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Quantitative Assessment of Cortical Auditory-tactile Processing in Children with Disabilities

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Defective auditory processing in a child with temporal epileptic focus.

Avinoam Shuper¹, Mordechai Medvedovsky², Sara Kivity³

¹Department of Pediatric Neurology and Epilepsy Center, Schneider Children's Medical Center of Israel, Petah Tiqva, Israel, affiliated to the Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel avinoams@clalit.org.il.

Journal of Child Neurology

|May 3, 2014

Summary

This summary is machine-generated.

A child

Keywords:

Landau-Kleffner hyperacusis temper tantrums temporal epileptic focus

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Area of Science:

Pediatric Neurology
Neurophysiology
Epileptology

Background:

Landau-Kleffner syndrome (LKS) is a rare neurological disorder characterized by acquired epileptic aphasia.
The spectrum of LKS may include atypical presentations beyond language deficits.

Observation:

A 9-year-old boy exhibited severe hyperacusis (noise intolerance) triggering temper tantrums and social withdrawal.
These episodes presented with features suggestive of complex partial seizures.

Findings:

Electroencephalography (EEG) and magnetoencephalography (MEG) identified a left temporal epileptic focus (T5).
Genetic analysis excluded GRIN2A mutations, a gene sometimes associated with similar conditions.

Implications:

This case suggests hyperacusis can be a variant presentation within the Landau-Kleffner spectrum.
Further research is needed to understand the neurobiological underpinnings of such presentations.