Biomedical subjects
Publications and source records attributed to J J Kelly.
We report a woman with Guillain-Barré syndrome who developed a flexion posture of the right arm and hand resembling upper motor neuron dysfunction. EMG demonstrated that involuntary peripherally generated continuous motor unit discharges caused the posture.
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Medicare records represent a potential resource for surveillance data on hospitalizations among the elderly. Several features make these data useful for describing hospitalization patterns: a) Medicare covers hospitalization expenses for approximately 95% of the U.S. population greater than or equal to 65 years, and these data are considered to represent accurately the hospitalization patterns of this group; b) The comprehensive nature of the information about the beneficiary population provides public health researchers with data to generate accurate rates for well-defined demographic and geographic subgroups; c) Because data for all covered hospitalizations have been included in the file since 1984, stable estimates for even relatively rare conditions can be generated for any years since then; d) A unique personal identifier on each record permits the differentiation of first from subsequent hospitalizations, thereby permitting public health researchers to generate both counts and rates of hospitalizations by person and by event; and e) Temporal trends in hospitalization can be generated for 1980 through 1988, because data are available in machine-readable form. This report presents surveillance data for elderly persons hospitalized in 1988 for several diseases of public health importance. In 1988, approximately 9.1 million hospitalizations covered by Medicare occurred among persons greater than or equal to 65 years. For major disease categories, the rates of persons discharged ranged from 7,448/100,000 for circulatory diseases to 274/100,000 for blood diseases. For the 49 discrete diseases and conditions studied, rates varied widely by gender, race, and age. Conditions also varied widely in the mean number of discharges per person and in the probability that the condition would be recorded as the principal diagnosis.
A battery of electrophysiologic tests was developed to assess the relative degree of lower and upper motor neuron (spasticity) deficit in a group of ALS patients enrolled in a therapeutic trial. Test results were correlated with strength in the tibialis anterior muscle as determined by measurement of maximum voluntary isometric contraction (MVIC), using strain gauge tensiometers, and were also correlated with a clinical spasticity rating scale. Patients were tested every 6 to 8 weeks over more than 1 year. Compound muscle action potential amplitude (CMAPa) from tibialis anterior correlated best with MVIC and also showed a strong linear correlation with time, as did MVIC. Other tests correlated poorly with MVIC on the average, although individual patients did show high correlations. In those patients where correlation between CMAPa and MVIC was low, MVIC did not show a high linear correlation with time and was also highly variable. This study suggests that the addition of CMAPa should be considered in ALS therapeutic trials if MVIC is not available. In addition, CMAPa can be useful in study samples where MVIC deterioration is not linear over time or is highly variable.
Electrodiagnostic studies were analyzed in patients with neuropathy associated with IgM monoclonal proteins, 7 with anti-myelin associated glycoprotein reactivity (MAG) and 7 nonreactive to MAG. The findings were distinctly different in the two groups. The electrodiagnostic studies of all the MAG-reactive patients had demyelinating features with slowing of conduction and prolonged distal latencies while only one of the MAG-nonreactive patients had a demyelinating pattern. In fact, the MAG-nonreactive patients were a heterogeneous group, both electrodiagnostically and clinically. This study supports the concept that MAG-reactive polyneuropathy is demyelinating in type, and is caused by the activity of the IgM M-protein directed at an antigen in the myelin sheath.
A policy on prenatal radiation exposure at two nuclear power plants was revised to give better assurance of compliance with NCRP recommendations on fetal radiation exposure. This action was taken after publication of NCRP 91 in June 1987 to provide better assurance that a total dose equivalent limit to an embryo-fetus be no greater than 0.5 mSv (0.05 rem) in any month and no more than 5 mSv (500 mrem) for a gestation period. For any female worker to receive radiation exposure greater than 1.5 mSv (0.15 rem) in a month at these nuclear power plants, she was asked to initiate an administrative request for radiation exposure in excess of this limit. In this request, she was asked to acknowledge that she was aware of the guidance in U.S. NRC Regulatory Guide 8.13. A worker who had the potential for radiation exposure in excess of 1.5 mSv (0.15 rem) refused to process this request and was consequently denied overtime work. She filed a grievance for denial of overtime, and this grievance was submitted for labor arbitration in June 1988. The arbitration decision and its basis and related NRC actions are discussed.
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Monoclonal gammopathy of undetermined significance (MGUS) is the most common paraproteinemia associated with polyneuropathy. Although the clinical and electrodiagnostic manifestations most resemble those of chronic inflammatory demyelinating polyneuropathy, some patients manifest a pure sensory neuropathy or neuronopathy. The M protein is usually IgM, and its concentration in serum is low. Nerve pathology from patients with demyelinating disease shows a reduction of large myelinated fibers and segmental demyelination with remyelination. In some cases, the M protein possesses antibody activity against components of the myelin sheath or axon. These neuropathies may respond to treatment with steroids, immunosuppressant agents, and plasma exchange.
We describe a patient with a chronic, symmetric, monophasic, acquired, pure motor, demyelinating polyneuropathy. Electrodiagnostic studies showed the presence of multifocal conduction blocks in motor nerves at sites not prone to compression. A sural nerve biopsy was normal. The patient responded to immunosuppressive therapy and plasma exchange. We postulate that this disorder is an unusual variant of chronic inflammatory polyradiculoneuropathy.
The acquired demyelinating polyneuropathies include acute (AIDP, Guillain-Barré syndrome, GBS) and chronic (CIDP, dysproteinemic) forms which differ primarily in their temporal profile. They are inflammatory-demyelinating diseases of the peripheral nervous system and likely have an immunologic pathogenesis. Although these neuropathies usually have a characteristic presentation, the electromyographer plays a central role in their recognition, since the demyelinating component of the neuropathy, which greatly reduces the differential diagnosis, is often first identified in the electromyography laboratory. In AIDP, the electromyographer, in addition to establishing the diagnosis, can sometimes predict the prognosis. Recognition of the chronic and dysproteinemic forms of acquired demyelinating polyneuropathy is important since they are treatable. The dysproteinemic forms also may be associated with occult systemic disorders that also may require treatment, independent of the neuropathy.
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Transcription of the simian virus 40 (SV40) late promoter is strongly activated by SV40 T antigen. We were interested in examining this process in relation to other T-antigen functions such as replication and repression of early transcription. To quantitate the various T-antigen effects, we used a vector which has a promoterless beta-globin gene flanking the early and late sides of the SV40 promoter region. Following cotransfection with a plasmid encoding T antigen, transcription from the two promoters can be measured with a single S1 mapping probe and replication can be assayed by Southern blot analysis of DNA recovered in Hirt extracts. In this study, transactivation was examined in HeLa and 293 cells, since these cells differ in their ability to support SV40 replication. The strength of the late promoter relative to the early promoter was approximately three- to fourfold higher in 293 cells. Replication in 293 cells was also more efficient, by the same margin. In both cell lines, late promoter transactivation was barely detectable on replication-defective templates. Taken together, the results suggest that T-antigen activation of late transcription occurs only on replicated, or replicating, DNA. T antigen also activated the late-early start sites, and while in HeLa cells they were seen to be only 30% as strong as the late promoter, in 293 cells late and late-early activities were almost equal.
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We studied ten patients with IgM monoclonal gammopathies. Five had M proteins that reacted with myelin-associated glycoprotein (MAG) and five had no recognizable antinerve activity. The neuropathy in the MAG-reactive patients was homogeneous by clinical and laboratory analysis, while the neuropathy in the MAG-nonreactive patients varied considerably. Both groups responded well to immunosuppressive therapy, which lowered the concentration of the serum M protein. The homogeneity of the MAG-reactive patients and their response to sustained lowering of the M protein levels support the concept that the IgM M protein directly damages nerve fibers and is the proximate cause of the polyneuropathy.