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Hemiconvulsion-Hemiplegia-Epilepsy in a girl with cobalamin C deficiency Volume 20, issue 6, December 2018

TEST YOURSELF

(1) The initial presentation of HHE is:

A. Progressive hemiplegia

B. Febrile hemiclonic status epilepticus

C. Multifocal myoclonic jerking

D. Developmental regression

(2) In HHE, chronic epilepsy most commonly:

A. Does not develop

B. Develops days after the initial acute presentation

C. Develops months to years after the initial acute presentation

D. Initially involves generalized seizure types

(3) Which of the following statements is false regarding HHE?

A. The acute HHE presentation may be confused with ischaemic stroke

B. The cytotoxic oedema seen during acute HHE presentation can lead to a mass effect and cerebral herniation

C. The acute HHE presentation is most often provoked by common viral illnesses

D. The majority of HHE cases are caused by SCN1A mutations

 

 

 

 

 

 

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Answers

(1) B.

(2) C.

(3) D.

 

 

 

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