Background:
@EN The Landau-Kleffner syndrome, or acquired epileptic aphasia is a relatively rare disorder characterized by epilepsy and progressive language loss with an apparent loss of auditory verbal understanding (agnosia) and speech. We carried out the
clinical study atudy about it to know its clinical characteristics.
@ES Methods:
@EN During the past five years (from March 1991 to February 1996), six patients were diagnosed as Landau Kleffner syndrome at the department of Pediatrics of Asan Medical Center and the Department of Pediatrics of Seoul National University
Hospital
in
Korea. We reviewed their clinical manifestations and courses, EEG, Brain MRI, Brain-Stem Auditory Evoked Response (BAER), and Brain Positron Emission Tomography (PET) findings.
@ES Results:
@EN Patients were all male children. Their seizures had begun between 8 months and 5 years and 11 months of age, and sensory aphasia between 12 months and 6 years and 2 months of age. EEG showed both, left or right temporal spike or sharp wave
discharges. The findings of brain MRI and BAER at the time of diagnosis wer enormal or unexplainable, but brain PET (checked in two patients) disclosed decrease of cerebral glucose utilization involving btoth temporo-parietal in one patient and
diffuse
cortical areas in another. During the follow-up period (1 to 5 years), the seizure remains intractable in 3 cases (50%) and aphasia persists in 5 cases (83.3%).
@ES Conclusion:
@EN As the onset of Landau-Kleffner syndrome begins in childhood and its course is not benign, especially in aphasia, so the disease have much possibility of an obstacle to becoming a normal adult. In addition to it, we confirmed the possibility
of
an
utility of brain PET to make an early diagnosis and understand the pathophysiology of Landau-Kleffner syndrome.
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