The Landau-Kleffner syndrome or 'acquired aphasia with convulsive disorder'. Long-term follow-up of six children and a review of the recent literature
P. F. Paquier, H. R. Van Dongen and C. B. Loonen
Department of Neurology, Antwerp, Belgium, University Hospital.
We present six patients with acquired aphasia with convulsive disorder
(Landau-Kleffner syndrome) and distill the main clinical features from a
review of the recent literature. Our series showed that the clinical
picture can vary at onset, as well as during the course of the illness, and
that the long-term outcome of the aphasia is quite unpredictable, despite
the fact that epilepsy and electroencephalographic abnormalities usually
regress or disappear with the years. We also call attention to the
electroencephalographic phenomenon of electrical status epilepticus during
slow sleep, and we suggest that the course of the aphasia may well be
linked to the appearance and disappearance of electrical status epilepticus
during slow sleep. Therefore, we recommend a sleep electroencephalogram in
all children with Landau-Kleffner syndrome. Finally, our findings did not
demonstrate the beneficial effect of treatment with anticonvulsants on the
aphasia, but recent studies have shown that treatment with corticosteroids,
whether combined with anticonvulsants, is effective.
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ABSTRACT
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J Child Neurol 1999;14:696-701.
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J Child Neurol 1998;13:296-300.
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