Landau-Kleffner Syndrome: the challenges of its treatment

Case presentation: A previously healthy 3-year-old boy presented with difficulty understanding commands and developed impairment of spoken language that lasted four months and remitted spontaneously. After 10 months, he suffered again language regression. Electroencephalogram (EEG) revealed electrical status epilepticus during sleep (ESES). He was treated with sulthiame, sodium valproate and clobazam with partial improvement. One year later, after an episode of COVID-19 infection, symptoms worsened and he was prescribed lamotrigine, levetiracetam and low dose prednisolone with partial response. After a few months, following an episode of otitis, he became severely ataxic and completely aphasic and searched our hospital. He was unable to sit and stand without support and also presented few episodes of absences. EEG persisted with ESES. Brain MRI was normal. He was started on 5 days of methylprednisolone and 5 days of immunoglobulin. By the time he was discharged, he was already able to speak a few words and walk independently. We decided to perform monthly steroid pulse therapy and associated ketogenic diet. After the third month of treatment, he showed significant EEG improvement and ESES was not observed. After the fifth course of methylprednisolone, he was able to surf and skateboard and evolved with marked speech improvement. He is currently on steroid tapering and his last EEG shows rare centrotemporal paroxysms.

Discussion: Landau Kleffner Syndrome (LKS) and epilepsy with continuous spike- waves during slow-wave sleep (CSWS) comprise a spectrum of diseases with strong activation of interictal epileptiform activity during sleep. LKS is a rare epileptic encephalopathy characterized by language regression and behavior abnormalities. Seizures occur in 70 - 85% of patients. It usually manifests in 3-to 8-year-old children with normal development. There are no controlled clinical trials investigating the therapeutic options for LKS and some authors have reported the use of immunotherapy with variable effects. We present a case of dramatic response with steroid pulse therapy, immunoglobulin and ketogenic diet.

Final comments: LKS should be suspected in previously healthy children with subacute aphasia and seizures. Treatment is challenging and literature on the subject is scarce. Although the etiology is not well stablished, the potential involvement of the immune system could justify the response to steroids. We suggest that pulse therapy with methylprednisolone and ketogenic diet should be considered.

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Publikationsverlauf

Artikel online veröffentlicht:
18. September 2023