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Chapter 10 - A Phenomenology-Based Approach to Inborn Errors of Metabolism with Spasticity

from Section I - General Principles and a Phenomenology-Based Approach to Movement Disorders and Inherited Metabolic Disorders

Published online by Cambridge University Press:  24 September 2020

Darius Ebrahimi-Fakhari
Affiliation:
Harvard Medical School
Phillip L. Pearl
Affiliation:
Harvard Medical School
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Summary

Spasticity is a motor abnormality characterized by increased muscular tone. It results from a lesion of the corticospinal (or pyramidal) tract, which includes motor neurons in the cerebral cortex and their axonal white matter projections through the brain and spinal cord, to the point of synapse with lower motor neurons in the spinal cord. Patients with spasticity, or their caregivers, typically complain of stiffness, and the predominant associated examination findings are a velocity-dependent resistance to passive movement of an affected limb, hyperreflexia, and pathological reflexes such as a Babinski sign. Spasticity is typically accompanied by weakness and impaired motor control in affected muscle groups. Depending on the site of the lesion in the central nervous system, spasticity may be present in both legs (termed diparesis or paraparesis), all four limbs (quadriparesis or tetraparesis), or one side of the body (hemiparesis).

Type
Chapter
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Movement Disorders and Inherited Metabolic Disorders
Recognition, Understanding, Improving Outcomes
, pp. 143 - 154
Publisher: Cambridge University Press
Print publication year: 2020

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