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Harrison's Principles of Internal Medicine, 18e
| Part 17. Neurologic Disorders > Section 2. Diseases of the Central Nervous System > |
Chapter 374. Amyotrophic Lateral Sclerosis and Other Motor Neuron Diseases
Sections: Amyotrophic Lateral Sclerosis, Other Motor Neuron Diseases, Further Readings.
Topics Discussed: amyotrophic lateral sclerosis; motor neuron disease.
Excerpt:"Amyotrophic lateral sclerosis (ALS) is the most common form of progressive motor neuron disease. It is a prime example of a neurodegenerative disease and is arguably the most devastating of the neurodegenerative disorders.The pathologic hallmark of motor neuron degenerative disorders is death of lower motor neurons (consisting of anterior horn cells in the spinal cord and their brainstem homologues innervating bulbar muscles) and upper, or corticospinal, motor neurons (originating in layer five of the motor cortex and descending via the pyramidal tract to synapse with lower motor neurons, either directly or indirectly via interneurons) (Chap. 22). Although at its onset ALS may involve selective loss of function of only upper or lower motor neurons, it ultimately causes progressive loss of both categories of motor neurons. Indeed, in the absence of clear involvement of both motor neuron types, the diagnosis of ALS is questionable...."
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