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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 the entity Myoclonic astatic epilepsy of early childhood (MAE). However, in the meantime, in 1989 he introduced the term Pseudo Lennox syndrome (PLS) using it as the synonym for Atypical benign partial epilepsy (ABPE), the entity defined by Aicardi back in 1982. Aicardi in his book "Epilepsy in children" from 1994 does not describe MAE. In 1995 Doose tried to distinguish MAe from ABPE i.e. PLS emphasising numerous overlapping features of the clinical symptoms and EEG abnormalities. Although Doose himself indicates that it is very difficullt 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. To date we have made a diagnosis of MAE in about 20 children. In every individual child with developed clinical and EEG symptoms, we consider MAE in differential diagnosis ti L-GS, but we only made the final diagnosis of MAE retrospectively, if tje child is finally without seizures and with normal psychomotr development. As in our opinion a problem exists in the 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 for ABPE. We call MAE Doose syndrom.

myoclonic astatic epilepsy; pseudo Lennox syndrome; atypical benign partial epilepsy

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