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 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.
What disease am I thinking of?
A) Causalgia B) Zellweger Syndrome C) Devic's Syndrome D) Hemifacial Spasm E) Riley-Day Syndrome
This question was created on February 21, 2007 by jdmiles.
This question was last modified on February 21, 2007.
ANSWERS AND EXPLANATIONS
A) Causalgia
This answer is incorrect.
Causalgia is a type of peripheral neuralgia in which there is persistent burning pain in an extremity, along with abnormal sympathetic innervation in the affected nerve. It is most often seen after trauma. (
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B) Zellweger Syndrome
This answer is incorrect.
Zellweger Syndrome is an autosomal recessive disorder that results in a lack of liver peroxisomes. It results in accumulation of very long chain fatty acids, and is terminal at a few months of age. (
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C) Devic's Syndrome
This answer is incorrect.
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. (
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D) Hemifacial Spasm
This answer is correct.
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)
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E) 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. (
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References:
1. Victor, M., and Ropper, A.H. (2001). Adams and Victor's Principles of Neurology, 7th Edition. McGraw-Hill, New York. | |
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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