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Benign adult familial myoclonus epilepsy is a progressive disorder: no longer idiopathic generalized epilepsy Volume 18, issue 1, March 2016

TEST YOURSELF

(1) What are the clinical characteristics of benign adult familial myoclonus epilepsy (BAFME)?


(2) What is the significance of a slow frequency of the posterior dominant rhythm (PDR) on EEG in BAFME?


(3) Why is BAFME not idiopathic generalized epilepsy?

 

 

 

 

 

 

 

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Answers

(1) BAFME manifests as an autosomal dominant trait and is characterized by cortical tremor resembling essentially tremor and generalized tonic-clonic seizures.


(2) PDR slowing in Japanese BAFME indicates mild, but clear diffuse, brain dysfunction, at least based on the clinical EEG.


(3) Diffuse brain dysfunction in BAFME supports the notion that BAFME is not non-progressive or so-called “benign”, and BAFME may thus not be regarded as idiopathic generalized epilepsy.

 

 

 

 

 

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