Local effects of recombinant rat interleukin-6 on the peripheral nervous system.
(25/1736)
Interleukin-6 (IL-6) is a multifunctional cytokine with a broad range of activities and can affect a variety of target cells or systems in multiple ways. However, there is currently no consensus on how IL-6 directly affects the peripheral nervous tissue. We performed histopathological and immunohistochemical analyses to investigate the direct effects of recombinant rat IL-6 (rrIL-6) following its intraneural injection into the sciatic nerve of adult Lewis rats. One day after injection, a large number of macrophages, major histocompatibility complex (MHC) class II positive cells, and CD4+ and CD8+ T cells appeared within the perineurium and endoneurium. From day 4 to day 7 after injection, we observed a gradual increase of inflammation and demyelination. On day 7, demyelination affected more than 80% of nerve fibres. In contrast, in the sterile phosphate-buffered saline (PBS)-injected control group, lower inflammation and fewer demyelinating nerve fibres were observed on days 4 and 7. Thus, intraneural injection of rrIL-6 into the sciatic nerve induces high inflammation and severe demyelination. This study improves our understanding of the effector mechanisms underlying inflammation and demyelination and identifies IL-6 as an essential mediator of inflammation and demyelination in the peripheral nervous system after local administration. (+info)
Systemic lupus erythematosus demonstrating serum anti-GM1 antibody, with sudden onset of drop foot as the initial presentation.
(26/1736)
In systemic lupus erythematosus (SLE), peripheral neuropathies are relatively uncommon and rarely present as the initial symptom. We herein describe a 61-year-old woman who developed a sudden onset of drop foot, which was indistinguishable from Guillain-Barre syndrome based on the clinical symptoms alone. Antibodies against ganglioside GM1 were detected in the serum, while no antibodies to Campylobacter jejuni were observed. An electrophysiological study showed axonal impairment rather than demyelination. A pathological examination of a sural nerve biopsy specimen and further laboratory examinations suggested the observed peripheral neuropathies to have arisen due to lupus vasculitis. The serological activities of SLE responded well to treatment with corticosteroids, mizoribine and immunoadsorption therapies, however, the drop foot symptoms did not change remarkably. (+info)
Diphtheritic polyneuropathy: a clinical study and comparison with Guillain-Barre syndrome.
(27/1736)
OBJECTIVES AND METHODS: Clinical features of 50 adults with diphtheritic polyneuropathy (DP) were studied in Riga, Latvia and compared with 21 patients with Guillain-Barre syndrome (GBS). RESULTS: Neurological complications occurred in 15% of patients admitted to hospital with diphtheria and usually after severe pharyngeal infection. Bulbar dysfunction occurred in 98% of patients with DP and only 10% of patients with GBS. Limb weakness was mild or absent in 30% of patients with DP. Ventilation dependent respiratory failure occurred in 20% of patients with DP. The first symptoms of DP occurred 2-50 days after the onset of local diphtheria infection. Neurological deterioration in DP continued for a median of 49 (range 15-83) days and improvement started 73 (range 20-115) days after onset. In 66% of patients with DP, the neuropathy was biphasic with a secondary worsening after 40 days. By contrast patients with GBS worsened for only 10 days on average (range 2-28 days) and improved after 21 (range 4-49) days. Eight patients with DP died, four from severe cardiomyopathy and four from multiple diphtheritic organ failure. Prolonged distal motor latencies (DMLs) were common to both DP and GBS, and more pronounced than motor conduction slowing. Limb symptoms continued after 1 year in 80% of the patients with DP, 6% were unable to walk independently, but independent respiratory and bulbar function had returned in all survivors. By comparison no patients with GBS died and none were severely disabled after 1 year. No death, in patients with DP occurred after antitoxin on days 1 or 2 after onset of diphtheria symptoms, whereas identical rates of death and peak severity of DP were seen both in those who received antitoxin on days 3-6 and those who did not receive it at all. CONCLUSION: Diphtheric polyneuropathy is much more likely than GBS to have a bulbar onset, to lead to respiratory failure, to evolve more slowly, to take a biphasic course, and to cause death or long term disability. Antitoxin seems ineffective if administered after the second day of diphtheritic symptoms. (+info)
Solitary plasmacytoma presenting as peripheral neuropathy: a case report.
(28/1736)
We report a 26 year old woman presenting with a chronic predominantly motor radiculoneuropathy. A biopsy proven solitary lytic plasmacytoma was detected in the left sixth rib. Serum immunoelectrophoresis revealed 'M' band (IgG, l light chain). Excision of the plasmacytoma resulted in gradual but steady recovery. We suggest that all patients with 'cryptogenic' polyneuropathy be investigated for this potentially treatable condition. A high index of suspicion assists the organization of appropriate diagnostic investigations. (+info)
Clinical range and MRI in Creutzfeldt-Jakob disease with heterozygosity at codon 129 and prion protein type 2.
(29/1736)
A 68 year old woman with sporadic Creutzfeldt-Jakob disease is described, who neither showed characteristic EEG abnormalities nor a positive test of the neuronal protein 14-3-3 or neuron specific enolase (NSE) in CSF, despite a clinical presentation with ataxia of cerebellar type, rapidly progressive dementia, myoclonus, and marked hyperintense signal abnormalities in the deep cortical layers and the basal ganglia on T2 and diffusion weighted MRI. Moreover she showed atypical clinical features with a syndrome of inappropriate antidiuretic hormone (ADH) secretion (SIADH) and a peripheral sensorimotor polyneuropathy. Whether these disturbances are independent of Creutzfeldt-Jakob disease or a feature of it is discussed. It has recently been shown that in Creutzfeldt-Jakob disease different clinical and pathological phenotypes correlate with the polymorphism at codon 129 of the prion protein gene (PRNP) and the type of the protease resistant fragment that accumulates in the brain. According to the new classification at least six sporadic variants of Creutzfeldt-Jakob disease exist. The molecular genetic analysis showed heterozygosity of PRNP at codon 129 for methionine and valine and the presence of PrP(CJD) type 2 in the brain of this patient. As a new feature of changes on MRI, striking cortical changes of hyperintense signals are described in diffusion weighted as well as T2 weighted MRI that directly correlate with the histomorphological spongy degeneration of the brain in this region. In cases of rapidly progressive dementia, Creutzfeldt-Jakob disease always needs to be considered even if unusual features are present and current diagnostic criteria are not in favour of this disease. (+info)
Potassium current suppression in patients with peripheral nerve hyperexcitability.
(30/1736)
Acquired neuromyotonia (Isaac's syndrome) is considered to be an autoimmune disease, and the pathomechanism of nerve hyperexcitability in this syndrome is correlated with anti-voltage-gated K(+) channel (VGKC) antibodies. The patch-clamp technique was used to investigate the effects of immunoglobulins from acquired neuromyotonia patients on VGKCs and voltage-gated Na(+) channels in a human neuroblastoma cell line (NB-1). K(+) currents were suppressed in cells that had been co-cultured with acquired neuromyotonia patients' immunoglobulin for 3 days but not for 1 day. The activation and inactivation kinetics of the outward K(+) currents were not altered by these immunoglobulins, nor did the immunoglobulins significantly affect the Na(+) currents. Myokymia or myokymic discharges, with peripheral nerve hyperexcitability, also occur in various neurological disorders such as Guillain-Barre syndrome and idiopathic generalized myokymia without pseudomyotonia. Immuno-globulins from patients with these diseases suppressed K(+) but not Na(+) currents. In addition, in hKv 1.1- and 1.6-transfected CHO (Chinese hamster ovary)-K1 cells, the expressed VGKCs were suppressed by sera from acquired neuromyotonia patients without a change in gating kinetics. Our findings indicate that nerve hyperexcitability is mainly associated with the suppression of voltage-gated K(+) currents with no change in gating kinetics, and that this suppression occurs not only in acquired neuromyotonia but also in Guillain-Barre syndrome and idiopathic generalized myokymia without pseudomyotonia. (+info)
A four-year follow-up study on subjective symptoms and functional capacities in workers using hand-held grinders.
(31/1736)
Fifty-three grinders in the metal industry were re-examined 4 years after their first examination. Information about age, occupation, daily vibration exposure, drinking and smoking habits, and presence of subjective symptoms such as vibration-induced white finger (VWF), and numbness and pain in the fingers was collected during the first and second examination. Cold provocation test (10 degrees C/10 min) was also employed to evaluate disturbances in the peripheral circulatory and peripheral nerves in all subjects. The frequency-weighted vibration acceleration of various types of hand-held tools was measured. There was no subject with VWF at the first examination; however, during the course of follow-up, two cases (3.8%) of VWF with latent interval of more than 25 years were diagnosed. Prevalence of numbness in the fingers and shoulder stiffness was significantly higher at the second examination. When the prevalence of subjective symptoms was tested by the subjects' total operating time (TOT) during the 4-year follow-up period, those whose TOT was equal to or more than 2500 hours showed higher prevalence compared to the other subgroup. The paired values of recovery rate of finger skin temperature and vibration sensation threshold after the cold water immersion test were significantly different at the first and second examination. On average, the diminution of hand-grip force during the 4-year follow-up course was 7.4%; the difference being significant at 0.01 level. Significant differences in the paired data of pinching power and tapping ability could be detected. The frequency-weighted vibration acceleration of various tools was in the range of 1.1-4.6 m/s2. It was concluded that: (1) prolonged occupational exposure to the vibration of hand-held grinding tools should be considered as a risk factor causing disturbances in the hand-arm system of the operators; (2) the results of recovery rate of finger skin temperature and the vibration sensation threshold seemed to be appropriate indicators for the assessment of peripheral vascular and peripheral nerve disturbances in workers exposed to hand-arm vibration; and (3) to reduce the subjects' physical stress, attention should be paid to ergonomic factors. (+info)
A study on the effects of countermeasures for vibrating tool workers using an impact wrench.
(32/1736)
The aims of this study were (1) to measure frequency-weighted vibration acceleration and (2) to study the effects of introducing a vibration-proof impact wrench on VWF in workers. The subject pool was 383 male workers who were regularly using an impact wrench and taking special medical examinations for vibration syndrome in a factory from 1982 to 1999. The prevalence of workers with VWF increased gradually after 1982, reached a peak value (4.8%) in 1986, gradually decreased after 1987, and disappeared in 1994. Sixteen subjects who had had VWF at least one time during the observation period were selected for this study. The stages of VWF were at stage I on the Stockholm Workshop scale in all subjects. After the vibration-proof impact wrench was introduced in 1986, the vibration acceleration of the impact wrench measured on the handle decreased from 8.6-11.1 m/s2 to 5.1-7.1 m/s2. The actual time per day that subjects were assumed to use the impact wrench was 108 minutes. The subjects actually used an impact wrench more than the occupational exposure limit allowed. However, VWF disappeared after the introduction of a vibration-proof impact wrench. This might have resulted from the combined effect of introducing the vibration-proof impact wrench and certain countermeasures that were taken against cold working environments. (+info)