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Atypical Motor Neuron Disorders

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Neuromuscular Disorders in Clinical Practice

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Abstract

Amyotrophic lateral sclerosis is a progressive neurodegenerative disorder of motor neurons and their axons characterized by signs of upper and lower motor neuron dysfunction. When clinical features are restricted to either upper or lower motor neurons, limited to one or two body regions, or associated with non-motor manifestations, it is important to consider atypical motor neuron disorders. These are a group of disorders with varied etiologies that resemble amyotrophic lateral sclerosis in many respects but may possess distinct clinical, pathologic, and laboratory features. Some of these disorders are potentially reversible or have a better prognosis than amyotrophic lateral sclerosis. Recognizing these disorders early is necessary in order to initiate specific treatments when available and appropriately counsel patients particularly in the setting of genetic disorders.

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Authors and Affiliations

  1. Department of Neurological Sciences, University of Nebraska Medical Center, Neurology Section, VA Nebraska Western Iowa Health Care System, 982045 Nebraska Medical Center, Omaha, NE, 68198-2045, USA

    J. Americo M. Fernandes Filho MD

  2. Neurology Section, VA Nebraska-Western Iowa Health Care System, 4101 Woolworth Ave, Omaha, NE, 68105, USA

    J. Americo M. Fernandes Filho MD

  3. Department of Neurology, Neuromuscular Immunopathology Research Laboratory, Baylor College of Medicine, One Baylor Plaza, Mailstop NB302, Houston, TX, 77030-3411, USA

    Eroboghene E. Ubogu MD

Authors
  1. J. Americo M. Fernandes Filho MD
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  2. Eroboghene E. Ubogu MD
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Correspondence to J. Americo M. Fernandes Filho MD .

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Editors and Affiliations

  1. School of Medicine Department of Neurology, University Hospitals Case Medical Center Case Western Reserve University, Cleveland, Ohio, USA

    Bashar Katirji

  2. Department of Neurology, George Washington University, Washington, District of Columbia, USA

    Henry J. Kaminski

  3. University School of Medicine Department of Neurology, Louis Stokes Cleveland VA Medical Center Case Western Reserve, Cleveland, Ohio, USA

    Robert L. Ruff

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Filho, J.A.M.F., Ubogu, E.E. (2014). Atypical Motor Neuron Disorders. In: Katirji, B., Kaminski, H., Ruff, R. (eds) Neuromuscular Disorders in Clinical Practice. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-6567-6_22

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