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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 39 year-old male presents to your office accompanied by a family member, who helps provide the history. The patient has had a 1 year history of worsening neurologic and psychiatric issues, including odd movements and memory problems and uncharacteristic depression. The patient is on metoprolol 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, motor impersistence, oculomotor dysfunction, 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) Decreased volume of the striatum is pathognomonic for this disease

B) Psychosis is uncommon in this disease

C) Pathophysiology of this disease involves a polyglutamine strand

D) Tetrabenazine can delay the progression of this disease

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

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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) Decreased volume of the striatum is pathognomonic for this disease

This answer is incorrect.

This patient has Huntington disease (HD). While patients with HD often have decreased striatum volume which is visible on imaging, this finding is neither very specific nor very sensitive for HD. (See References)


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B) 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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C) Pathophysiology of this disease involves a polyglutamine strand

This answer is correct.

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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D) 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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E) 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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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) Decreased volume of the striatum is pathognomonic for this disease This answer is incorrect.

B) Psychosis is uncommon in this disease This answer is incorrect.

C) Pathophysiology of this disease involves a polyglutamine strand This answer is correct.

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