Chapter 188: ALS and Other Motor Neuron Diseases

Amyotrophic lateral sclerosis (ALS) is the most common form of progressive motor neuron disease (Table 188-1). ALS is caused by degeneration of motor neurons at all levels of the CNS, including anterior horns of the spinal cord, brainstem motor nuclei, and motor cortex. Familial ALS (FALS) represents 5–10% of the total and is inherited usually as an autosomal dominant disorder.

TABLE 188-1: Sporadic Motor Neuron Diseases
CHRONIC	ENTITY
Upper and lower motor neuron	Amyotrophic lateral sclerosis
Predominantly upper motor neuron	Primary lateral sclerosis
Predominantly lower motor neuron	Multifocal motor neuropathy with conduction block
	Motor neuropathy with paraproteinemia or cancer
	Motor predominant peripheral neuropathies
Other
Associated with other neurodegenerative disorders
Secondary motor neuron disorders (see Table 188-2)
Acute
Poliomyelitis
Herpes zoster
Coxsackie virus
West Nile virus

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