Biomedical subjects
Publications and source records attributed to J J Kelly.
There were two patients with severe dermatomyositis refractory to immunosuppressive therapy treated with 150 rad of total body irradiation given over a period of 5 weeks. Both patients responded promptly with minimal side-effects and remain in partial remission 42 and 18 months after completion of the treatment. Total body irradiation is effective in some patients with dermatomyositis who are refractory to standard therapy.
A 67-year-old woman developed a fixed, flexed posture of the right arm and hand due to a cervical astrocytoma. EMG showed continuous motor unit potential firing at 8 to 15 Hz with an abnormal silent period, presumably due to the loss of local spinal cord inhibitory interneuronal input.
The raphe-spinal pathway, which contains co-localized serotonin (5-HT), thyrotropin-releasing hormone (TRH), and several TRH-prohormone-derived non-TRH peptides, projects to the ventral horn of the spinal cord. Pharmacologic ablation of this pathway with the 5-HT neurotoxin, 5,7-dihydroxytryptamine, in neonatal rats resulted in deficient recovery of plantar foot muscles, functionally denervated with botulinum toxin type A. Failure of reinnervation was suggested by slower and incomplete recovery of the plantar foot compound muscle action potential amplitude and by a reduced mean diameter of plantar foot muscle fibers in ablated rats. These findings indicate that deprivation of alpha motor neurons from descending raphe-spinal input interferes with their ability to respond to muscle-derived signals for reinnervation.
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To identify predictors of bacterial infection in patients developing postoperative fever, we prospectively studied 434 adults who underwent abdominal surgery. Of the 434 study patients, 163 (38%) developed postoperative fever (38.1 degrees C [100.6 degrees F] or greater) and 26 (16%) of the febrile patients were found to have bacterial infection. Logistic-regression analysis showed that postoperative infection was associated with a WBC count of less than 5000 or greater than 10,000/mm3, a BUN of 15 mg/dl or greater, and fever onset after the second postoperative day. A predictive index, constructed from these three features, created a useful gradient for estimating the likelihood of postoperative infection. In patients with zero, one, two or three of the index features, the proportions having infection were 2 per cent (1/50), 14 per cent (12/88), 45 per cent (10/22), and 100 per cent (3/3), respectively (P less than 0.0001). This simple index, which uses readily available clinical data, may help reduce the cost of postoperative care by identifying patients with a low probability of infection in whom cultures, imaging studies, and empirical antibiotics do not appear necessary. Thorough diagnostic evaluation in patients with two or more index features may also reduce delay in the detection and treatment of postoperative infection. The predictive value of this index should be validated in a new patient set, however, before widespread application of the index is warranted.
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Thyrotropin-releasing hormone (TRH), present in high concentrations in the mammalian spinal cord, exerts excitatory effects on the alpha-motorneuron (AMN) via axodendritic contacts. We used the neurotoxin 5,7-dihydroxytryptamine (5,7-DHT) to deplete TRH from the ventral horn of the spinal cord of adult rats to determine whether the tripeptide may be trophic to the AMN. The rats were studied blindly and sequentially for 11 weeks. Motor performance remained normal by clinical and electrophysiologic assessments. AMN counts were not reduced in the lumbar cord, and gastrocnemius muscle showed no evidence of denervation in treated rats. We conclude that in the adult rat chronic ventral horn TRH deficiency does not lead to AMN degeneration and is not associated with a significant alteration of AMN function.
This report describes a recent case in which colonoscopy of a patient with the presenting complaint of rectal bleeding revealed the right colon to be carpeted with 50-100 pseudopolyps, each 3-4 mm in diameter. Biopsy specimens taken during colonoscopy revealed the presence of Strongyloides stercoralis in the bowel wall. This diagnosis should be considered in any patient with gastrointestinal complaints and a history of travel to an endemic area.
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Immunoblot analysis was performed on the serum from 29 patients with polyneuropathy and monoclonal gammopathy. Nine patients had IgM spikes, and six of the nine had reactivity against myelin-associated glycoprotein (MAG) associated with a slowly progressive, predominantly sensory neuropathy. In contrast, 23 patients who lacked anti-MAG reactivity had more severe sensory motor neuropathy. Thus, IgM monoclonal gammopathy with reactivity against MAG may define a distinct clinical entity.
Four patients with inclusion body myositis were treated with 150 rad of total body irradiation given in 5 weeks. One patient responded subjectively and transiently, but no patient showed clear benefit. This treatment is not recommended for inclusion body myositis.
Over the last decade, the increasing use of serum and urine protein electrophoretic screening of patients with idiopathic peripheral neuropathy has led to greater recognition of peripheral neuropathy syndromes that are associated with monoclonal proteins and plasma cell dycrasias. After careful evaluation, most of these patients have benign monoclonal gammopathy, followed in frequency by primary systemic amyloidosis and osteosclerotic myeloma, with occasional cases associated with osteolytic multiple myeloma, Waldenstrom's macroglobulinemia, gamma heavy chain disease, and other rare disorders. Several of these syndromes have distinctive presentations and are recognizable clinically, whereas others (especially multiple myeloma neuropathy) are diverse clinically and are not clearly distinguishable from other chronic neuropathies. The discovery of IgM-kappa monoclonal proteins directed at myelin antigens in some patients with benign monoclonal gammopathy and the delineation of the syndrome of neuropathy and multiorgan involvement in osteosclerotic myeloma are important developments which may shed light on the mechanism of the remote effects of malignancies on the nervous system.
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A 51-year-old woman with an inherited polyneuropathy had fatiguability and a dramatic historical response to prostigmine. Repetitive motor nerve stimulation produced a prominent decrement of the compound muscle action potential in distal muscles with marked facilitation after brief exercise. Defective neuromuscular transmission paralleled the polyneuropathy in distribution and severity. We hypothesize that deficient release of acetylcholine by regenerating or degenerating nerve terminals likely caused the defect of neuromuscular transmission in this patient.