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Motor Neuron Disorders

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Clinical Handbook of Neuromuscular Medicine

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Abstract

ALS is the most common adult-onset motor neuron disorder. ALS affects upper and lower motor neurons, resulting in a combination of spasticity, hyperreflexia, atrophy, and weakness. SMA is the most common childhood-onset motor neuron disorder and is an autosomal recessive disorder of the SMN gene. Other less common motor neuron disorders include SBMA, PLS, Hirayama disease, and viral poliomyelitis. The differential diagnosis and management of motor neuron disorders are discussed in this chapter.

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Abbreviations

ALS:

Amyotrophic lateral sclerosis

EMG:

Electromyography

FTD:

Frontotemporal dementia

FUS:

Fused in sarcoma protein

IBM:

Inclusion body myositis

MRI:

Magnetic resonance imaging

MMN:

Multifocal motor neuropathy

NCS:

Nerve conduction studies

PLS:

Primary lateral sclerosis

SBMA:

Spinobulbar muscular atrophy

SMA:

Spinal muscular atrophy

SOD1:

Superoxide dismutase

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Authors and Affiliations

  1. Department of Neurology, University of Minnesota, Minneapolis, MN, USA

    David Walk

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  1. David Walk
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Correspondence to David Walk .

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  1. Department of Neurology, University of Minnesota, Minneapolis, Minnesota, USA

    David Walk

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Walk, D. (2018). Motor Neuron Disorders. In: Walk, D. (eds) Clinical Handbook of Neuromuscular Medicine . Springer, Cham. https://doi.org/10.1007/978-3-319-67116-1_4

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  • DOI: https://doi.org/10.1007/978-3-319-67116-1_4

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-67115-4

  • Online ISBN: 978-3-319-67116-1

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