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 40 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 psychiatric and neurologic issues, including memory problems and odd movements and uncharacteristic irritability. The patient is on metoprolol 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 oculomotor dysfunction, cognitive slowing, slightly impaired memory, generalized choreiform movements, 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) Depression in this disorder typically does not respond to standard antidepressant medications

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

C) The choreiform movements will worsen throughout the course of this disease

D) Approximately one quarter of patients with this disorder attempt suicide

E) The degree of chorea is a reliable marker of the severity of 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) Depression in this disorder typically does not respond to standard antidepressant medications

This answer is incorrect.

This patient has Huntington disease (HD). Depression in HD often responds to standard antidepressant therapy. (See References)


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B) 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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C) The choreiform movements will worsen throughout the course of this disease

This answer is incorrect.

This patient has Huntington disease (HD). Choreiform movements are a hallmark of this disease, but they often subside in the later stage of the disease, giving way to rigidity and dystonia. (See References)


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D) Approximately one quarter of patients with this disorder attempt suicide

This answer is correct.

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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E) 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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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
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A patient with cognitive and movement complaints 02

Topic: Adult

ANSWERS AND EXPLANATIONS

A) Depression in this disorder typically does not respond to standard antidepressant medications This answer is incorrect.

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

C) The choreiform movements will worsen throughout the course of this disease This answer is incorrect.

D) Approximately one quarter of patients with this disorder attempt suicide This answer is correct.