Parkinsonism due to n-hexane exposure.
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Biomedical subjects
Publications and source records attributed to S Barbieri.
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Twenty-seven patients with neuropathy and IgM monoclonal gammopathy were tested for antigen specificity of the M-protein and for anti-myelin-associated glycoprotein (MAG) IgM levels by immunoblot. In 16 patients (59.2%) the M-protein reacted with MAG and with cross-reactive glycoconjugates. Anti-MAG IgM titers in these patients ranged between 1:12,800 and 1:100,000. A fainter IgM reactivity with MAG and related glycoconjugates was detected in 3 additional patients with neuropathy, but also in 8 of 24 patients with IgM M-protein without neuropathy (33.3%). This reactivity was not due to the M-protein and corresponded to antibody titers of 1:400 or less in all but 1 patient with a titer of 1:3,200. Low titers of anti-MAG IgM (1:200 or less) were also detected in 17 of 101 control patients without IgM M-proteins (16.8%), while 1 patient with neuropathy of unknown cause had anti-MAG IgMK titers of 1:25,600. In 1 patient with neuropathy and IgM M-protein that was not anti-MAG, the M-protein bound to other antigens in nerve, while in 6, other possible causes or mechanisms for the neuropathy were found. In this study, high titers of anti-MAG IgM antibodies were always associated with neuropathy. The presence of low levels of anti-MAG IgM in a significant proportion of controls suggests that monoclonal expansion of naturally occurring B-cell clones secreting anti-MAG IgM may be responsible for the high incidence of this antigen specificity of the M-protein.
Five patients with neuropathy and IgM M-proteins that reacted with myelin-associated glycoprotein (MAG) were treated for 10 to 20 months with cytostatic agents. In 2 patients, a decrease in serum M-protein and in anti-MAG IgM levels coincided with a progressive improvement of neuropathy. No clinical improvement and no decrease of anti-MAG IgM were observed in the other patients. The close relationship between the decrease of anti-MAG M-proteins and clinical improvement in these patients supports the pathogenetic role of the M-protein in the neuropathy.
This work was set up to investigate the possible presence of peripheral nervous system involvement as a long-term effect of the exposure to dioxin in 152 subjects with chloracne from the Seveso area; 123 age- and sex-matched subjects living in nearby towns with similar environmental pollution formed the control group. The accident in Seveso took place in July, 1976, and this study was carried out from October, 1982, to May, 1983. Although a peripheral neuropathy was not found in any of the subjects, a significant increase of the number of individuals presenting at least two bilateral clinical signs (p less than 0.05) or one abnormal electrophysiological parameter (p less than 0.02) was found in the Seveso group. Principal component analysis did not show any subdivision between these two groups. The Fisher approach to discriminant analysis reveals a clear subdivision between the group of the most exposed subjects and randomly selected subgroups of control subjects. In conclusion, clinical and electrophysiological signs of peripheral nervous system involvement occur with a statistically increased frequency in the Seveso population 6 years after the accident, although a peripheral neuropathy was not evident in any of the chloracne patients using the World Health Organization diagnostic criteria.
Visual evoked potentials were studied in 11 patients with neuropathy and macroglobulinemia. The P100 latency was increased bilaterally in 5 of the 6 patients whose IgM M-proteins reacted with myelin-associated glycoprotein (MAG) and in 1 of the other patients. In patients whose M-protein bound to MAG, abnormal visual evoked potentials correlated with the presence of the M-protein in the cerebrospinal fluid. Subclinical involvement of the central nervous system is frequent in patients with neuropathy and anti-MAG M-proteins and may be due to the binding of M-proteins to central nervous system myelin.
Mitochondria and myosin were isolated from a muscle biopsy of a 9-year-old boy with an unusual congenital myopathy characterized by type I fiber uniformity, jagged Z-line, and transverse network hypertrophy of mitochondria. Biochemical examination of isolated mitochondria showed that only citrate synthase activity was significantly reduced. Electrophoresis of myosin heavy chains and immunoenzymatic analysis of myosin heavy and light chains with antibodies specific to either fast or slow myosins showed that only the slow-type isoform of myosin was detectable. Indirect immunofluorescence of muscle biopsy showed that all muscle fibers homogeneously expressed only the slow type of myosin.
Peripheral neuropathy was found in 12 (46%) of 26 patients with macroglobulinemia. The neuropathy was subclinical in two. Anti-myelin-associated glycoprotein (MAG) activity was found in six (50%) patients with neuropathy. Sural nerve biopsies showed demyelination and IgM deposits on the myelin sheath. In one patient who had no anti-MAG activity, the serum IgM bound to peripheral myelin by indirect immunofluorescence and to several protein bands in peripheral nerve and other tissues by immunoblot. In the other five patients with neuropathy, we found no binding of M proteins to nerve components, but in three patients there were endoneurial IgM deposits in nerve biopsy. Peripheral neuropathy may be related to the antigen-specificity of M proteins.
Histological, histochemical and ultrastructural studies of muscle biopsy in a case of congenital neuromuscular disease revealed unusual findings consisting of muscle fibers uniformity which were all type I and of small diameter, jagged Z-line and abnormally developed transverse network of mitochondria. E.M.G. examination demonstrated a myopathic pattern, but mitochondrial changes are quite different from those reported in mitochondrial myopathies and jagged Z-line seems poorly correlated with Z-line streaming present in denervation atrophy, target fibers, core-like lesions or other Z-line abnormalities of the nemaline myopathy. On the other hand type I histochemical uniformity seems more likely related to some dysfunction of the neuronal mechanisms that control both the fiber type differentiation and other trophic influences. It also suggests that myogenic E.M.G. pattern might actually be pseudo-myopathic and due to a reduction of the cross sectional area of the individual muscle fibers composing the motor unit.
Nineteen patients with chronic respiratory insufficiency, mean age 61.4 +/- 12.2, have been investigated with pulmonary function tests, clinical neurological examination and neurophysiological methods including motor and sensory conduction studies and needle electromyography. None of them had conditions known to affect the peripheral nervous system such as diabetes, alcoholism, or uraemia. The motor and sensory conduction studies showed only a reduced mean amplitude of the ulnar nerve SAP and of the compound muscle action potential of the APB and EDB muscles. The EMG was abnormal in 94.7% of the cases and showed an increased percentage of polyphasic potentials and a reduced recruitment pattern of motor units firing at high frequency. The data seem to support the hypothesis of an involvement of motor neurons in this condition although the evidence for a neuropathy is lacking.
The authors report a case of hemorrhage for upper mesenteric artery erosion secondary to a pancreatic pseudocyst. After a review of literature data they outline the possible causes regarding the hemorrhagic event in course of pseudocyst and the modalities it happens. They finally underline the importance of a precocious diagnosis concerning the hemorrhage kind and seat through selective angiography, for an opportune and rational surgical management.
Four patients with muscular pain, fasciculations, contractures or cramps are presented. Evidence of peripheral nerve involvement was revealed by electromyography and nerve conduction studies. Muscle biopsy showed mild signs of denervation and reinnervation and, at electron microscopy, dilatations of terminal cisternae were found. All patients showed a remarkable improvement after therapy with diphenylhydantoin or carbamazepine. These clinical, neurophysiological and morphological data underline the role of peripheral nerve pathology in various syndromes of abnormal continuous muscular activity.
The aim of this work is the study of the influence of the MUAP morphology on the compound EMG signal by means of a simulation model. The simulated EMG is obtained as the sum of a number of MUS chosen on the basis of considerations concerning the muscle fiber dimension, the concentric needle electrode uptake area and the conduction volume. Myopathic and neurogenic conditions have been simulated together with the normal case, adopting typical MUAPs. The interspike interval (ISI) has been simulated by means of a stochastic variable. Both time and frequence domain analysis have been performed in the simulated EMG. The parameters obtained have been compared with those obtained from the experimental EMG in corresponding groups of pathologies. The model has provided clarifying data concerning the influence of the morphology of the MUAP, confirmed by the experimental findings. Simulation of abnormalities in the firing pattern as well as in the recruitment, seems to be necessary in order to improve the prediction capabilities of the model.
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BACKGROUND: To validate the accuracy of SAPS II, APACHE III and TRISS for the prediction of mortality in Intensive Care Unit (ICU) at polytrauma patients admission. The outcome of multiple trauma patients is often linked to the degree of physiologic dysfunction and to the extension of anatomic lesions, the age of the patient and the lesion mechanism. METHODS: The study population consisted of 93 cases of multiple injured patients hospitalised at the ICU of the Padua hospital from October 1998 to October 1999; the term polytraumatized patient is referred to patients who have multiple lesions of which at least one potentially endangers, immediately or in a short term, their life. These cases were evaluated with the APACHE III, SAPS II, Revised Trauma Score and Injury Severity Score. The predictive power of each system was evaluated by using decision matrix analysis to compare observed and predicted outcome with a decision criterion of 0.50 and 0.40 for risk of hospital death. RESULTS: All trauma score systems under study showed high accuracy rates, above all if they are used with a 40% positive test. CONCLUSIONS: The prognostic scales used in this study showed a good correlation between expected and observed cases, particularly with TRISS and APACHE III systems. The APACHE III system seems to be the most reliable of the different methods analysed. These prognostic systems are seldom or occasionally used in the ICU, in Padua and in the whole of Italy, so Italian data are not suitable to be compared to international ones. Due to urgency, the importance of the evaluation scales is often underestimated, but even if they require time and attention, they surely can be useful in the evaluation of the treatment, and not only of a polytraumatized patient.
AIM: This study aims to evaluate the management of intensive care beds according to the demands received by the SUEM 118 of Padua. It has been carried out by examining the reports drawn up by SUEM physicians from October 1996 to December 2001. The study rated the number of patients for whom an admission to the Intensive Care Unit (ICU) was required, according to the specific clinical situation at the moment of the request. A secondary objective was to evaluate if the critically ill patients had been admitted and treated in the most appropriate medical facility. METHODS: The research is based on 7 087 reports concerning a population of adult and pediatric patients for whom an ICU bed was required in the period previously mentioned. For each report, it analyses the following data (keeping them anonymous): date of demand, main pathology and severity of clinical condition, sex and age, provenence and destination. RESULTS: Even though the number of annual demands for an ICU bed made to SUEM Central 118 has remained unchanged (approximately 1 350 per year), the number of beds made available in the operating rooms of the Hospital of Padua markedly increased. What has been experienced so far, and the data collected in this study has revealed, was that the requests for an intensive treatment for the overall population (hospitalized and non hospitalized) increased disproportionally in relation to the availability of ICU beds. In fact, the total number of hospitalizations in the different ICUs rose steadily year by year (from 3 495 in 1996 to 4 640 in 2001). CONCLUSION: The Hospital of Padua is a landmark center for patients who need specialized treatment. It is therefore important to increase the assistance and safety standards of its ICUs. In recent years there has been a great need for specialized ICUs either for more aggressive procedures (neurosurgical, cardiosurgical, respiratory, cardiologic, etc.) or for the increased use of adequate and invasive treatment for advanced diseases. The available resources of ICU beds should be more rationally distributed between the peripheral and the Regional Hospitals, since the activation of an ICU bed in the operating theatre is a valid, transient option.
The presence of small areas of ossification in the Achilles tendon is a relatively frequent finding. It is instead rare to find large, extensive ossifications for more than half of the tendon. The authors describe a case of a man aged 45 years who, in the wake of an unknown lesion of the Achilles tendon, developed clinically symptomatic ossification 9.5 cm in length and 2.3 cm in width. The patient was treated surgically by removal of the ossified portion and repair of the diastasis with a proximal Achilles flap. After 17 months, the patient is asymptomatic, there are no disorders in walking, and he exclusively shows moderate limitation in range of movement of the ankle joint associated with tricipital hyposthenia. Bone metaplasia of the Achilles tendon is a rare event, that can follow trauma or surgery. The size of the area to be removed implies considerable difficulty in repair of the continuity and in recovery of the length of the tendon.
The clinical and neurophysiological features of 23 patients affected by essential cryoglobulinemia (EC) have been studied. It was possible to perform sural nerve biopsy in 3 cases. Six patients were found to be affected by a peripheral neuropathy, according to the WHO criteria, while in 8 other patients clinical and neurophysiological signs of a milder peripheral nervous system (PNS) involvement were evident. The incidence of PNS involvement seems to be high (60.9%). Neurophysiological and histological studies were indicative of a mainly axonal damage.