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.
Neurocutaneous Syndromes With Interstitial Lung Disease
Lourdes R. Laraya-Cuasay, Walter T. Hughes in Interstitial Lung Diseases in Children, 2019
Diffuse cerebral atrophy and dilatation of the ventricular system may show on pneumoencephalography.48,49 Anterior horn cell disease is suggested by fibrillation potentials demonstrated by electromyograms.50 Loss of Purkinje and granular cell layer in the cerebellum,49 evidence of anterior horn cell degeneration and posterior column demyelination,47 skeletal muscle degeneration, degeneration of the acidophilic cells of the pituitary gland,51 and rare thalamic and hypothalamic lesions have all been described.
Neurology and neurosurgery
Jagdish M. Gupta, John Beveridge in MCQs in Paediatrics, 2020
The Duchenne and Becker types of muscular dystrophy are both sex-linked recessive. Fasciculations are characteristic of anterior horn cell disease. In peripheral neuropathies there is weakness and sensory loss distally. In spinal muscular atrophy, electromyography shows evidence of denervation of muscle including low potentials and fasciculations. Nerve conduction studies are within the normal range. In Duchenne muscular dystrophy the serum creatine kinase level is raised at birth and is 10 times the normal value at the age of 1 month.
Ventral longitudinal intraspinal fluid collection: Rare presentation as brachial amyotrophy and intracranial hypotension
Published in The Journal of Spinal Cord Medicine, 2019
Veeramani Preethish-Kumar, Seena Vengalil, Sarbesh Tiwari, Kiran Polavarapu, M Netravathi, Aravinda Hanumanthapura Ramalingaiah, Atchayaram Nalini
Patient was initially evaluated by the neurosurgeons for cervical compressive myelopathy and he underwent X-rays and contrast MRI of the spine. Subsequently he was evaluated by the neurologist for anterior horn cell disease. Routine biochemical tests were normal. Nerve conduction studies included motor and sensory conductions of median, ulnar, common peroneal and sural nerves. A comprehensive concentric needle electromyography was performed from bilateral Abductor Digiti Minimi (ADM), biceps, triceps, right vastus lateralis and right tibialis anterior. The electrophysiological findings are summarized in Table 1.
Cell cycle deregulation in neurodegenerative diseases
Published in International Journal of Neuroscience, 2023
Xiaobo Zhang, Shuxin Song, Wenpeng Peng
ALS, the most common form of human motor neuron disease, is exemplified by neuronal loss in the motor cortex, brainstem, and spinal cord ventral horn. This progressive neurodegeneration results in limb weakness, muscle atrophy, paralysis, and respiratory failure, even death [26]. Just like other neurodegenerative disease, its mechanism contains a lot. Recent studies also focus on cell cycle reentry. During ALS, excess excitotoxins, accumulation of oxidative free radicals, or damage to DNA in motor neurons may cause an aberrant cell cycle [50].
Validation of discharge diagnosis coding for amyotrophic lateral sclerosis in an Italian regional healthcare database
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2020
Francesca Palese, Federica Edith Pisa
We identified 1559 records with code for ALS (335.20) and 467 with code for other anterior horn cells diseases (335.X) (Table 1). Complete clinical documentation was retrieved for 1985 records (98.0%), which were considered in the analysis, while it was unavailable for 41 (2.0%, 40 records coded as ALS and one record coded as pseudobulbar palsy).
Related Knowledge Centers
- Dysphagia
- Motor Neuron
- Muscle Atrophy
- Muscle Weakness
- Spasticity
- Dysarthria
- Skeletal Muscle
- Neurodegenerative Disease
- Motor Neuron Diseases
- Fasciculation