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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 42 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 apathy. 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 oculomotor dysfunction, motor impersistence, generalized choreiform movements, slightly impaired memory, 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) Clinical onset of symptoms usually occurs between 55 and 65 years of age

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

C) Typical life expectancy after clinical diagnosis with this disease is about 10 years

D) Rapid finger tapping is likely to be normal in patients with this disease

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

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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) Clinical onset of symptoms usually occurs between 55 and 65 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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B) 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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C) Typical life expectancy after clinical diagnosis with this disease is about 10 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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D) Rapid finger tapping is likely to be normal in patients with this disease

This answer is incorrect.

This patient has Huntington disease (HD). Fine motor skills are impaired in HD. Having the patient rapidly tap his or her forefinger against his or her thumb is a sensitive test of this impairment. Movements are likely to be slow or irregular. (See References)


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E) 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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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) Clinical onset of symptoms usually occurs between 55 and 65 years of age This answer is incorrect.

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

C) Typical life expectancy after clinical diagnosis with this disease is about 10 years This answer is incorrect.

D) Rapid finger tapping is likely to be normal in patients with this disease This answer is incorrect.