Faculty Bibliography

Neurologists are prescribing intravenous immunoglobulin (IVIg) with increasing frequency to treat many neurologic conditions that have a proven or presumed autoimmune or inflammatory pathogenesis. Although IVIg is not FDA approved for any neurologic condition, physicians can safely prescribe it for several disorders with reasonable certainty that the cost for the agent will be reimbursed by third-party carriers. This article discusses present FDA indications for using IVIg, off-label uses of IVIg, policies of third-party payers toward reimbursement, the Local Medical Review Policy of Medicare, coding, billing, and reimbursement for IVIg infusion, and approaches to use when reimbursement is delayed or denied.

Femoral, saphenous, and obturator neuropathies have diverse causes, many of which are iatrogenic. They have overlapping, but distinct, clinical features. Electrodiagnostic testing can distinguish between these disorders and others in the differential diagnosis. Imaging studies may demonstrate the origin of the neuropathy in some cases. Conservative treatment is usually sufficient, but occasionally surgical exploration of the affected nerve is indicated.

This review discusses the state of neurology and the Internet at the turn of the millennium. First, some basic definitions about the Internet and its component protocols are presented. Next, ways neurologists and patients can use the Internet are enumerated. Internet resources or applications are available or are being created that can aid in the successful fulfillment of a neurologist's core professional activities: clinical care, teaching, research, and practice issues. Currently, the most useful categories of Internet resources for neurologists are electronic communication and access to knowledge bases. They fulfill needs that are not met by traditional, non-electronic media. There are many other types of Internet applications that supplement traditional medical methodologies. Finally, some problems and prospects concerning medical uses of the Internet are discussed: technological infrastructure including usability, security, meaning, validity/quality, value, outcomes, and responsibility. These issues must be successfully addressed if Internet computing is to become truly useful 'just in time' at the point of medical care. Solutions are actively under development today. The prospects are bright for neurology, and medicine in general, on the Internet. The Internet will become an essential medical device in the near future.

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.

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/METHODS: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.

Intracellular ionized Ca2+ concentration was measured in clonal mouse anterior pituitary tumor cells with the fluorescent Ca2+ indicator Quin-2. In control physiological solution, free cytoplasmic Ca2+ concentration was found to be 139 +/- 11 nM. Replacement of 50 mM NaCl by 50 mM KCl in the extracellular fluid caused a 29 mV depolarization and a 4.2-fold increase in the concentration of free cytoplasmic Ca2+. Under comparable depolarizing conditions, a specific influx of 2.66 nmole of 45Ca2+ per mg protein was detected 1 min after addition of high K+, accompanied by a marked increase in the initial rate of beta-endorphin secretion. In the absence of external Ca2+, depolarization by K+ produced little or no increase in either intracellular free Ca2+ or hormone release. Incubation of AtT-20/D16-16 cells in the secretagogue norepinephrine led to a depolarization accompanied by an increase in spontaneous action potential frequency and a marked elevation in cytosolic Ca2+ concentration. Exposure of cells to somatostatin, an inhibitor of hormone release, led to only transient decreases in burst frequency and no significant reduction in intracellular Ca2+ levels. These results indicate that in addition to intracellular Ca2+, other factors also control secretory activity in AtT-20/D16-16 anterior pituitary cells.