Dysferlin has recently been identified as a novel gene involved in limb–girdle muscular dystrophy type 2B (LGMD2B) and its allelic disease, Miyoshi myopathy. The predicted structure of dysferlin suggests that it is a transmembrane protein possibly involved in membrane fusion. Thus, unlike previously identified structural proteins in muscular dystrophy, dysferlin is likely involved in a novel pathogenic mechanism for this disease. In this study, we have analyzed the expression of dysferlin in skeletal muscle of patients with disruptions in the dystrophin–glycoprotein complex and patients with a clinical diagnosis of LGMD2B or Miyoshi myopathy. We show expression of dysferlin at the sarcolemma in normal muscle and reduced sarcolemmal expression along with accumulation of intracellular staining in dystrophic muscle. Electron microscopy in Miyoshi myopathy biopsies suggests that the cytoplasmic staining could be a result of the abundance of intracellular vesicles. Our results indicate that dysferlin expression is perturbed in LGMD and that both mutations in the dysferlin gene and disruption of the dystrophin–glycoprotein complex can lead to the accumulation of dysferlin within the cytoplasm. Ann Neurol 2000;48:902–912

Summary:Purpose: To determine the prevalence, manifestations, lateralizing value, and surgical prognostic value of somatosensory auras (SSAs) in patients with refractory temporal lobe epilepsy (TLE).Methods: Eighty-one consecutive patients undergoing temporal lobectomy for refractory complex-partial seizures were screened for SSAs. The characteristics of the somatosensory phenomena, occurrence of other aura types, seizure semiology, findings of EEG and imaging studies, temporal lobe neuropathology, and postoperative seizure outcome were determined in each patient with SSAs.Results: Nine (11%) of 81 patients with refractory temporal lobe seizures reported distinct SSAs as part of their habitual seizures. The most common manifestation of SSAs was tingling (eight of nine, 89%), but sensory loss (one of nine, 11%) and pain (one of nine, 11%) also were reported. Five patients had unilateral somatosensory symptoms, and four patients had bilateral somatosensory symptoms. Seizure origin was in the contralateral temporal lobe in four (80%) of five patients with unilateral SSAs, including all patients with unilateral SSAs affecting a limb. Partial temporal lobe resection produced complete seizure remission in all nine (100%) patients 1 year after surgery and in seven (78%) of nine patients 2 years after surgery.Conclusions: SSAs occur more frequently than previously appreciated in patients with refractory temporal lobe seizures and usually manifest as either unilateral or bilateral tingling. In patients with temporal lobe seizures, unilateral SSAs involving a limb suggest a seizure origin in the contralateral temporal lobe. The surgical outcome of TLE patients with SSAs is favorable. Thus the presence of SSAs should not serve as a deterrent to temporal lobe resection in patients with clearly defined TLE.

Epilepsy surgery outcomes in the U.S. military are similar to those reported from nonmilitary centers, with the majority of patients experiencing seizure remission and improvements in quality-of-life measures. Complete seizure remission after successful anterior temporal lobectomy enables some active duty military members to continue service in the U.S. Armed Forces.

Objective: Changes in health care delivery required substitution of a number of alternatives for the traditional inpatient clerkship used in the neurology education of fourth-year medical students at the Uniformed Services University of the Health Sciences and for third-year medical students from Georgetown University. Methods: We retrospectively analyzed grades on a locally generated multiple-choice examination based on a student objective list. Scores from students rotating on ambulatory neurology, neurosurgery, child neurology, neurorehabilitation, and rotations at other military hospitals over a 2-year period were compared with those achieved by students in a traditional clerkship at Walter Reed Army Medical Center. Results: There were no significant differences in the grades between any of the groups. Conclusions: Student acquisition of factual material was not influenced by the type of clinical experience or by whether the student is in the third or fourth year of medical school.