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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 male 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 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, cognitive slowing, slightly impaired memory, generalized choreiform movements, and motor impersistence. 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) Typical life expectancy after clinical diagnosis with this disease is about 30 years

B) Tetrabenazine can delay the progression of this disease

C) Dysphagia is a common complication of this disease

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

E) Typical life expectancy after clinical diagnosis with this disease is about 10 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) 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) Tetrabenazine can delay the progression of this disease

This answer is incorrect.

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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C) Dysphagia is a common complication of this disease

This answer is correct.

This patient has Huntington disease (HD). Dysphagia is common in this disease, and many patients with HD eventually die from dysphagia or aspiration. (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) 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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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) Tetrabenazine can delay the progression of this disease This answer is incorrect.

C) Dysphagia is a common complication of this disease This answer is correct.

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