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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 35 year-old female presents to your office accompanied by a family member, who helps provide the history. The patient has had a 3 year history of worsening psychiatric and neurologic issues, including memory problems and odd movements and uncharacteristic irritability. The patient is on hydrochlorothiazide for hypertension, 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, motor impersistence, oculomotor dysfunction, generalized choreiform movements, and slightly impaired memory. MRI 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) Tetrabenazine can be helpful in symptomatic treatment of the chorea in this disease

B) Typical life expectancy after clinical diagnosis with this disease is about 5 years

C) Pathophysiology of this disease involves a polyphenylalanine strand

D) This disease does not typically shorten a person's life expectancy

E) Typical life expectancy after clinical diagnosis with this disease is about 30 years

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

This answer is correct.

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. (See References)


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B) Typical life expectancy after clinical diagnosis with this disease is about 5 years

This answer is incorrect.

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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C) Pathophysiology of this disease involves a polyphenylalanine 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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D) This disease does not typically shorten a person's life expectancy

This answer is incorrect.

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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E) Typical life expectancy after clinical diagnosis with this disease is about 30 years

This answer is incorrect.

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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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) Tetrabenazine can be helpful in symptomatic treatment of the chorea in this disease This answer is correct.

B) Typical life expectancy after clinical diagnosis with this disease is about 5 years This answer is incorrect.

C) Pathophysiology of this disease involves a polyphenylalanine strand This answer is incorrect.

D) This disease does not typically shorten a person's life expectancy This answer is incorrect.

E) Typical life expectancy after clinical diagnosis with this disease is about 30 years This answer is incorrect.

References:

User Comments About This Question:

A) Tetrabenazine can be helpful in symptomatic treatment of the chorea in this disease

This answer is correct.

B) Typical life expectancy after clinical diagnosis with this disease is about 5 years

This answer is incorrect.

C) Pathophysiology of this disease involves a polyphenylalanine strand

This answer is incorrect.

D) This disease does not typically shorten a person's life expectancy

This answer is incorrect.

E) Typical life expectancy after clinical diagnosis with this disease is about 30 years

This answer is incorrect.