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 NEURO........  



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  #1

A 9 y.o boy presents with confusion & decreased school performance.He soon develops a spastic gait with dysarthria & dysphagia.He also develops visula loss.An MRI scan shows massive demyelination of the white matter in the posterior areas of the hemispheres.Which of the following is the most likely diagnosis?
1.Multiple sclerosis
2.Metachromatic leukodystrophy
3.Adrenoleukodystrophy
4.Subacute sclerosing panencephalitis
5.Creutzfeldt-jacob ds.




  #2

it's adrenoleukodystrophy, X linked disoder with calssic presentation. Also there is no history of measle disease and he's too young , so it could be SSPE or MS. And it's could be arylsulfatase A deficiensy, metachromatic leukodystrophy without other organs involvement


  #3

Yes galad , it's adrenoleukodystrophy..........CORRECT grin
and esplly. with posterior hemisphere predominance here, it fits that the best. In metachromatic, weakness & decreased reflexes r seen instead of spasticity.......... grin , so, it's not the choice too


  #4

Thanks, smitha. It's a great idea to give explanation to the Q


  #5

I recentily saw a movie about how a family fought for the development of adrenoleukoddystrophy's ttt - extraordinary. forgot it's name though sad





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