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CLINICAL PRESENTATION AND DIAGNOSIS

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Acquired epileptic aphasia1 is an epileptic syndrome described in the International Classification of Epilepsies by the eponym Landau–Kleffner syndrome (LKS). Typical LKS2,3 is part of the epileptic encephalopathy of late childhood defined by1: age of onset ranging from 3 to 10 years in children with previously normal language development;2 insidious or abrupt acquired aphasia with verbal auditory agnosia; behavior disturbances (attention deficit and hyperactivity);3 seizures that may be nocturnal, focal motor, secondarily generalized, or atypical absences with an awake electroencephalographic (EEG) showing focal or multifocal spikes-and-waves predominantly over the temporal regions (Fig. 30–1);4 and sleep EEG reveals activation of interictal EEG abnormalities and, during the course of the syndrome in the acute phase, nonrapid eye movement (NREM) subcontinuous or continuous spike-waves during sleep (CSWS) (Fig. 30–1).

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Figure 30–1.

Awake (A) and sleep (B) EEG in 6-year-old girls with auditory agnosia in a typical case of Landau–Kleffner syndrome.

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Typical LKS is not related to nonconvulsive status epilepticus, which presents as a subacute progressive aphasia in adults,4 or acquired lesions with seizures such as cysticercosis5 or astrocytoma.6 LKS represents a subtype of idiopathic focal epilepsies with acquired cognitive deficits.7,8 Other subtypes of idiopathic focal epilepsies related to LKS are described by several terms in the literature: acquired opercular syndrome with epilepsy, frontal epilepsy with or without autistic regression, atypical partial benign epilepsy or pseudo-Lennox–Gastaut syndrome, and idiopathic or symptomatic CSWS8 (Table 30–1)

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TABLE 30–1.COMPARISON OF DIAGNOSIS AND TREATMENTS OF LANDAU–KLEFFNER SYNDROME WITH RELATED EPILEPTIC SITUATIONS

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