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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 44 year-old female presents to your office accompanied by a family member, who helps provide the history. The patient has had a 1 year history of worsening psychiatric and neurologic issues, including odd movements and memory problems and uncharacteristic irritability. The patient is on an antipsychotic for recent onset of hallucinations, 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 oculomotor dysfunction, cognitive slowing, motor impersistence, generalized choreiform movements, and slightly impaired memory. 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) Levodopa can be helpful in symptomatic treatment of the chorea in this disease

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

C) The earliest cognitive change in this disorder is usually memory loss

D) Suicide is rare in this disorder

E) There is currently no effective disease-modifying treatment for this disorder

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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) Levodopa can be helpful in symptomatic treatment of the chorea in 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. Levodopa is not used for treatment of chorea. (See References)


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B) 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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C) The earliest cognitive change in this disorder is usually memory loss

This answer is incorrect.

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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D) 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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E) There is currently no effective disease-modifying treatment for this disorder

This answer is correct.

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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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) Levodopa can be helpful in symptomatic treatment of the chorea in this disease This answer is incorrect.

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

C) The earliest cognitive change in this disorder is usually memory loss This answer is incorrect.

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