| A type of temporal lobe epilepsy that presents typically between 10 and 30 years (range 0.5 to 54 years) with focal aware sensory auditory seizures and/or focal cognitive seizures with receptive aphasia. Auditory seizures typically consist of simple unformed sounds (e.g., humming, buzzing, or ringing), or less commonly auditory distortions (such as alteration in volume) or complex sounds (e.g., specific songs or voices). Cognitive seizures manifest as receptive aphasia, consisting of an inability to understand spoken language in the absence of an impairment of awareness. Focal impaired awareness and focal to bilateral tonic-clonic seizures may occur. Development and cognition are typically normal. Neurological examination is normal. The interictal EEG is normal but may have focal (usually temporal) sharp-and-wave or spikes, that can also be widespread. MRI is normal or shows focal cortical dysplasia. |
