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 neurologic and psychiatric issues, including odd movements and memory problems 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 generalized choreiform movements, motor impersistence, slightly impaired memory, cognitive slowing, 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) Haloperidol can be helpful in symptomatic treatment of the chorea in this disease
B) Clinical onset of symptoms does not occur before age 35
C) This disease does not affect swallowing
D) Clinical onset of symptoms usually occurs between 55 and 65 years of age
E) The earliest cognitive change in this disorder is usually memory loss
This question was created on September 23, 2008 by jdmiles.
This question was last modified on September 23, 2008.
ANSWERS AND EXPLANATIONS
A) Haloperidol 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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B) 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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C) 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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D) Clinical onset of symptoms usually occurs between 55 and 65 years of age
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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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)
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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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