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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 36 year-old male presents to your office accompanied by a family member, who helps provide the history. The patient has had a 3 year history of worsening neurologic and psychiatric issues, including memory problems and odd movements and uncharacteristic irritability. The patient is on an antipsychotic for recent onset of hallucinations, 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 cognitive slowing, generalized choreiform movements, oculomotor dysfunction, slightly impaired memory, and motor impersistence. 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) This disorder has a higher prevalence among blacks than among caucasians or asians
 
        B) Cognitive decline is usually a late finding in this disease
 
        C) Decreased volume of the striatum is not diagnostic of this disease
 
        D) Early diagnosis can allow for intervention which can significantly extend a patient's life
 
        E) The suicide rate in this disorder is approximately 2%
 

 


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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) This disorder has a higher prevalence among blacks than among caucasians or asians

This answer is incorrect.


This patient has Huntington disease (HD). Prevalence of HD among caucasians is approximately 10 cases per 100,000. Prevalence is much less in black and Asian populations.   (See References)

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B) Cognitive decline is usually a late finding in this disease

This answer is incorrect.


This patient has Huntington disease (HD). Cognitive decline is often evident by the time the patient presents with other clinical findings.   (See References)

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C) 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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D) Early diagnosis can allow for intervention which can significantly extend a patient's life

This answer is incorrect.


This patient has Huntington disease (HD). While research is ongoing, there is currently no effective treatment which will alter the course of this progressive and terminal illness.   (See References)

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E) The suicide rate in this disorder is approximately 2%

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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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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