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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 2 year history of worsening neurologic and psychiatric issues, including odd movements and memory problems and uncharacteristic irritability. 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 generalized choreiform movements, cognitive slowing, oculomotor dysfunction, motor impersistence, and slightly impaired memory. 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) This disorder has a higher prevalence among blacks than among caucasians or asians
 
        B) Rapid finger tapping is likely to be normal in patients with this disease
 
        C) Cognitive decline is usually a late finding in this disease
 
        D) Clinical onset of symptoms does not occur before age 35
 
        E) Psychosis is uncommon in this disease
 

 


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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) This disorder has a higher prevalence among blacks than among caucasians or asians

This answer is incorrect.


This patient has Huntington disease (HD). Prevalence of HD among caucasians is approximately 10 cases per 100,000. Prevalence is much less in black and Asian populations.   (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) Cognitive decline is usually a late finding in this disease

This answer is correct.


This patient has Huntington disease (HD). Cognitive decline is often evident by the time the patient presents with other clinical findings.   (See References)

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D) 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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E) 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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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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