[The Landau-Kleffner syndrome. SPECT and EEG investigations]

Area of Science:

• Pediatric Neurology
• Developmental Neuroscience
• Clinical Electrophysiology

Background:

• Acquired epileptic aphasia, also known as Landau-Kleffner syndrome (LKS), is a rare neurological disorder.
• LKS is characterized by sudden or gradual loss of language abilities in children with previously normal speech and hearing.
• The syndrome presents significant diagnostic and management challenges in developmental neurology.

Purpose of the Study:

• To describe the clinical presentation and electroencephalographic (EEG) dynamics in four children diagnosed with Landau-Kleffner syndrome.
• To investigate potential correlations between clinical symptom fluctuations and EEG findings.
• To explore cerebral changes using single photon emission computed tomography (SPECT) during periods of clinical remission.

Main Methods:

• Case series observation of four pediatric patients with LKS.
• Longitudinal monitoring of clinical symptoms and neurological examinations.
• Serial electroencephalography (EEG) to assess brain electrical activity.
• Single photon emission computed tomography (SPECT) imaging during clinical remission phases.

Main Results:

• Observed significant fluctuations in both clinical symptoms and EEG patterns over time.
• Demonstrated variability in the dynamic of acquired epileptic aphasia.
• Identified specific brain changes through SPECT imaging correlating with remission periods.

Conclusions:

• Landau-Kleffner syndrome exhibits dynamic clinical and electrophysiological characteristics.
• SPECT imaging may reveal underlying cerebral alterations associated with LKS remission.
• Further research is warranted to elucidate the pathophysiology and optimize treatment strategies for LKS.