Motor Neuron Diseases
In: Electrodiagnostic Medicine ISBN: 9783030749965; (2021)
Online
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Zugriff:
Motor neuron diseases (MND) comprise a group of neurodegenerative conditions that can cause rapidly progressive muscle weakness by causing gradual degeneration of the motor neuron. It can affect both the upper motor neurons (UMN) and/or the lower motor neurons (LMN), depending on the variant type. Amyotrophic Lateral Sclerosis (ALS) belongs to this group of diseases, causing dysfunction of the UMN and LMN. ALS is mostly considered sporadic, but a small percentage (about 5–10%) of cases are familial/genetic, some with suspected association with detrimental environmental contributors. There are other variants like: Primary Lateral Sclerosis (PLS) which affects mostly the UMN, Progressive Muscular Atrophy (PMA) selectively affecting the LMN, and Progressive Bulbar Palsy (PBP) primarily causing damage to the motor neurons of the bulbar muscles. The latter disease can progress to involve other regions including limbs, as seen in ALS, or may remain restricted to the bulbar muscles. There are many other diseases that can manifest with some of the classic symptoms of MND such as: weakness, fasciculations, spasticity, cramps, and pathologic hyperreflexia. It is imperative that the clinician distinguish amongst diseases that can mimic MND, because some can be potentially treated. Electrodiagnostic evaluation (EDX) is key in assisting the clinician with the diagnosis of MND. However, EDX has its pitfalls and limitations, and careful performance and interpretation of the nerve conduction studies (NCS) and needle electromyography (EMG) are important for proper diagnosis. Guidelines have been established for the clinical diagnosis of ALS (e.g Revised El Escorial criteria from the World Federation of Neurology), including EDX criteria for NCS and EMG. We will discuss in detail some of the MND mimics and their clinical features, EDX protocols and limitations, and provide an example case of MND/ALS type.
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Motor Neuron Diseases
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Autor/in / Beteiligte Person: | Dompenciel, Michelle M. |
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Quelle: | Electrodiagnostic Medicine ISBN: 9783030749965; (2021) |
Veröffentlichung: | Springer International Publishing, 2021 |
Medientyp: | unknown |
ISBN: | 978-3-030-74996-5 (print) |
DOI: | 10.1007/978-3-030-74997-2_9 |
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