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1. A 16 yrs old caucasian male is evaluated for progressive gait instability and disarthria. His brother died of a neurologic disease at age 21. MRI of the brain and spines shows degeneration of the posterior columns and spinocerebellar tracts. This pt conditionmost closely mimicked wich of the followings conditions?///////
a.lead intoxication
b.thiamine deficiency
c.vit. e deficiency
d.prion disease
e.poliomyelitis
f.acute intermitent porphyria
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CCCCCC... Resembles Friedricks Ataxia
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i thot vit b-12 resembles F.Ataxia?
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This is a anticipation case......Fried Hunt My Fragyl Spino (Friedreich/Huntington/Myotonic distrophy/Fragyl sx/ Spinocerebellar ataxia) ...and this pt look like Friedreich that mimic Vit E deficiency
CCCC
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the most notable change of vit E def. occurs in the nervous system, with degeneration of spinocerebellar tracts, dorsal column of the spinal cord and peripheral nerves are seen, and it closely mimic friederic ataxia b/c many of the same areas of the CNS are affected and sx of both disease included ataxia, dysarthria, loss of position and vibration sensation.
