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Motor Neuron Disorders 01

Topic: Adult

Created on Thursday, February 12 2009 by jdmiles

Last modified on Thursday, February 12 2009.

Which of the following statements is true about primary lateral sclerosis?

 
        A) It is a demyelinating disease which selectively affects motor nerves
 
        B) It is a disease in which there is loss of lower motor neurons only, and upper motor neurons are unaffected
 
        C) It is a disease in which there is unilateral loss of motor neurons resulting in hemiparesis
 
        D) It is a disease in which there is loss of upper and lower motor neurons
 
        E) It is a disease in which there is loss of upper motor neurons only, and lower motor neurons are unaffected
 

 


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This question was created on February 12, 2009 by jdmiles.
This question was last modified on February 12, 2009.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ANSWERS AND EXPLANATIONS




A) It is a demyelinating disease which selectively affects motor nerves

This answer is incorrect.


Multifocal motor neuropathy is a disorder which results in demyelination of motor nerves in the peripheral nervous system. Progressive muscular atrophy is a motor neuron disease which only affects the lower motor neurons, sparing upper motor neurons.  (See References)

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B) It is a disease in which there is loss of lower motor neurons only, and upper motor neurons are unaffected

This answer is incorrect.


Lower motor neurons are not affected in primary lateral sclerosis. Progressive muscular atrophy is a motor neuron disease which only affects the lower motor neurons, sparing upper motor neurons.  (See References)

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C) It is a disease in which there is unilateral loss of motor neurons resulting in hemiparesis

This answer is incorrect.


Progressive muscular atrophy is a motor neuron disease which only affects the lower motor neurons, sparing upper motor neurons. It can affect both sides of the body. Mills' syndrome is a motor neuron disease which results in strictly unilateral weakness.  (See References)

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D) It is a disease in which there is loss of upper and lower motor neurons

This answer is incorrect.


Anterior lateral sclerosis (ALS or Lou Gerhig's disease) is a motor neuron disorder which affects both upper and lower motor neurons. Lower motor neurons are not affected in primary lateral sclerosis.  (See References)

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E) It is a disease in which there is loss of upper motor neurons only, and lower motor neurons are unaffected

This answer is correct.


Primary lateral sclerosis is a disease of upper motor neurons only. Lower motor neurons are not affected in primary lateral sclerosis.  (See References)

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References:

1. Pestronk, A. (1998). "Multifocal motor neuropathy: diagnosis and treatment." Neurology, 51(6 Suppl 5) S22-4. (PMID:9851726)
2. Rajabally, Y.A., Hbahbih, M., and Abbott, R.J. (2005). "Hemiplegic ALS: Mills syndrome." Neurology, 64(11) 1984-5. (PMID:15955966)
3. Gastaut, J.L., and Bartolomei, F. (1994). "Mills' syndrome: ascending (or descending) progressive hemiplegia: a hemiplegic form of primary lateral sclerosis?" J Neurol Neurosurg Psychiatry, 57(10) 1280-1. (PMID:7931406)
4. Singer, M.A., Statland, J.M., Wolfe, G.I., and Barohn, R.J. (2007). "Primary lateral sclerosis." Muscle Nerve, 35(3) 291-302. (PMID:17212349)
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adult
Motor Neuron Disorders 01
Question ID: 021209148
Question written by J. Douglas Miles, (C) 2006-2009, all rights reserved.
Created: 02/12/2009
Modified: 02/12/2009
Estimated Permutations: 120

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