engleski

Myoclonic astatic epilepsy of early childhood, Atypical benign partial epilepsy and Pseudo Lennox syndrome - controversy in nosology

In 1985. Doose defined and in 1992. he further elaborated entity of Myoclonic astatic epilepsy of early childhood (MAE). However, in the meantime, he in 1989. introduced the term Pseudo Lennox syndrome (PLS) using it as the synonym to Atypical benign partial epilepsy (ABPE), the entity defined by Aicardi back in 1982. Aicardi in his book &#8216 ; ; Epilepsy in children&#8217 ; ; from 1994. does not describe MAE. In 1995. Doose tried to distinguish MAE from ABPE i.e. PLS emphasising numerous overlapping of the clinical symptoms and EEG abnormalities. Although Doose himself indicates that it is very difficult to differentiate MAE from PLS, even from Lennox-Gastaut syndrome (L-GS), it is surprising that he uses PLS as a synonym for ABPE. It is even stranger that Aicardi almost ignores MAE as a separate entity. Until now we have made a diagnosis of MAE in about 20 children. In every particular child with developed clinical and EEG symptoms, we consider MAE in differential diagnosis to L-GS, but we made the final diagnosis of MAE only retrospectively, if the child is at last without seizures and of normal psychomotor development. As in our opinion the problem exists in differentiation between MAE from L-GS, while it is almost irrelevant in differentiation between ABPE from L-GS, the term PLS is more appropriate as a synonym for MAE than as for ABPE. We call MAE as Doose syndrome.

Myoclonic Astatic Epilepsy; Pseudo Lennox Syndrome; Atypical Benign Partial Epilepsy

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