Mesial TLE with HS as its cause is not recognized in the classification of epileptic syndromes (3) (see Chapter 1). It is nonetheless an important entity that has a special significance because of the potential for surgical cure of a psychosocially and medically disabling illness.
If there is a syndrome deserving special attention, it is the following. Patients have an increased familial incidence of febrile convulsions in early childhood. Typically, patients have a history of complicated febrile seizures during the first years of life. After a silent period lasting from 3 to 20 years, patients develop complex partial seizures. At first, seizures may be controlled on anticonvulsants, but with time, intractable seizures become the dominant feature. The clinical symptoms include visceral and experiential auras, with other auras less commonly seen. The patients often report an aura followed by arrest of activity and clouding of consciousness. Oroalimentary and manual automatisms followed by contralateral dystonic posturing of the upper extremity are often present. Postictal confusion with dysphasia (dominant hemisphere) are also prominent.
Neurologic evaluation usually reveals no focal neurologic findings, although studies have reported facial asymmetries in patients with mesial temporal sclerosis (381). Material specific memory deficits can sometimes be detected using sophisticated neuropsychologic testing, particularly when the epilepsy arises in the dominant hemisphere.
Electroencephalographic studies demonstrate interictal sharp and slow wave activity from the anterior temporal regions. Interictal activity is usually increased during stage 1 sleep and may become bilateral with slow sleep stages. Ictal scalp recordings usually show lateralizing rhythmic theta activity, and with intracranial recordings focal discharges originating in the hippocampus and amygdala are commonly seen.
Imaging features include the classical MRI abnormalities described in this chapter and functional studies (PET and SPECT) demonstrate metabolic and blood flow pattern abnormalities showing lateralized defects involving the abnormal temporal lobe (chapter PET and SPECT).
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