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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 44 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 psychiatric and neurologic issues, including odd movements and memory problems and uncharacteristic aggressive behavior. 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 motor impersistence, slightly impaired memory, generalized choreiform movements, oculomotor dysfunction, and cognitive slowing. 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) The choreiform movements may subside with time

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

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

D) Clinical onset of symptoms usually occurs between 15 and 25 years of age

E) Psychosis is uncommon 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) The choreiform movements may subside with time

This answer is correct.

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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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) 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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D) Clinical onset of symptoms usually occurs between 15 and 25 years of age

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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E) Psychosis is uncommon in this disease

This answer is incorrect.

This patient has Huntington disease (HD). Approximately 25% of patients with HD develop psychosis. (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) The choreiform movements may subside with time This answer is correct.

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

C) Approximately 5% of patients with this disorder attempt suicide This answer is incorrect.

D) Clinical onset of symptoms usually occurs between 15 and 25 years of age This answer is incorrect.