dysfunctions of COVID-19
Wenguang Xia, Xiaolin Huang in Rehabilitation from COVID-19, 2021
The inevitable long-term bed rest and inactivity of COVID-19 patients during the course of the disease is a major cause of physical dysfunction. Its symptoms are malaise, easy fatigue, muscle soreness, and palpitate and some patients have amyotrophy and decrease of muscle strength. At the same time, in addition to the psychological stress and trauma brought by the disease, there are also physical symptoms closely related to psychological factors, such as insomnia, fatigue, palpitation, chest tightness, dysphagia, urinary frequency, and so on. These physical symptoms may involve nervous, circulatory, digestive, respiratory, urogenital, endocrine, motor, and other systems. Therefore, in the assessment of patients’ physical dysfunction, the whole psychological and physiological analyses should be carried out, and careful observation should be made to identify whether physiological factors or psychological factors are dominant, which guides the formation of the rehabilitation treatment plan. The more common physical dysfunctions in COVID-19 patients during the convalescent period are discussed below.
Co-occurrence of MS and ALS: a clue in favor of common pathophysiological findings?
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2018
Anne-marie Guennoc, Maud Pallix-guyot, Emmanuelle Le Page, Damien Le Port, Mathieu Daryabin, Rudolph Hergesheimer, Stéphane Beltran, Ayman Tourbah, Gilles Edan, Philippe Corcia
A 60-year-old woman had a 30-year history of MS diagnosed after an episode of right optic neuritis, which improved after pulses of corticoids without sequela. Over the next 30 years, she complained of transient sensory defects in the lower limbs treated by pulses of Solumedrol. The current neurological history started nine months ago with the occurrence of gait disturbances due to left leg weakness. At the first visit, she was unable to walk without aid resulting from weakness and amyotrophy affected upper limbs during the three prior months. Neurological examination showed diffuse motor weakness in the four limbs. Amyotrophy was identified in the legs and arms in distality. DTRs were all brisk. Fasciculations were noted in the arms, legs, and tongue. There were no sensory, sphincter, cerebellar or cognitive abnormalities. Cerebral and spinal MRIs did not reveal active plaques or increases in the lesion burden. The disease continued to progress until death by respiratory failure after 22 months of progression (Figure 1).
RNA therapeutics for mood disorders: current evidence toward clinical trials
Published in Expert Opinion on Investigational Drugs, 2021
Marguerite Le Marois, Eleni Tzavara, El Chérif Ibrahim, Olivier Blin, Raoul Belzeaux
Since the first licensed RNA therapeutic (fomivirsen), approved by Food and Drug Administration in 1998 and by European Agency for the Evaluation of Medicinal Products in 1999, eleven RNA therapeutics were approved in the US or the EU, and eight of them are currently marketed [61]. Current marketing statutes were checked using the Drugs@FDA and European Medicine Agency online databases on 11 December 2020. Most target rare, gene-specific related diseases; only one is used for the treatment of a neurological disorder – spinal muscular amyotrophy- through intrathecal administration. None is used for mood disorders or other psychiatric disorders.
Reflections of patients and therapists on a multidisciplinary rehabilitation programme for persons with brachial plexus injuries
Published in Disability and Rehabilitation, 2019
Renske M. J. Janssen, Ton Satink, Jos Ijspeert, Nens van Alfen, Jan T. Groothuis, Tanya L. Packer, Edith H. C. Cup
Persons with neuralgic amyotrophy (NA) experience severe pain, fatigue, and problems in performing movements of the upper extremity [1]. NA limits forward reaching, lifting the arms above shoulder height, maintaining static arm positions, and repetitive movements. The combination of limited movement, pain, and fatigue compromise the performance of many activities and roles in daily life, making NA a very debilitating condition.
Related Knowledge Centers
- Differential Diagnosis
- Muscle
- Type 2 Diabetes
- Als
- Proximal Diabetic Neuropathy
- Monomelic Amyotrophy