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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 41 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 odd movements and memory problems and uncharacteristic depression. 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 generalized choreiform movements, cognitive slowing, slightly impaired memory, motor impersistence, and oculomotor dysfunction. 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) Cognitive decline is usually a late finding in this disease

B) Tetrabenazine can delay the progression of this disease

C) The choreiform movements will worsen throughout the course of this disease

D) This disease does not affect swallowing

E) Depression in this disorder often responds to standard antidepressant medications

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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) Cognitive decline is usually a late finding in this disease

This answer is incorrect.

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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B) Tetrabenazine 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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C) The choreiform movements will worsen throughout the course of this disease

This answer is incorrect.

This patient has Huntington disease (HD). Choreiform movements are a hallmark of this disease, but they often subside in the later stage of the disease, giving way to rigidity and dystonia. (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) Depression in this disorder often responds to standard antidepressant medications

This answer is correct.

This patient has Huntington disease (HD). Depression in HD often responds to standard antidepressant therapy. (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) Cognitive decline is usually a late finding in this disease This answer is incorrect.

B) Tetrabenazine can delay the progression of this disease This answer is incorrect.

C) The choreiform movements will worsen throughout the course of this disease This answer is incorrect.

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