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

Guess the disease!

Topic: Behavior

Created on Wednesday, February 21 2007 by jdmiles

Last modified on Wednesday, February 21 2007.

GUESS WHAT I'M THINKING!

I'm thinking of a disorder which may be inherited or sporadic. It begins in early infancy with loss of smile, loss of interest in surroundings, episodes of sweating, seizures, and diffuse myoclonic jerks. As it progresses, there follows diffuse spasticity, blindness, delayed growth, and progressive microcephaly. In late stages, the patient is essentially decorticate. The etiology is not yet understood.
What disease am I thinking of?

 
        A) Hemifacial Spasm
 
        B) Alpers-Hutttenlocher syndrome
 
        C) Sydenham Chorea
 
        D) Dancing Eyes-Dancing Feet Syndrome
 
        E) Alpers' 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 February 21, 2007 by jdmiles.
This question was last modified on February 21, 2007.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ANSWERS AND EXPLANATIONS




A) Hemifacial Spasm

This answer is incorrect.


Hemifacial Spasm is a disorder characterised by painless twitching of the muscles on one side of the face (in 5%, bilateral, but asynchronous when it is). Usually caused by arterial compression of the facial nerve, which results in focal demyelination followed by ephaptic coupling of parallel axons. It can also result from Bell's palsy. Medcial treatments include Carbemazepine, baclofen, gabapentin and botulinum toxin. Surgical decompression of the nerve is a highly successful procedure, with some risks, including monaural deafness.  (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) Alpers-Hutttenlocher syndrome

This answer is incorrect.


Alpers-Hutttenlocher syndrome is a subtype of progressive cerebral poliodystrophy in which there are also hepatic symptoms: jaundice, fatty degeneration, and cirrhosis. Other symptoms include anemia, thrombocytopenia, and trichorrhexis.  (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) Sydenham Chorea

This answer is incorrect.


Sydenham Chorea is a choreiform disorder associated with streptococcal infection.   (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) Dancing Eyes-Dancing Feet Syndrome

This answer is incorrect.


Dancing Eyes-Dancing Feet Syndrome is a cerebellar ataxia with polymyoclonus which may be postinfectious.  (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) Alpers' Disease

This answer is correct.


Alpers' Disease is a disorder which may be inherited or sporadic. It begins in early infancy with loss of smile, loss of interest in surroundings, episodes of sweating, seizures, and diffuse myoclonic jerks. As it progresses, there follows diffuse spasticity, blindness, delayed growth, and progressive microcephaly. In late stages, the patient is essentially decorticate. The etiology is not yet understood.  (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. Victor, M., and Ropper, A.H. (2001). Adams and Victor's Principles of Neurology, 7th Edition. McGraw-Hill, New York.
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
behavior
Guess the disease!
Question ID: 02210701
Question written by J. Douglas Miles, (C) 2006-2009, all rights reserved.
Created: 02/21/2007
Modified: 02/21/2007
Estimated Permutations: 0

User Comments About This Question:

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