A patient with cognitive and movement complaints 02
Topic: AdultCreated on Tuesday, September 23 2008 by jdmiles
Last modified on Tuesday, September 23 2008.
A 38 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 psychiatric and neurologic issues, including odd movements and memory problems and uncharacteristic apathy.
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 cognitive slowing, slightly impaired memory, oculomotor dysfunction, generalized choreiform movements, and motor impersistence.
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) Clinical onset of symptoms usually occurs between 35 and 45 years of age
B) This disorder has a higher prevalence among blacks than among caucasians or asians
C) Clinical onset of symptoms does not occur before age 35
D) Approximately 5% of patients with this disorder attempt suicide
E) Tetrabenazine can delay the progression of this disease
This question was created on September 23, 2008 by jdmiles.
This question was last modified on September 23, 2008.
ANSWERS AND EXPLANATIONS
A) Clinical onset of symptoms usually occurs between 35 and 45 years of age
This answer is correct.
This patient has Huntington disease (HD). Symptoms of HD usually begin between 35 and 45 years of age, but cases with onset as early as 2 years and as late as 92 years have been reported.
( See References)
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B) This disorder has a higher prevalence among blacks than among caucasians or asians
This answer is incorrect.
This patient has Huntington disease (HD). Prevalence of HD among caucasians is approximately 10 cases per 100,000. Prevalence is much less in black and Asian populations.
( See References)
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C) Clinical onset of symptoms does not occur before age 35
This answer is incorrect.
This patient has Huntington disease (HD). Symptoms of HD usually begin between 35 and 45 years of age, but cases with onset as early as 2 years and as late as 92 years have been reported.
( See References)
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D) Approximately 5% of patients with this disorder attempt suicide
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) 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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References:
1. Walker, F.O. (2007). "Huntington's Disease." Semin Neurol, 27(2) 143-50. (PMID:17390259)
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2. Walker, F.O. (2007). "Huntington's disease." Lancet, 369(9557) 218-28. (PMID:17240289)
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3. Zaidat, O.O., and Lerner, A.J. (2002). The Little Black Book of Neurology, 4th Edition. Mosby, St. Louis (ISBN:0323014151)
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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).
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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).
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adult
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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