ganesha123
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A 6-year-old boy is brought to the physician by his mother because of progressive visual loss over the past year. Over the past 2 years, he has had deterioration of his hearing, speech, writing, and intellectual performance. His maternal uncle had similar symptoms. Visual acuity is 20/200 bilaterally. Funduscopic examination shows optic atrophy. His hearing is markedly impaired. There is weakness and spasticity of all extremities. Deep tendon reflexes are extremely hyperactive. Babinski's sign is present bilaterally. On mental status examination, he is not oriented to place, year, month, or the names of his siblings. An MRI of the brain shows marked symmetric white matter disease involving all lobes. Diagnostic studies are most likely to show which of the following?
A
) Abnormally decreased serum cholesterol level
B
) Acanthocytes on blood smear
C
) An excess of very long chain fatty acids
D
) Normal nerve conduction studies
A 42-year-old man is brought to the physician by his wife because of a 2-month history of staring spells that last 1 to 2 minutes each. During episodes, he also smacks his lips and picks at his shirt collar. Four years ago, he was comatose for 2 weeks after sustaining a head injury in a motorcycle collision; he required 6 months of rehabilitation. He reports that, over the past year, he has had intermittent episodes of smelling burnt rubber that occur approximately every 2 weeks. He hears an intense hissing sound during these episodes. Examination shows no abnormalities. Which of the following is the most likely diagnosis?
A
) Absence seizures
B
) Complex partial seizures
C
) Transient ischemic attack
D
) Tourette's disorder
E
) Limbic encephalopathy
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| peter90036
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c
a ...?....e?
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| ganesha123
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could u explain the first one..?? if its krabbe's??
and 2nd one i think is complex partial.. not sure tho...
Edited by ganesha123 on 03/21/08 - 01:40 PM
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| ganesha123
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cud be limbic enceph too.... could some one clarify..???
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| peter90036
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its elimination of what seems wrong
and pick what seems right... right-er
1st: ...
young child
progressive
neuro si/sx all over, + optic nerve,
+ entire brain
!!!
= something "metabolic" ,enzyme , one of those storage diseases
definitely not b or d
a... maybe?
but C sounds right
-- i dont know the real answer or disease ---
#2
same approach
...B but no loss of consciousness
...C but no history of cv dz
...D no
...E ... maybe - smelling burnt rubber, intense hissing sounds... but i dont know the disease - also "encephalopathy" sounds like it should be all the time with symptoms.
...A because of the presentation 
--- dont know, but in a test i'd pick A, and go on to the next Q
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| ganesha123
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Complex partial or temporal lobe seizures—During this type of seizure, you will briefly lose contact with reality, stop purposeful activity, and begin a series of automatic gestures, such as lip smacking, handwringing, picking at clothing, etc. Not everyone develops automatic gestures during these seizures; sometimes it simply appears as a brief moment of confusion or loss of attentiveness. Occasionally this type of seizure is accompanied by the perception of unusual sights, sounds, or smells
found this online......so i conclude with the answer being comlex partial seizure..there is no need to lose conciousness...!!
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| peter90036
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+1 for you
i should go back and read some more 
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| ganesha123
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my exams z in three days.. i dont even have time to read anymore.... 
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| peter90036
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just don't stress out !
and good luck to you
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| ganesha123
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thanx.. 
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