Benign adult familial myoclonic epilepsy is an autosomal dominant idiopathic epileptic syndrome characterized by adult-onset tremulous finger movement, myoclonus, epileptic seizures, and nonprogressive course. It was recently recognized in Japanese families. In this study, we report that the gene locus is assigned to the distal long arm of chromosome 8, by linkage analysis in a large Japanese kindred with a maximum two-point LOD score of 4.31 for D8S555 at recombination fraction of 0 (maximum multipoint LOD score of 5.42 for the interval between D8S555 and D8S1779). Analyses of recombinations place the locus within an 8-cM interval, between D8S1784 and D8S1694, in which three markers, D8S1830, D8S555, and D8S1779, show no recombination with the phenotypes. Although three other epilepsy-related loci on chromosome 8q have been recognized-one on chromosome 8q13-21 (familial febrile convulsion) and two others on chromosome 8q24 (KCNQ3 and childhood absence epilepsy)-the locus assigned here is distinct from these three epilepsy-related loci. This study establishes the presence of a new epilepsy-related locus on 8q23.3-q24.11.

Summary:Purpose: To evaluate further the relevance of designating psychotic episodes as either postictal or interictal, we compared several biologic variables between epilepsy patients with and without psychosis.Methods: The study subjects comprised 282 patients with psychosis (36 with postictal psychosis, 224 with interictal psychosis, and 22 with both postictal and interictal psychoses, i.e., bimodal psychosis), and 658 epilepsy patients without psychosis. The clinical characteristics of these patients were reviewed retrospectively by experienced neuropsychiatrists. Factors predicting the development of each type of psychosis were determined by serial multivariate logistic regression analyses.Results: Factors that were comparable between postictal and interictal psychoses were intellectual function, family history of psychosis, epilepsy type, and the presence of complex partial seizures. In contrast, age at the onset of epilepsy and at the onset of psychosis and the presence of generalized tonic-clonic seizures differed for the three types of psychosis. Patients with bimodal psychosis showed characteristics associated with both postictal and interictal psychoses.Conclusions: This study documented conditions, including both general factors and epilepsy-related factors, common to epilepsy patients with psychosis, regardless of chronologic distinctions. Certain epileptic processes appear to have equal influence on postictal and interictal psychoses. However, some differences between postictal and interictal psychoses suggest that these chronologic descriptors are valid. Our findings confirmed that psychosis associated with epilepsy should not be defined as a single, simple condition but rather as a complex condition with several possible subcategories.

A family history of psychosis, earlier age at onset of epilepsy, complex partial seizures or generalized tonic clonic seizures, and borderline intellectual functioning were the most important predictors for development of interictal psychosis.

Background and purposeAutomated subfield volumetry of hippocampus is desirable for use in temporal lobe epilepsy (TLE), but its utility has not been established. Automatic segmentation of hippocampal subfields (ASHS) and the new version of FreeSurfer software (ver.6.0) using high-resolution T2-weighted MR imaging are candidates for this volumetry. The aim of this study was to evaluate hippocampal subfields in TLE patients using ASHS as well as the old and new versions of FreeSurfer.Materials and methodsWe recruited 50 consecutive unilateral TLE patients including 25 with hippocampal sclerosis (TLE-HS) and 25 without obvious etiology (TLE-nonHS). All patients and 45 healthy controls underwent high-resolution T2-weighted and 3D-volume T1-weighted MRI scanning. We analyzed all of their MR images by FreeSurfer ver.5.3, ver.6.0 and ASHS. For each subfield, normalized z-scores were calculated and compared among groups.ResultsIn TLE-HS groups, ASHS and FreeSurfer ver.6.0 revealed maximal z-scores in ipsilateral cornu ammonis (CA) 1, CA4 and dentate gyrus (DG), whereas in FreeSurfer ver.5.3 ipsilateral subiculum showed maximal z-scores. In TLE-nonHS group, there was no significant volume reduction by either ASHS or FreeSurfer.ConclusionsASHS and the new version of FreeSurfer may have an advantage in compatibility with existing histopathological knowledge in TLE patients with HS compared to the old version of FreeSurfer (ver.5.3), although further investigations with pathological findings and/or surgical outcomes are desirable.

We report the findings of single photon emission computed tomography using 123 I-IMP and magnetic resonance image studies of five patients with Charles Bonnet syndrome (CBS) while they were having visual hallucinations. All patients developed complex visual hallucinations after suffering from eye disease. The mean age at onset of CBS was 71.6 years. Single photon emission computed tomography studies in all patients disclosed hyperperfusion areas with some asymmetrical appearances in the lateral temporal cortex, striatum and thalamus. These results suggest that when elderly people suffer from eye disease, subsequent excessive cortical compensation in the lateral temporal cortex, striatum and thalamus may precipitate the development of visual hallucinations.