Education research: neurology residency training in the new millennium.

OBJECTIVE: To survey adult neurology program directors (ANPD) to identify their most pressing needs at a time of dramatic change in neurology resident education. METHODS: All US ANPD were surveyed in 2007 using an instrument adjusted from a 1999 survey instrument. The goal was to characterize current program content, the institution and evaluation of the core competencies, program director characteristics, program director support, the institution of work duty hour requirements, resident support, and the curriculum needs of program directors and programs. RESULTS: A response rate of 82.9% was obtained. There is a significant disconnect between administration time spent by ANPD and departmental/institutional support of this, with ANPD spending approximately 35% of a 50-hour week on administration with only 16.7% salary support. Rearrangement of rotations or services has been the most common mode for ANPD to deal with work duty hour requirements, with few programs employing mid level providers. Most ANPD do not feel work duty hour reform has improved resident education. More residents are entering fellowships following graduation than documented in the past. Curriculum deficiencies still exist for ANPD to meet all Neurology Program Requirements, especially for nontraditional neurology topics outside the conventional bounds of clinical neurology (e.g., practice management). Nearly one quarter of neurology residency programs do not have a meeting or book fund for every resident in the program. CONCLUSIONS: Adult neurology program directors (ANPDs) face multiple important financial and organizational hurdles. At a time of increasing complexity in medical education, ANPDs need more institutional support.

The National Registry of Veterans with amyotrophic lateral sclerosis.

BACKGROUND: The Department of Veterans Affairs (VA) Cooperative Studies Program has established a National Registry of Veterans with Amyotrophic Lateral Sclerosis (ALS). This article describes the objectives, methods, and sample involved in the registry. METHODS: United States military veterans with ALS were identified through national VA electronic medical record databases and nationwide publicity efforts for an enrollment period of 4 1/2 years. Diagnoses were confirmed by medical record reviews. Registrants were asked to participate in a DNA bank. Follow-up telephone interviews are conducted every 6 months to track participants' health status. RESULTS: As of September 30, 2007, 2,400 veterans had consented to participate in the registry, 2,068 were included after medical record review, 995 were still living and actively participating, and 1,573 consented to participate in the DNA bank. 979 participants had been enrolled in the registry for at least 1 year, 497 for at least 2 years, and 205 for at least 3 years. Fourteen studies have been approved to use registry data for epidemiological, observational, and interventional protocols. CONCLUSION: This registry has proven to be a successful model for identifying large numbers of patients with a relatively rare disease and enrolling them into multiple studies, including genetic protocols.

Use of specialized coagulation testing in the evaluation of patients with acute ischemic stroke.

OBJECTIVE: To investigate the use and appropriateness of specialized coagulation tests in the evaluation of patients with acute ischemic stroke and identify factors that influence test ordering. BACKGROUND: Coagulation abnormalities are a rare but recognized cause of ischemic stroke. METHODS: Patient demographics, stroke risk factors, history of venous thrombosis or miscarriage, family history of stroke, and the results of specialized tests for coagulation disorders were recorded for a consecutive series of ischemic stroke patients over age 18 admitted to an academic medical center over 3 years (n = 674). Factors associated with testing were identified with univariate analyses in a random sample of two-thirds of the patients (n = 450). Multivariate logistic regression modeling was then used to identify variables independently associated with testing and then validated in the remaining patients (n = 224). RESULTS: Of the 31% of patients (n = 208) tested for coagulopathies, 29% (n = 60) were tested when the result was unlikely to influence therapeutic decisions. Historical factors associated with an increased incidence of a coagulopathy, such as history of venous thrombosis or miscarriage, were not commonly documented. The absence of small-artery atherosclerosis (OR 0.36, 95% CI 0.17 to 0.76) and younger age (OR 0.89 per year, 95% CI 0.87 to 0.92) were independently related to the frequency of specialized coagulation testing. CONCLUSIONS: One-third of specialized coagulation tests were ordered when the test results were unlikely to affect therapeutic decisions. Age was the only clinical factor increasing the likelihood of a coagulopathy that appeared to influence ordering of specialized coagulation tests.

Apolipoprotein E and neuromuscular disease: a critical review of the literature.

Molecular mechanisms that alter the incidence and rate of neuromuscular disease progression are, in many cases, only partially understood. Several recent studies have asked whether apolipoprotein E (apoE for the protein, APOE for the gene) influences these aspects of specific neuromuscular disorders, as it does in central nervous system disorders such as Alzheimer disease. Although these studies are open to methodological criticism, several interesting trends have emerged. First, the APOE4 allele seems to be associated with an increased risk for developing certain neuromuscular diseases, including diabetic neuropathy and human immunodeficiency viral neuropathy. Second, this allele appears to be associated with faster progression of some neuromuscular diseases, including diabetic neuropathy and possibly motor neuron disease. Third, the APOE2 allele seems to confer protection against developing certain neuromuscular diseases, including the amyotrophic lateral sclerosis (ALS)/parkinsonism/dementia complex of Guam. Finally, this allele is associated with a better prognosis in neuromuscular diseases such as motor neuron disease. The effect of various APOE alleles on neuromuscular diseases therefore parallels their influence on central nervous system diseases. Arch Neurol. 2000;57:1561-1565

Cyclosporin A-related cerebral vasculopathy.

The use of cyclosporin A has been associated with several side-effects, including neurotoxicity. The mechanism of toxicity is not well known. We report two patients treated with cyclosporin A who developed lesions in the cerebral white matter associated with abnormally elevated cerebral blood flow velocities on transcranial doppler ultrasound and abnormal vascular appearance on magnetic resonance angiography. Bone Marrow Transplantation (2000) 26, 801-804.

Ulnar nerve function following total elbow arthroplasty: a prospective study comparing preoperative and postoperative clinical and electrophysiologic evaluation in patients with rheumatoid arthritis.

A study was conducted to determine the incidence of ulnar and peripheral neuropathy in patients with rheumatoid arthritis undergoing total elbow arthroplasty and the effect it has on ulnar nerve function after surgery. Preoperative and postoperative clinical and electrodiagnostic examinations were completed in 10 patients. Before surgery 4 patients had clinical and electrophysiologic evidence of a neuropathy (2 each with a peripheral neuropathy and an ulnar neuropathy). One patient had subclinical evidence of a chronic T-1 radiculopathy. After surgery 2 patients showed neurologic improvement (1 had ulnar neuropathy and 1 had diabetic neuropathy). One patient who had normal test results before surgery developed transient ulnar sensory symptoms after surgery. An electrodiagnostic study confirmed an ulnar neuropathy that was not detected on physical examination; the electrodiagnostic findings improved 4 months later. We found that a large percentage of patients (40%) with rheumatoid arthritis had evidence of ulnar or peripheral neuropathy before surgery. The presence of an ulnar or peripheral neuropathy did not predispose patients to develop postoperative ulnar nerve dysfunction either clinically or electrophysiologically. Preoperative and postoperative physical and electrodiagnostic examination results correlated in 9 of the 10 patients.

Initial evaluation of suspected dementia. Asking the right questions.

The initial clinical approach to suspected dementia involves asking the right questions of the patient and a knowledgeable family member or caregiver. These questions should focus on the nature of the cognitive problem (e.g., the onset, duration, and severity of memory difficulties). The clinical interview is supplemented with thorough examination of the patient's physical, neurologic, mental, and functional status. Once a diagnosis of dementia is established, the underlying cause should be identified by a standardized workup, including laboratory testing and, in most cases, neuroimaging. Genetic testing for the APOE epsilon 4 allele can help confirm the diagnosis in patients with late-onset familial or sporadic Alzheimer's disease.

Update on Alzheimer's disease. Promising advances in detection and treatment.

In the past decade, significant progress has been made in the understanding and treatment of Alzheimer's disease. Modest success has been achieved using new drugs in treatment of mild symptoms of the disease. Vitamin E, estrogen, and NSAIDs may slow the progression of Alzheimer's disease, but their routine use is not without risk, and there is no evidence that these agents are effective in preventing the disease in asymptomatic people. With our increased understanding of the pathogenesis of Alzheimer's disease, it is likely that the next decade will see further refinement and development of treatment strategies to halt progression and possibly reduce the incidence of disease.

Determining the cause of memory loss in the elderly. From in-office screening to neuropsychological referral.

Improved understanding of neurobehavior in normal aging, Alzheimer's disease, and late-life depression makes early detection of neurodegenerative conditions possible. Primary care physicians can screen patients' mental status and mood states with simple in-office tests. When screening results or the clinical picture is ambiguous or complex, neuropsychological evaluation is useful in making an early, reliable differentiation between dementia and normal aging. Early identification of neurologic problems provides an opportunity to enhance quality of life and long-term care. Medical interventions, such as a trial of donepezil hydrochloride (Aricept) or other memory-enhancing medications as they become available, can be started when results are likely to be optimal. Common coexisting problems (e.g., depression, falls) can be sought and managed. Additional important medical decisions (e.g., elective surgeries) may be considered differently when dementia is diagnosed early.

Managing common behavioral problems in dementia. How to improve quality of life for patients and families.

Dementia is the most common reason for nursing home placement, and related behavioral symptoms are the primary factors precipitating the decision. Disruptive behaviors such as depression, sleep disturbance, agitation, aggression, and psychosis can tax family members' abilities to care for a loved one who has reached that stage of the illness. Working with families to institute effective management strategies may help delay institutionalization. In this article, Drs Burke and Morgenlander present a systematic approach to identifying and treating common problems in dementia.

Electron microscopic diagnosis of human flavivirus encephalitis: use of confocal microscopy as an aid.

The distinction between intracranial viral infections and inflammatory conditions requiring immunosuppression is important. Although specific laboratory reagents are readily available for some viruses, diagnosis of arbovirus infection is more difficult. Transmission electron microscopy (TEM) theoretically allows identification of viral particles independent of reagent availability, but it has limited sensitivity. We report two cases of human flavivirus encephalitis diagnosed by TEM. Laser scanning confocal microscopy (LSCM) was used in one case to survey unembedded tissue slices for focal abnormalities, from which fragments smaller than 1 mm2 were excised for epoxy embedding. This facilitated TEM identification of intracytoplasmic, budding, 35-40 nm spherical virus particles, confirmed by serology as St. Louis encephalitis. In contrast to mosquitoes and newborn mice, in which high viral loads are associated with minimal tissue responses, these biopsies showed florid angiodestructive inflammation and microgliosis, with rare virions in necrotic perivascular cells and astrocytes. To our knowledge, this represents the first ultrastructural study of St. Louis encephalitis in humans, indicating the potential value of LSCM-aided TEM.

Transient lumbosacral polyradiculopathy after prostatectomy: association with spinal stenosis.

Mononeuropathies are common after pelvic surgery. They are usually the result of unnatural positioning during surgery or faulty restraining devices. Polyneuropathy in the postoperative setting is rare. We report two cases of polyradiculopathy after radical prostatectomy using two different patient positions. Both patients complained of paresthesias and weakness in their lower extremities on postoperative day 1. Neurologic examination in each case was consistent with a polyradiculopathy. Significant spinal stenosis of the lumbosacral spine was found in both patients by magnetic resonance imaging. We propose that spinal stenosis is a risk factor for this type of neurologic injury.

Intravascular malignant lymphomatosis with neurologic presentation: factors facilitating antemortem diagnosis.

Intravascular malignant lymphomatosis (IML) is a rare disorder of small and medium size vessels that frequently goes undiagnosed until the time of autopsy. The clinical courses of two such patients were examined to determine factors that would facilitate antemortem diagnosis. Both patients had mental status changes, pyramidal tract signs, and peripheral neuropathy. Despite postmortem evidence of widespread lymphocytic invasion of vessels throughout the body including peripheral and central nervous systems, neuroimaging studies, cerebrospinal fluid analysis, peripheral blood studies, and bone marrow biopsy failed to reveal diagnostic evidence of the underlying neoplastic process. Although markedly abnormal, nerve conduction studies were nonspecific. Familiarity with IML and its consideration in the differential diagnosis when central and peripheral nervous system dysfunction occur concurrently may guide the physician to tissue biopsy facilitating antemortem diagnosis and institution of appropriate therapy.

Peripheral neuropathy and connective tissue disease. Watch for this twosome when hunting for a diagnosis.

Coexistent peripheral neuropathy and connective tissue disease is fairly common, possibly because immune-mediated factors are often present in both disorders. Awareness of the association between the two conditions can be important during diagnostic evaluation, because neuropathy is sometimes the presenting feature of previously unrecognized connective tissue disease. In this article, the authors examine clinical findings in vasculitic, nonvasculitic, and treatment-related neuropathy, concentrating on the essentials of diagnosis and treatment.

Peripheral neuropathy in patients with chronic renal failure. A treatable source of discomfort and disability.

Uremic polyneuropathy occurs in about half of patients undergoing dialysis and is characterized by axonal degeneration with secondary segmental demyelination. Hemodialysis or peritoneal dialysis halts the progress of polyneuropathy but usually does not bring improvement. However, improvement invariably occurs with successful renal transplantation. Mononeuropathies resulting from placement of forearm arteriovenous fistulas are also seen and include the commonly encountered carpal tunnel syndrome and the rare but catastrophic ischemic monomelic neuropathy. The latter constitutes a medical emergency; immediate surgical closure of the fistula is required to avoid severe and permanent neurologic dysfunction.

Surgical treatment of carpal tunnel syndrome in patients with peripheral neuropathy.

Outcome after carpal tunnel surgery was studied retrospectively in 32 patients with peripheral neuropathy and carpal tunnel syndrome. Nocturnal paresthesias were almost universally relieved, followed in order of responsiveness by pain, numbness, and weakness. Twenty-five of 28 patients said they would have the surgery again if the outcome were the same. Patients with carpal tunnel syndrome and peripheral neuropathy benefit from surgical treatment of carpal tunnel syndrome.

Recognizing peripheral neuropathy. How to read the clues to an underlying cause.

Several systemic diseases, including some common ones (eg, diabetes, rheumatoid arthritis, thyroid disease) can cause symptoms of peripheral nerve dysfunction. Efficient identification and treatment of the underlying disorder can avoid costly and unnecessary testing. Patients' descriptions of symptoms and their onset, specific deficits found on physical examination, and family and medical history provide many clues to the cause of the neuropathy. Nerve-conduction studies, electromyography and, when necessary, nerve biopsy serve as complements to clinical evaluation.