We describe a new syndrome of familial temporal lobe epilepsy in 38 individuals from 13 unrelated white families. The disorder was first identified in 5 concordant monozygotic twin pairs as part of a large-scale twin study of epilepsy. When idiopathic partial epilepsy syndromes were excluded, the 5 pairs accounted for 23% of monozygotic pairs with partial epilepsies, and 38% of monozygotic pairs with partial epilepsy and no known etiology. Seizure onset for twin and nontwin subjects usually occurred during adolescence or early adult life. Seizure types were simple partial seizures with psychic or autonomic symptoms, infrequent complex partial seizures, and rare secondarily generalized seizures. Electroencephalograms revealed sparse focal temporal interictal epileptiform discharges in 22% of subjects. Magnetic resonance images appeared normal. Nine affected family members (24%) had not been diagnosed prior to the study. Pedigree analysis suggested autosomal dominant inheritance with age-dependent penetrance. The estimated segregation ratio was 0.3, indicating an overall penetrance of 60% assuming autosomal dominant inheritance. The mild and often subtle nature of the symptoms in some family members may account for lack of prior recognition of this common familial partial epilepsy. This disorder has similarities to the El mouse, a genetic model of temporal lobe epilepsy with a major gene on mouse chromosome 9, which is homologous with a region on human chromosome 3.

Patients with dementia often manifest troublesome and disruptive behaviors in addition to intellectual impairments. This study evaluated behavioral disturbances in 126 demented patients examined sequentially, using questionnaires administered to primary caregivers to quantify the types and severity of behavioral disturbances. Eighty-three percent of the patients exhibited one or more of the targeted behaviors. The most common troublesome and disruptive behaviors clustered into three categories: aggressive, ideational, and vegetative. The prevalence and severity of the behaviors increased with global severity of dementia, but did not differ in either frequency or type when patients with three diagnoses were compared: Alzheimer's disease (AD), multi-infarct dementia (MID), and mixed AD and MID (MIX). The occurrence and severity of the target behaviors correlated modestly with the severity of dementia. Impairments of mental status correlated weakly with only a single troublesome and disruptive behavior--assaultiveness. These results suggest that troublesome and disruptive behaviors are a very frequent component of dementing disorders, are related to disease severity, and parallel (but are probably not determined by) intellectual deficit.

The COBRA scale provides a convenient, comprehensive, and reliable means for caretakers to identify the types and measure the severity of OBs in demented outpatients and nursing home inpatients. If additional studies confirm these observations, COBRA will be a useful instrument for assessing the effects of interventions on OBs in patients with dementia.

A 45-year-old man with no significant medical history presented following an electric current injury (380 V). He developed multiple systemic injuries including third degree burns and after 1 week of hospitalisation he reported unilateral visual changes. Examination suggested the presence of optic nerve oedema without evidence of haemorrhage, exudate or vessel abnormality. This was considered to be related to the electric shock. A trial of corticosteroids was considered. He was followed up to 5 months in clinic and was noted to have developed unilateral optic atrophy and no other systemic manifestations. Initial and 5 months follow-up optic nerve colour photograph and optical coherence topography were documented. The present case highlights the fact that electric current injury can present with only a unilateral ischaemic optic neuropathy, the need for early diagnosis for timely treatment and the controversial role of corticosteroids.

Electing to continue formal medical education through a fellowship is an important career decision. Unlike finding a residency, the process of finding fellowships and the accreditation status of fellowships are not standardized. This article attempts to clarify the advantages and disadvantages of fellowships in neurology, as well as to describe how to learn of subspecialties that offer fellowships, fellowship availability, and accreditation systems. BACKGROUND After residency, fellowship is often the next step in formal graduate medical education. Approximately 74% of neurology residents pursue fellowships. 1 Unlike the process for finding internship and residency positions, the process and timetable for seeking out, interviewing, evaluating, and, finally, selecting a fellowship depend on the type of fellowship and the institution. There is no national match for fellowships, no standardization of fellowship applications, no centralized list of fellowship positions, and no single accreditation body for all fellowships. Fellowships offer one a competitive edge when applying for private practice positions. In fact, subspecialty fellowship training is often a requirement for private practice or academic positions. Fellowships also offer one a chance to delve into a field and to gain clinical and laboratory experience in a subspecialty area. Many fellowships offer the opportunity to try writing and teaching, thus providing a chance to experience some aspects of academic neurology.