There are 484 questions on various topics in Neurology in the FrontalCortex neurology question bank.

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 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 psychiatric and neurologic issues, including odd movements and memory problems and uncharacteristic aggressive behavior. The patient is on atorvastatin for dyslipidemia, 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 slightly impaired memory, oculomotor dysfunction, motor impersistence, cognitive slowing, and generalized choreiform movements. 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) Psychosis is common in this disease

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

C) Suicide is rare in this disorder

D) Pathophysiology of this disease involves a polyvaline strand

E) The degree of chorea is a reliable marker of the severity of 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) Psychosis is common in this disease

This answer is correct.

This patient has Huntington disease (HD). Approximately 25% of patients with HD develop psychosis. (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) Suicide is rare in this disorder

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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D) Pathophysiology of this disease involves a polyvaline strand

This answer is incorrect.

This patient has Huntington disease (HD). Genetically, HD appears to be caused by a CAG trinucleotide expanding repeat on chromosome 4. This reults in a polyglutamine strand incorporated into the translated huntingtin protein. (See References)


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E) The degree of chorea is a reliable marker of the severity of this disease

This answer is incorrect.

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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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 common in this disease This answer is correct.

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

C) Suicide is rare in this disorder This answer is incorrect.

D) Pathophysiology of this disease involves a polyvaline strand This answer is incorrect.