Synonyms
Familial spastic paraplegia; Strümpell disease
Definition
The principal neurological pathway over which signals pass to drive a deliberate movement can be divided into 2 stages. In the first stage, a descending connection is made from neurons in the motor cortex of the brain (often referred to clinically as “upper motor neurons”) to cells in the anterior and posterior horns of the spinal cord, while in the second stage “lower motor neurons” in the anterior horn of the spinal cord connect to the skeletal muscles at the neuromuscular junction. The axons that project from the upper motor neuron cell bodies to the spinal cord constitute the corticospinal tract and this tract is “crossed”, so that upper motor neurons in the motor cortex of one side innervate lower motor neurons on the opposite side of the spinal cord. A wide variety of insults to the corticospinal tract result in spastic paralysis. The hereditary spastic paraplegias (HSPs) are single gene disordersin which the...
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References
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© 2005 Springer-Verlag
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Reid, E. (2005). Hereditary Spastic Paraplegia. In: Encyclopedic Reference of Genomics and Proteomics in Molecular Medicine. Springer, Berlin, Heidelberg . https://doi.org/10.1007/3-540-29623-9_1330
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DOI: https://doi.org/10.1007/3-540-29623-9_1330
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-540-44244-8
Online ISBN: 978-3-540-29623-2
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