Which of the following statements about Amyotrophic Lateral Sclerosis (Lou Gehrig’s disease) is true?
A) It is a progressive motor neuron disease that affects the corticospinal tracts.
B) The onset is usually before 20 years of age.
C) It typically destroys sensory function.
D) It may respond to high-dose steroid administration.
E) Dementia is common.
Answer and Discussion
The answer is A. (It is a progressive motor neuron disease that affects the corticospinal tracts)
Amyotrophic Lateral Sclerosis (Lou Gehrig’s disease) is a progressive motor neuron disease that affects the corticospinal tracts and/or the anterior horn cells and/or bulbar motor nuclei.
Onset of the disease is usually after 40 years of age, and the disease is more common in men. Approximately 5% to 10% of cases are familial and are associated with an autosomal-dominant
mode of transmission.
Clinical Features: The hands are usually affected first with cramps, followed by weakness. Other manifestations include atrophy, muscle fasciculations, spasticity, and increased reflex response. There is usually a combination of upper and lower motor neuron signs. Dysarthria and dysphagia may occur; however, extraocular muscles, sensory function, sexual function, and urinary continence are usually not affected. Dementia is usually not present. Late in the illness, inappropriate, involuntary, and uncontrollable laughter or crying may occur.
Diagnosis is usually made with EMG findings that correlate with the clinical presentation.
Management: Unfortunately, there is no treatment other than supportive care. Baclofen has been used to treat muscular spasticity and cramping.
Prognosis: Death as a result of respiratory failure usually occurs within 5 years.
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