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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 female presents to your office accompanied by a family member, who helps provide the history. The patient has had a 2 year history of worsening neurologic and psychiatric 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 cognitive slowing, motor impersistence, slightly impaired memory, generalized choreiform movements, and oculomotor dysfunction. 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) Psychosis is uncommon in this disease

B) This disease does not affect swallowing

C) Typical life expectancy after clinical diagnosis with this disease is about 40 years

D) The degree of chorea does not correlate well with disease severity

E) Clinical onset of symptoms does not occur before age 35

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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) 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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B) This disease does not affect swallowing

This answer is incorrect.

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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C) Typical life expectancy after clinical diagnosis with this disease is about 40 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) The degree of chorea does not correlate well with disease severity

This answer is correct.

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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E) Clinical onset of symptoms does not occur before age 35

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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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) Psychosis is uncommon in this disease This answer is incorrect.

B) This disease does not affect swallowing This answer is incorrect.

C) Typical life expectancy after clinical diagnosis with this disease is about 40 years This answer is incorrect.

D) The degree of chorea does not correlate well with disease severity This answer is correct.