Objective: To compare accuracy of ultrasound and MRI for detecting focal peripheral nerve pathology, excluding idiopathic carpal or cubital tunnel syndromes.Methods: We performed a retrospective review of patients referred for neuromuscular ultrasound to identify patients who had ultrasound and MRI of the same limb for suspected brachial plexopathy or mononeuropathies, excluding carpal/cubital tunnel syndromes. Ultrasound and MRI results were compared to diagnoses determined by surgical or, if not performed, clinical/electrodiagnostic evaluation.Results: We identified 53 patients who had both ultrasound and...

Objective: To identify the clinical features of Japanese patients with suspected hereditary sensory and autonomic neuropathy (HSAN) on the basis of genetic diagnoses.Methods: On the basis of clinical, in vivo electrophysiologic, and pathologic findings, 9 Japanese patients with sensory and autonomic nervous dysfunctions were selected. Eleven known HSAN disease-causing genes and 5 related genes were screened using a next-generation sequencer.Results: A homozygous mutation, c.3993delGinsTT, was identified in exon 22 of SCN9A from 2 patients/families. The clinical phenotype was characterized by...

Objective: To determine the frequency, timing, causes, and risk factors of death in Guillain-Barre syndrome (GBS).Methods: Prospectively collected data were reviewed from a cohort of 527 patients with GBS previously included in 1 observational and 3 therapeutic studies. Risk factors were identified by comparing deceased and surviving patients with GBS.Results: Fifteen (2.8%) of 527 patients with GBS died within 6 months of follow-up at highly variable time points during the disease course, with a median time from onset of weakness...

Objective: To assess whether there are differences in the strength of association with incident stroke for specific periods of life in the Stroke Belt (SB).Methods: The risk of stroke was studied in 24,544 black and white stroke-free participants, aged 45+, in the Reasons for Geographic and Racial Differences in Stroke study, a national population-based cohort enrolled 2003-2007. Incident stroke was defined as first occurrence of stroke over an average 5.8 years of follow-up. Residential histories (city/state) were obtained by questionnaire....

Objective: The study aims to examine [alpha]-synuclein in the CSF of patients with severe traumatic brain injury (TBI) and its relationship with clinical characteristics and long-term outcomes.Methods: This prospective case-control study enrolled patients with severe TBI (Glasgow Coma Score <=8) who underwent ventriculostomy. CSF samples were taken from each TBI patient at admission and daily for up to 8 days after injury and successively assessed by ELISA. Control CSF was collected for analysis from subjects receiving lumbar puncture for other...

Summary: Peripheral neuropathy is a common disorder, often prompting an extensive initial laboratory evaluation. The initial evaluation is particularly challenging to primary care physicians and neurologists because of the broad differential diagnosis. Although screening thyroid and rheumatologic tests are frequently ordered, the diagnostic yield of these tests is unclear. Data from our institution were collected on patient demographics, clinical characteristics including warning signs suggestive of a diagnosis other than distal symmetric polyneuropathy, history of thyroid or rheumatologic disease, and laboratory...

Summary: Unruptured intracranial aneurysms (UIA) are a common finding, occurring in about 2% of the population, making them very likely to be seen by most practitioners, and present a challenge in the recommendations for optimal management and screening. The consequences of aneurysm rupture are dire, with high likelihood of significant morbidity and mortality. Most aneurysms do not rupture and patients harboring these lesions often remain asymptomatic. There are effective surgical and endovascular interventions to prevent rupture, but these procedures carry...

Summary: Varicella-zoster virus (VZV) reactivation from latently infected ganglia causes multiple neurologic diseases. The most common is herpes zoster, which is frequently complicated by postherpetic neuralgia, meningoencephalitis, and vasculopathy, including VZV temporal arteritis, myelopathy, and retinal necrosis. All of these disorders can develop without rash. Importantly, VZV vasculopathy is emerging as a significant cause of TIAs and stroke. In particular, a subset of patients who present with symptoms and signs of giant cell arteritis (GCA), but whose temporal artery biopsies...

Summary: Neurocysticercosis is the most common parasitic brain disease worldwide. Its clinical heterogeneity is related to localization, number and stage of evolution of the parasites, sex, age, and intensity of the host brain inflammatory reaction. In addition to the localization of the parasite, inflammation is the main phenomenon responsible of symptomatology. Acute symptomatic seizures are the most common symptom in patients with parenchymal parasites, but most do not evolve into epilepsy. Neurocysticercosis diagnosis is based mainly on neuroimaging. New imaging...

Summary: Myasthenia gravis (MG) is the most common autoimmune disease affecting neuromuscular junction transmission. MG is characterized by muscle weakness that worsens with activity and fluctuates over the course of the day. Involvement of respiratory musculature can lead to life-threatening crisis requiring intensive care unit care. Antibody testing is positive in most patients with MG. Treatment of MG includes short-term symptomatic treatment, chronic immunosuppression, surgical intervention, and immunomodulatory therapies for severe disease or crisis. We review advances in 5 areas...

Summary: Recent health policy initiatives designed to improve care coordination have stimulated the resurgence of the patient-centered medical home (PCMH) model. The details of how primary and specialty care are coordinated within the PCMH model are of interest to specialists. A good medical home "neighbor" must adhere to principles that complement the PCMH team-based approach and personal relationship to the patient. One issue for neurologists considering participation in this model is whether they will function as the principal physician for...

Summary: The purchase, implementation, and maintenance of an electronic health record (EHR) are among the most significant financial investments a practice will make. A practice's choice of EHR will have long-term and wide-ranging implications for how that practice operates. A successful EHR implementation may potentially result in increased efficiency, improved quality of patient care, and a possibly more successful practice. Extensive research and thoughtful planning, done with the involvement of all stakeholders, the commitment of adequate time, staff, and financial...

Summary: Social and traditional media are revolutionizing health care. Medicine, once an art practiced behind closed doors, is now part of the public domain. This article will help neurologists navigate a complex maze of technology to optimize patient care without compromising privacy. We offer ideas for improving our digital "footprint." Guidance is given on maintaining professional demeanor in all private and public interactions to help us avoid personal or patient insult and injury. Acknowledging that neurology is becoming a specialty...

Summary: Otherwise benign MRI findings (so-called "incidentalomas") are a frequent cause of worry on the part of both clinician and patient. Knowledge of the imaging characteristics of incidentalomas, their (typically slight) pathologic significance, and how to distinguish these from less benign pathologic mimics can help avoid unnecessary worry, expense, and workup.(C)2013 American Academy of Neurology...

Summary: Generic substitution of antiepileptic drugs remains a controversial area without a clear consensus to guide clinicians. The US Food and Drug Administration (FDA) requires rigorous testing of generic products and states that all approved products are interchangeable. FDA studies involve single doses in normal subjects so may not represent the performance of generic products in people with epilepsy. Physician surveys, case reports, and retrospective pharmacy database analyses suggest that antiepileptic drug generic substitution is associated with more health problems...