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A retrospective single-blind study was carried out to assess the reliability of magnetic resonance imaging (MRI) for determining lateralization of the electrographic focus in 45 patients with intractable temporal lobe epilepsy. With strictly defined MRI diagnostic criteria, the electroencephalographic (EEG) focus was correctly lateralized in 86% of patients. Excluding patients with structural lesions, the criteria provided for correct lateralization of the epileptogenic focus in 78% and false lateralization in 5%. Hippocampal atrophy on T1-weighted images and increased signal intensity from mesial structures on T2-weighted scans were highly reliable for lateralization. Postoperative outcome did not differ between the patients with normal and those with abnormal findings on MRI, but the group sample was inadequate to assess the issue of surgical outcome. These findings suggest that with appropriate techniques and strictly defined diagnostic criteria, MRI can provide reliable seizure lateralization in patients with intractable temporal lobe epilepsy.

Topiramate had well-documented efficacy as an adjunctive agent for partial-onset seizures in 5 double-blind, parallel trials, reducing seizure frequency by 35-47% at 400 mg/day, the optimal dose for most patients. It can also be used as monotherapy for partial-onset seizures. A broad spectrum of action is suggested by studies showing efficacy against a variety of generalized-onset seizure types, including primary generalized tonic-clonic seizures and the component seizures of Lennox-Gastaut syndrome. The major adverse effects of topiramate are of CNS origin. Psychomotor slowing is most common, but disappears in most patients with time or dosage adjustments; 11-28% of patients discontinue the drug because of side effects. No serious skin, liver or blood toxicity has been identified. Efficacy and adverse effect profiles are similar for children and adults. Introduction at rates not exceeding 25-50 mg increases per week will minimize adverse effects and be satisfactory for most adults.

We identified a large Georgia kinship in which a hereditary autosomal dominant chorea appeared at an average age of 65 years, much later than usual for Huntington's chorea. Progression was slow. Dementia was not an obvious initial feature. Family members denied that affected persons became demented, and those affected cognitively intact with bedside testing. However, deficits of memory were apparent on formal psychologic testing. In the propositus' generation, five of 12 siblings were affected during their 60s. Of 23 persons in a younger generation, aged 30 to 60 years, none was yet affected. A number of variants of Huntington's chorea have been proposed. In elderly patients without obvious dementia, psychologic testing may be of diagnostic importance, revealing characteristic memory deficits. When the disorder consistently occurs at an advanced age and progresses slowly, the implications for the family may be less grave than with Huntington's chorea of earlier onset.