Guess the disease!

Topic: Behavior

Created on Wednesday, February 21 2007 by jdmiles

Last modified on Wednesday, February 21 2007.

 
        A) Alexander Disease
 
        B) Brown-Sequard Syndrome
 
        C) Tropical Spastic Paraparesis
 
        D) Riley-Day Syndrome
 
        E) Devic's Syndrome
 

 

	= Go back to the top of the page.
	= Reload a different version of this question ().
	= Load a random question from the database.
	= Use this question as a template to create a totally NEW question.
	= Enter detailed rating for this question!
	= 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) Alexander Disease

This answer is incorrect.

Alexander Disease is a rare, congenital disease which does not appear to be inherited. No metabolic cause has been found. Clinical features include seizures, psychomotor retardation, and failure to thrive, followed by progressive macrocephaly. The frontal lobes show white matter degeneration, and pathology shows Rosenthal fibers found near the pia and blood vessels, these are thought to be detritus from glial destruction.  (See References)

Please log in if you want to rate questions.

 

 

 

 

 

 

 

 

 

---

B) Brown-Sequard Syndrome

This answer is incorrect.

Brown-Sequard Syndrome is a homolateral paralysis which spares the face, with ipsilateral loss of position and vibratory sense, and contralateral loss of temperature and pain. Results from a unilateral spinal cord lesion.  (See References)

Please log in if you want to rate questions.

 

 

 

 

 

 

 

 

 

---

C) Tropical Spastic Paraparesis

This answer is incorrect.

Tropical Spastic Paraparesis is a myelopathy related to infection with the HTLV-I virus. It is endemic to Martinique, Jamaica, Colombia and Japan. Key clinical signs include slowly progressing paraparesis with increased DTRs and positive Babinski.  (See References)

Please log in if you want to rate questions.

 

 

 

 

 

 

 

 

 

---

D) Riley-Day Syndrome

This answer is incorrect.

Riley-Day Syndrome is a congenital, autosomal recessive disease which predominately affects Jewish children. Failure to thrive and fever are early signs. Hyporeflexia and decreased pain & temperature sensation are noted on exam. There is also poor function of the autonomic nervous system. The standard test is for dopamine B-hydroxylase, which is diminished.  (See References)

Please log in if you want to rate questions.

 

 

 

 

 

 

 

 

 

---

E) Devic's Syndrome

This answer is correct.

Devic's Syndrome is a variant of multiple sclerosis (MS), in which optic neuritis and transverse myelitis occur together, or at least within weeks of each other. The transverse myelitis frequently leads to necrosis, and is thus more permanent than the usual transverse myelitis associated with MS. It also differs from MS in that the lesions are often limited to those described above, and the rest of the CNS is spared. Serologic finidngs are also frequently different from those of MS, e.g., no oligoclonal bands.  (See References)

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.

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