Abstract

Background Landau Kleffner syndrome (LKS) is well defined epileptic encephalopathy best described by the synonym – acquired epileptic aphasia. Etiopathogenesis remains unknown. Structural lesions are rare. A few published cases postulated triggering role of head trauma in functional disconnection of cortical speech areas. Objective To evaluate the clinical presentation and management of a boy with LKS following a minor head trauma. Case report and results A ten years old boy of far consanguineous parents, at his age of six developed a generalized tonic clonic seizure following a head trauma two weeks before upon fall. The immediate period was uneventful, the child had only nose bleeding, brain CT was normal. However, consequent EEG showed ESES and he was started on valproates. Noncompliance led to another seizure six months later. Over that period, gradual regression in the ability to use or comprehend spoken language was noted. EEG showed more or less pronounced bilateral ESES affecting predominantly left temporo-parietal regions. Perinatal period and developmental before the onset of the illness were normal. Family history was unremarkable. Metabolic work up was normal. MRI brain done twice was unremarkable. Neurological status, apart from aphasia, was normal. Parents reported impulsivity and cognitive decline. He had one episode of focal seizure two years since the onset, but further has remained controlled. He went through several antiepileptic drugs combinations including valproates, clobazam, levetiracetam, clonazepam and lamotrigine. Ethosuximide was stopped due to intolerance. Two trials with oral prednisolone: the first lasting four months, complicated by hypertension, the second, of one month prednisolone with evening diazepam, resulted in transient clinical and EEG moderate improvement. Ketogenic diet was recommended. Conclusion Aphasia, inspite of various therapeutical attempts and favorable seizure control, remained the main concern. Corticosteoids transiently and partially improved the condition. Possible role of head trauma in LKS etiopatogenesis needs further consideration.

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