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A patient with cognitive and movement complaints 02

Topic: Adult

Created on Tuesday, September 23 2008 by jdmiles

Last modified on Tuesday, September 23 2008.

A 38 year-old male presents to your office accompanied by a family member, who helps provide the history. The patient has had a 1 year history of worsening psychiatric and neurologic issues, including odd movements and memory problems and uncharacteristic depression. The patient is on eyedrops for glaucoma, and has no other past medical history. The patient confirms that other family members have had similar symptoms, and some have died at an early age. On exam, you note slightly impaired memory, cognitive slowing, generalized choreiform movements, motor impersistence, and oculomotor dysfunction. CT of the brain is remarkable only for slight atrophy of the caudate bilaterally. The patient's diagnosis was confirmed by genetic testing, which showed a CAG trinucleotide repeat on chromosome 4.
Of the following statements, which is most accurate about this patient's disease?

 
        A) Decreased volume of the striatum is not diagnostic of this disease
 
        B) Approximately 5% of patients with this disorder attempt suicide
 
        C) The degree of chorea is a reliable marker of the severity of this disease
 
        D) Pathophysiology of this disease involves a polyserine strand
 
        E) Levodopa can be helpful in symptomatic treatment of the chorea in this disease
 

 


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This question was created on September 23, 2008 by jdmiles.
This question was last modified on September 23, 2008.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ANSWERS AND EXPLANATIONS




A) Decreased volume of the striatum is not diagnostic of this disease

This answer is correct.


This patient has Huntington disease (HD). While patients with HD often have decreased striatum volume which is visible on imaging, this finding is neither very specific nor very sensitive for HD.   (See References)

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B) Approximately 5% of patients with this disorder attempt suicide

This answer is incorrect.


This patient has Huntington disease (HD). The suicide rate in HD is estimated to be greater than 10%, and as many as 25% of patients with HD attempt suicide.   (See References)

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C) The degree of chorea is a reliable marker of the severity of this disease

This answer is incorrect.


This patient has Huntington disease (HD). While chorea is a hallmark finding in HD, the degree of chorea does not correlate well with the severity of the disease. Some patients may only have mild or transient chorea during the course of the illness, and it is common for chorea to become less prominent in the late stages of the disease. Motor impersistence may be a better marker of disease severity.   (See References)

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D) Pathophysiology of this disease involves a polyserine strand

This answer is incorrect.


This patient has Huntington disease (HD). Genetically, HD appears to be caused by a CAG trinucleotide expanding repeat on chromosome 4. This reults in a polyglutamine strand incorporated into the translated huntingtin protein.   (See References)

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E) Levodopa can be helpful in symptomatic treatment of the chorea in this disease

This answer is incorrect.


This patient has Huntington disease (HD). There is currently no disease-altering treatment, but the choreiform movements are sometimes alleviated with neuroleptics (e.g., haloperidol) or tetrabenazine. Levodopa is not used for treatment of chorea.   (See References)

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References:

1. Walker, F.O. (2007). "Huntington's Disease." Semin Neurol, 27(2) 143-50. (PMID:17390259)
2. Walker, F.O. (2007). "Huntington's disease." Lancet, 369(9557) 218-28. (PMID:17240289)
3. Zaidat, O.O., and Lerner, A.J. (2002). The Little Black Book of Neurology, 4th Edition. Mosby, St. Louis (ISBN:0323014151) Advertising:
4. Bertelson, J.A., and Price, B.H. (2004). Depression and psychosis in neurological practice. In Bradley, W.G., Daroff, R.B., Fenichel, G.M., and Jankovic, J. (Eds.). Neurology in Clinical Practice, Fourth Edition. Butterworth Heinemann, Philadelphia, pp. 103-116 (ISBN:0750674695). Advertising:
5. Shannon, K.M. (2004). Movement disorders. In Bradley, W.G., Daroff, R.B., Fenichel, G.M., and Jankovic, J. (Eds.). Neurology in Clinical Practice, Fourth Edition. Butterworth Heinemann, Philadelphia, pp. 2125-2168 (ISBN:0750674695). Advertising:
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adult
A patient with cognitive and movement complaints 02
Question ID: 010108123
Question written by J. Douglas Miles, (C) 2006-2009, all rights reserved.
Created: 09/23/2008
Modified: 09/23/2008
Estimated Permutations: 49140000

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