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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 35 year-old female 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 aggressive behavior. 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 cognitive slowing, oculomotor dysfunction, generalized choreiform movements, slightly impaired memory, and motor impersistence. 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) The earliest cognitive changes in this disorder usually involve executive function
 
        B) Typical life expectancy after clinical diagnosis with this disease is about 30 years
 
        C) Rapid finger tapping is likely to be normal in patients with this disease
 
        D) Haloperidol can delay the progression of this disease
 
        E) Early diagnosis can allow for intervention which can significantly extend a patient's life
 

 


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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 earliest cognitive changes in this disorder usually involve executive function

This answer is correct.


This patient has Huntington disease (HD). Cognitive and psychiatric changes are a uniform part of the clinical picture of HD. The most frequent initial cognitive symptom involves some impairment of executive function, and is often first noticed at the patient's workplace.   (See References)

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B) 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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C) 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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D) Haloperidol 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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E) Early diagnosis can allow for intervention which can significantly extend a patient's life

This answer is incorrect.


This patient has Huntington disease (HD). While research is ongoing, there is currently no effective treatment which will alter the course of this progressive and terminal illness.   (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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adult
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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