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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 38 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 odd movements and memory problems and uncharacteristic aggressive behavior. The patient has no significant past medical history, and is on no medications at home_,The patient is on lisinopril 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 motor impersistence, generalized choreiform movements, slightly impaired memory, oculomotor dysfunction, and cognitive slowing. 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) Typical life expectancy after clinical diagnosis with this disease is about 30 years

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

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

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

E) Depression in this disorder typically does not respond to standard antidepressant medications

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

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

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

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