FrontalCortex, Inc. is a non-profit corporation dedicated to neurology education.

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 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 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, cognitive slowing, oculomotor dysfunction, 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) Rapid finger tapping is likely to be normal in patients with this disease
 
        B) This disease does not affect swallowing
 
        C) There is currently no effective disease-modifying treatment for this disorder
 
        D) Typical life expectancy after clinical diagnosis with this disease is about 40 years
 
        E) Decreased volume of the striatum is pathognomonic for this disease
 

 


Back to the question = Go back to the top of the page.
See another question like this one = Reload a different version of this question ().
Click here for a random question = Load a random question from the database.
Clone this question = Use this question as a template to create a totally NEW question.
Rate this question = Enter detailed rating for this question!
Average user rating for this question = 4 = How users like you have rated this question.
This question was created on September 23, 2008 by jdmiles.
This question was last modified on September 23, 2008.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ANSWERS AND EXPLANATIONS




A) Rapid finger tapping is likely to be normal in patients with this disease

This answer is incorrect.


This patient has Huntington disease (HD). Fine motor skills are impaired in HD. Having the patient rapidly tap his or her forefinger against his or her thumb is a sensitive test of this impairment. Movements are likely to be slow or irregular.   (See References)

Back to the questionSee another question like this oneClick here for a random questionClone this question Rate this questionAverage user rating for this question = 4
Please log in if you want to rate questions.

 

 

 

 

 

 

 

 

 




B) 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)

Back to the questionSee another question like this oneClick here for a random questionClone this question Rate this questionAverage user rating for this question = 4
Please log in if you want to rate questions.

 

 

 

 

 

 

 

 

 




C) 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)

Back to the questionSee another question like this oneClick here for a random questionClone this question Rate this questionAverage user rating for this question = 4
Please log in if you want to rate questions.

 

 

 

 

 

 

 

 

 




D) Typical life expectancy after clinical diagnosis with this disease is about 40 years

This answer is incorrect.


This patient has Huntington disease (HD). HD is progressive and terminal. Once the clincial diagnosis is made, the typical life expectancy for a person with HD is about 20 years.   (See References)

Back to the questionSee another question like this oneClick here for a random questionClone this question Rate this questionAverage user rating for this question = 4
Please log in if you want to rate questions.

 

 

 

 

 

 

 

 

 




E) 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)

Back to the questionSee another question like this oneClick here for a random questionClone this question Rate this questionAverage user rating for this question = 4
Please log in if you want to rate questions.

 

 

 

 

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:
Back to the questionSee another question like this oneClick here for a random questionClone this question Rate this questionAverage user rating for this question = 4
Please log in if you want to rate questions.

 

FrontalCortex.com -- Neurology Review Questions -- Neurology Boards -- Board Review -- Residency Inservice Training Exam -- RITE Exam Review
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

User Comments About This Question:

0 user entries
Please log in if you'd like to add a comment.