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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 43 year-old female presents to your office accompanied by a family member, who helps provide the history. The patient has had a 1 year history of worsening neurologic and psychiatric issues, including memory problems and odd movements and uncharacteristic apathy. The patient is on atorvastatin for dyslipidemia, 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, motor impersistence, generalized choreiform movements, 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) Levodopa can be helpful in symptomatic treatment of the chorea in this disease

B) Pathophysiology of this disease involves a polyleucine strand

C) Typical life expectancy after clinical diagnosis with this disease is about 20 years

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

E) Clinical onset of symptoms does not occur before age 35

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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) 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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B) Pathophysiology of this disease involves a polyleucine 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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C) Typical life expectancy after clinical diagnosis with this disease is about 20 years

This answer is correct.

This patient has Huntington disease (HD). HD is progressive and terminal. Once the clincial diagnosis is made, the typical life expectancy for a person with HD is about 20 years. (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) Clinical onset of symptoms does not occur before age 35

This answer is incorrect.

This patient has Huntington disease (HD). Symptoms of HD usually begin between 35 and 45 years of age, but cases with onset as early as 2 years and as late as 92 years have been reported. (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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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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A patient with cognitive and movement complaints 02

Topic: Adult

ANSWERS AND EXPLANATIONS

A) Levodopa can be helpful in symptomatic treatment of the chorea in this disease This answer is incorrect.

B) Pathophysiology of this disease involves a polyleucine strand This answer is incorrect.

C) Typical life expectancy after clinical diagnosis with this disease is about 20 years This answer is correct.

D) Early diagnosis can allow for intervention which can significantly extend a patient's life This answer is incorrect.