Ectopic impulse generation in peripheral nerve hyperexcitability syndromes and amyotrophic lateral sclerosis. 2018

Yu-Ichi Noto, and Neil G Simon, and Alexis Selby, and Nidhi Garg, and Kazumoto Shibuya, and Nortina Shahrizaila, and William Huynh, and José M Matamala, and Thanuja Dharmadasa, and Susanna B Park, and Steve Vucic, and Matthew C Kiernan
Brain and Mind Centre, Sydney Medical School, University of Sydney, Sydney, NSW, Australia.

To elucidate differences in the distribution and firing frequency of fasciculations between peripheral nerve hyperexcitability syndromes and amyotrophic lateral sclerosis (ALS) and to explore the generator site of fasciculations. Ultrasound of 14 preselected muscles was performed in patients with peripheral hyperexcitability and ALS. The distribution and firing frequency of fasciculations were calculated. Cortical excitability assessment was also done by threshold tracking transcranial magnetic stimulation. In total, 518 muscles from 37 peripheral hyperexcitability patients and 756 muscles from 54 ALS patients were examined. Regarding the detection rate, 74% of muscles in ALS patients demonstrated fasciculations, compared with 34% of muscles in peripheral hyperexcitability patients (P < 0.001). The number of unique repeating focal muscle fasciculation movements per muscle and firing frequency of individual fasciculations in ALS were both significantly higher than those in peripheral hyperexcitability (P < 0.001). Furthermore, cortical silent period duration negatively correlated with the number and firing frequency of fasciculations in ALS (P < 0.05). A similar relationship was not evident in peripheral hyperexcitability. In ALS patients, fasciculations were more widespread, greater in number and higher in firing frequency than in peripheral hyperexcitability patients. A significant proportion of fasciculations in ALS may be influenced by changes in central excitability.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D009046 Motor Neurons Neurons which activate MUSCLE CELLS. Neurons, Motor,Alpha Motorneurons,Motoneurons,Motor Neurons, Alpha,Neurons, Alpha Motor,Alpha Motor Neuron,Alpha Motor Neurons,Alpha Motorneuron,Motoneuron,Motor Neuron,Motor Neuron, Alpha,Motorneuron, Alpha,Motorneurons, Alpha,Neuron, Alpha Motor,Neuron, Motor
D010523 Peripheral Nervous System Diseases Diseases of the peripheral nerves external to the brain and spinal cord, which includes diseases of the nerve roots, ganglia, plexi, autonomic nerves, sensory nerves, and motor nerves. Peripheral Nerve Diseases,Peripheral Neuropathies,PNS (Peripheral Nervous System) Diseases,PNS Diseases,Peripheral Nervous System Disease,Peripheral Nervous System Disorders,Nerve Disease, Peripheral,Nerve Diseases, Peripheral,Neuropathy, Peripheral,PNS Disease,Peripheral Nerve Disease,Peripheral Neuropathy
D004576 Electromyography Recording of the changes in electric potential of muscle by means of surface or needle electrodes. Electromyogram,Surface Electromyography,Electromyograms,Electromyographies,Electromyographies, Surface,Electromyography, Surface,Surface Electromyographies
D005207 Fasciculation Involuntary contraction of the muscle fibers innervated by a motor unit. Fasciculations may be visualized as a muscle twitch or dimpling under the skin, but usually do not generate sufficient force to move a limb. They may represent a benign condition or occur as a manifestation of MOTOR NEURON DISEASE or PERIPHERAL NERVOUS SYSTEM DISEASES. (Adams et al., Principles of Neurology, 6th ed, p1294) Fasciculation, Benign,Fasciculation, Muscular,Fasciculation, Skeletal Muscle,Fasciculation, Tongue,Benign Fasciculation,Benign Fasciculations,Fasciculations,Fasciculations, Benign,Fasciculations, Muscular,Fasciculations, Skeletal Muscle,Fasciculations, Tongue,Muscle Fasciculation, Skeletal,Muscle Fasciculations, Skeletal,Muscular Fasciculation,Muscular Fasciculations,Skeletal Muscle Fasciculation,Skeletal Muscle Fasciculations,Tongue Fasciculation,Tongue Fasciculations
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000071080 Cortical Excitability Measurable changes in activities in the CEREBRAL CORTEX upon a stimulation. A change in cortical excitability as measured by various techniques (e.g., TRANSCRANIAL MAGNETIC STIMULATION) is associated with brain disorders. Cortical Excitabilities,Excitabilities, Cortical,Excitability, Cortical
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults

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