Neil A. Busis

Myofibrillar myopathy with abnormal foci of desmin positivity. I. Light and electron microscopy analysis of 10 cases

A number of myopathies whose common denominator is abnormal foci of desmin positivity have been described under the rubrics of spheroid body myopathy, cytoplasmic body myopathy, Mallory body myopathy, myopathy with granulofilamentous inclusions, desmin storage myopathy, and intermediate filament myopathy. In this study we reevaluate the light microscopic and ultrastructural features of the myopathy with abnormal foci of desmin positivity. In 10 cases of the disease, ultrastructural analysis reveals 2 major types of lesions: (a) foci of myofibrillar destruction and (b) hyaline structures that appear as spheroidal bodies on electron microscopy. The foci of myofibrillar destruction consist of fiber areas containing disrupted myofilaments, Z-disk-derived bodies, dappled dense structures of Z-disk origin, and streaming Z-disks that are sometimes adjacent to lakes of dense material. The spheroid bodies are composed of compacted and degraded myofibrillar elements. Membrane-bound vacuoles harboring degenerating membranous organelles are a less frequent and probably secondary abnormality. None of the lesions in muscle comprise 8 to 10 nm intermediate filaments. The findings imply that spheroid body myopathy, cytoplasmic body myopathy, Mallory body myopathy, and myopathy with granulofilamentous inclusions are consequences of a single or closely related pathologic processes. Because the common denominator appears to be focal dissolution of the myofibrils followed by accumulation of the products of the degradative process, we propose the term myofibrillar myopathy to cover the observed spectrum of pathologic changes.

Neurological Manifestations of Acute Posterior Multifocal Placoid Pigment Epitheliopathy

BACKGROUND Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a chorioretinal disease that causes acute visual symptoms with characteristic fundus findings. Although this entity has been associated with a variety of neurological complications, it has received little attention in the neurological literature. We wanted to emphasize the spectrum of neurological involvement, in particular the occurrence and management of strokes in patients with APMPPE. CASE DESCRIPTIONS We report three patients with APMPPE and neurological disease. All three presented with marked visual disturbances and headaches. One patient developed recurrent strokes involving different vascular territories of the brain and required immunosuppressive treatment for presumed cerebral vasculitis. The other two patients had cerebrospinal fluid pleocytosis and persistent headaches but recovered spontaneously. The review of the literature demonstrates a particular pattern of neurological complications in a subgroup of patients with APMPPE. CONCLUSIONS APMPPE should be considered among the causes of stroke and aseptic meningitis in young adults. The diagnosis is critically dependent on a thorough ophthalmologic examination. Severe neurological complications are difficult to predict at the onset of the ophthalmologic disease. The patients should be monitored closely. If investigations suggest cerebral vasculitis, immunosuppressive treatment may be helpful to prevent recurrences.

Burnout, career satisfaction, and well-being among US neurologists in 2016

Objective: To study prevalence of and factors that contribute to burnout, career satisfaction, and well-being in US neurologists. Methods: A total of 4,127 US American Academy of Neurology member neurologists who had finished training were surveyed using validated measures of burnout, career satisfaction, and well-being from January 19 to March 21, 2016. Results: Response rate was 40.5% (1,671 of 4,127). Average age of participants was 51 years, with 65.3% male and nearly equal representation across US geographic regions. Approximately 60% of respondents had at least one symptom of burnout. Hours worked/week, nights on call/week, number of outpatients seen/week, and amount of clerical work were associated with greater burnout risk. Effective support staff, job autonomy, meaningful work, age, and subspecializing in epilepsy were associated with lower risk. Academic practice (AP) neurologists had a lower burnout rate and higher rates of career satisfaction and quality of life than clinical practice (CP) neurologists. Some factors contributing to burnout were shared between AP and CP, but some risks were unique to practice setting. Factors independently associated with profession satisfaction included meaningfulness of work, job autonomy, effectiveness of support staff, age, practicing sleep medicine (inverse relationship), and percent time in clinical practice (inverse relationship). Burnout was strongly associated with decreased career satisfaction. Conclusions: Burnout is common in all neurology practice settings and subspecialties. The largest driver of career satisfaction is the meaning neurologists find in their work. The results from this survey will inform approaches needed to reduce burnout and promote career satisfaction and well-being in US neurologists.

To revitalize neurology we need to address physician burnout

Illness causes suffering in unexpected ways. Patients suffer not only from their disease but also from diagnostic tests and treatment.1 Physicians are susceptible to adverse health consequences of their chosen profession.

Chronic care management codes: A small step in the right direction

Coordinating the care of patients with chronic diseases is essential if we are to improve patient care, enhance population health, and reduce health care costs. Care coordination assists the patients team of providers in making the right diagnostic and therapeutic choices, avoids duplication of services, and facilitates incorporating patient preferences regarding goals of care and socioeconomic determinants of health into the care plan.1

The AAN and the triple aim

The American Academy of Neurology (AAN) has long been a leader among medical professional societies in being dedicated to providing tools aimed at improving the quality of neurologic care. Since the emergence of a Federal call for evidence-based guidelines in the early 1990s, AAN subcommittees have produced almost 200 guidelines using the most stringent systematic review methods. More recently, other AAN subcommittees translated some of our guidelines into quality indicators for several neurologic disorders (e.g., epilepsy, Parkinson disease, amyotrophic lateral sclerosis, distal symmetrical neuropathy). This set of tools is aimed at helping neurologists in ambulatory care settings improve quality, which should, if well-implemented, lead to improved health outcomes.