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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 43 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 aggressive behavior. The patient is on eyedrops for glaucoma, 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 motor impersistence, oculomotor dysfunction, cognitive slowing, slightly impaired memory, 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) Depression in this disorder typically does not respond to standard antidepressant medications

B) Pathophysiology of this disease involves a polycysteine strand

C) The suicide rate in this disorder is approximately 2%

D) This disease does not affect swallowing

E) Tetrabenazine can be helpful in symptomatic treatment of the chorea 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) Depression in this disorder typically does not respond to standard antidepressant medications

This answer is incorrect.

This patient has Huntington disease (HD). Depression in HD often responds to standard antidepressant therapy. (See References)


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B) Pathophysiology of this disease involves a polycysteine 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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C) The suicide rate in this disorder is approximately 2%

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) 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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E) Tetrabenazine can be helpful in symptomatic treatment of the chorea in this disease

This answer is correct.

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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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) Depression in this disorder typically does not respond to standard antidepressant medications This answer is incorrect.

B) Pathophysiology of this disease involves a polycysteine strand This answer is incorrect.

C) The suicide rate in this disorder is approximately 2% This answer is incorrect.

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