Risk of fracture in patients with Guillain-Barré syndrome.

UNLABELLED: The aim of this study was to evaluate fracture risk in patients with Guillain-Barré syndrome (GBS). No association with risk of fracture was observed for GBS patients compared with controls. Only GBS patients using pain treatment had a doubled risk of fracture. INTRODUCTION: Symptoms of Guillain-Barré syndrome (GBS) may vary from mild difficulty in walking to complete paralysis, which may increase the risk of fractures. Therefore, the aim of this study was to evaluate fracture risk in patients with GBS. METHODS: We conducted a retrospective cohort study using the UK Clinical Practice Research Datalink (1987-2012). Each patient with GBS was matched by year of birth, sex, and practice, up to six patients without a history of GBS. Outcome measure was any fracture. RESULTS: There were no associations between GBS and any fracture, adjusted hazard ratio (AHR) 1.01 (95 % confidence interval [CI] 0.77-1.33), or osteoporotic fracture, AHR 0.76 (95 % CI 0.50-1.17), compared with controls. Stratification to gender, age, and duration since diagnosis did not show an association either. Only for GBS patients using pain treatment, risk of fracture was doubled AHR 1.97 (95 % confidence CI 1.21-3.21) compared with controls. The risk of fracture in GBS patients exposed to pain treatment was equivalent to risk of fracture among controls exposed to pain treatment. CONCLUSIONS: No association with risk of fracture was observed for GBS patients compared with controls. Only GBS patients using pain treatment had a doubled risk of fracture, but their risk was equivalent to fracture risk among controls exposed to pain treatment.

Risk of fracture in patients with muscular dystrophies.

UNLABELLED: The aim of the study was to determine fracture risk in incident muscular dystrophy (MD) patients. Patients with MD are at a 1.4-fold increased risk of fracture as compared with population-based control patients. Risk further increased among elderly and female patients and among patients exposed to oral glucocorticoids. INTRODUCTION: Muscular dystrophies (MDs) are inherited diseases causing muscle weakness and thereby increase the risk of falling and detrimental effects on bone. Both are recognised risk factors for fracture. Therefore, the aim of this study was to determine the hazard ratio of fracture in patients with MD. METHODS: We conducted a retrospective cohort study using the UK General Practice Research Database (1987-2012). Each patient with MD was matched by year of birth, sex and practice to up to six patients without a history of MD. Outcome measure was all fractures. RESULTS: As compared with control patients, risk of any fracture was statistically significantly increased in MD patients (adjusted hazard ratio [AHR], 1.40; 95 % confidence interval [CI], 1.14-1.71). An increased risk of fracture was observed among MD patients with female gender (AHR, 1.78; 95 % CI, 1.33-2.40) and an increasing age as compared with control patients. Stratification to Duchenne MD showed no association with fracture, whereas risk of fracture was increased twofold among patients with myotonic dystrophy (AHR, 2.34; 95 % CI, 1.56-3.51). MD patients had an almost tripled risk of fracture when they used oral glucocorticoids in the previous 6 months as compared to non-users with MD. CONCLUSION: Patients with MD are at a 1.4-fold increased risk of fracture as compared with population-based control patients. Especially in older age groups and female gender, the fracture risk of MD versus non-MD patients is increased, whereas exposure to glucocorticoids further increased fracture risk among MD patients.

Risk of fracture in patients with Parkinson's disease.

UNLABELLED: The aim of the study was to determine fracture risk in incident Parkinson's disease (PD) patients. This study showed that fracture risk assessment may be indicated among patients with PD, in particular when they have recently used selective serotonin re-uptake inhibitors or high-dose antipsychotics, or have a history of fracture, falling, low body mass index (BMI) or renal disease. INTRODUCTION: PD is a movement disorder associated with falling and detrimental effects on bone. Both are recognized risk factors for fracture. Therefore, the aim was to determine fracture risk in incident PD patients stratified by treatment, severity, duration of disease and related comorbidities. METHODS: We conducted a retrospective cohort study using the UK General Practice Research Database (1987-2011). Each PD patient was matched by age, sex, calendar time and practice to a control patient without history of PD. RESULTS: We identified 4,687 incident PD patients. Compared to controls, a statistically significant increased risk was observed for any fracture (adjusted hazard ratio [AHR], 1.89; 95 % confidence interval [CI], 1.67-2.14), osteoporotic fracture (AHR, 1.99; 95 % CI, 1.72-2.30) and hip fracture (AHR 3.08; 95 % CI, 2.43-3.89). Fracture risk further increased with history of fracture, falling, low BMI, renal disease, antidepressant use and use of high-dose antipsychotics. CONCLUSION: This study showed that incident PD patients have a statistically significant increased risk of fracture. Therefore, fracture risk assessment may be indicated among PD patients, who, besides the general risk factors for fracture, like increasing age and female gender, have recently used selective serotonin re-uptake inhibitors or high-dose antipsychotics or have a history of fracture, falling, low BMI or renal disease.

The reliability of transcranial duplex scanning in parkinsonian patients: comparison of different observers and ultrasound systems.

PURPOSE: To establish the inter-observer and intra-transducer reliability of "on-line" and "off-line" assessment of substantia nigra (SN) and raphe nuclei (RN) by transcranial duplex scanning (TCD) in a mixed study population. MATERIALS AND METHODS: Out-patient neurology department of the University Hospital Maastricht. In total 24 subjects were investigated: 9 patients with idiopathic Parkinson's disease, 10 with parkinsonism from yet unclear origin, 1 with essential tremor and 4 healthy volunteers. Each patient was assessed four times by two independent experienced sonographers using two different ultrasound devices: SONOS 5500 and iU22; both Philips, Eindhoven, The Netherlands. The echointensity of the SN is evaluated qualitatively and quantitatively and the RN only qualitatively. 1. In the "on-line" assessment we determined: a) the inter-observer agreement of the four possible combinations. b) the intra-observer agreement of both sonographers using two different ultrasound systems. 2. In the "off-line" assessment a third sonographer re-examined the stored images. We determined the inter-observer agreement of the third sonographer with the "on-line" assessment of the other two sonographers. Cohen's k value was calculated for the agreement. RESULTS: 1a) The "on-line" inter-observer agreement of the four possible combinations of sonographer and transducer was: kappa 0.23 - 0.39 for the qualitative evaluation of the SN, kappa 0.31 - 0.56 for the quantitative evaluation of the SN and kappa 0.03 - 0.15 for the evaluation of the RN. 1b) The "on-line" intra-observer agreement was: kappa 0.53 - 0.67 for the qualitative evaluation of the SN, kappa 0.55 - 0.76 for the quantitative evaluation of the SN and kappa 0.45 - 0.47 for the evaluation of the RN. 2. The "off-line" inter-observer agreement was: kappa 0.32 - 0.67 for the qualitative evaluation of the SN, kappa 0.53 - 0.61 for the quantitative evaluation of the SN and kappa 0.08 - 0.33 for the evaluation of the RN. CONCLUSION: For the SN we found mediocre accordance comparing both observers "on-line" with each other as well as comparing an "off-line" observer with both "on-line" observers. On the whole, the inter-observer and intra-observer agreement were moderate to substantial for the evaluation of the SN. "On-line" and "off-line" comparisons yielded comparable results. The agreement for the evaluation of the RN, on the contrary, was considerably lower. Our findings indicate that this TCD technique is not yet ready for the application in large population screenings.

Predictive value of clinical characteristics in patients with suspected cauda equina syndrome.

BACKGROUND AND PURPOSE: Overlooking a potential diagnosis of cauda equina syndrome (CES) can result in severe long-term neurologic deficits. There is a growing trend to order urgent magnetic resonance imaging (MRI) scans of the lumbar spine in any patient presenting with signs suspicious for CES. A substantial number of these MRI scans do not show cauda compression. The purpose of this study is to assess whether clinical characteristics can predict MRI-confirmed cauda compression. METHODS: We retrospectively studied 58 consecutive cases of suspected CES who presented at our hospital's emergency room. RESULTS: Eight of 58 patients had cauda compression on MRI. When measured, MRI + CES patients (6) had more than 500 ml urinary retention. Moreover, when these patients had at least two of the following characteristics: bilateral sciatica, subjective urinary retention or rectal incontinence symptoms, MRI was more probable to demonstrate cauda compression with an OR of 48.00, 95% (CI 3.30-697.21), which was also significant (P of 0.04). The presence of other symptoms or signs alone was not significantly different between both groups. CONCLUSION: In our series, urinary retention of more than 500 ml alone or in combination with two or more specific clinical characteristics were the most important predictors of MRI confirmed cauda compressions.

The ictal bradycardia syndrome.

The ictal bradycardia syndrome is an uncommon diagnosis in which bradycardia is accompanied by simultaneous epileptic discharges in the EEG. We describe a patient who was referred to the emergency ward because of syncope. Ictal semeiology and EEG-EG findings are discussed and compared with those published in the literature. Therapeutic options are discussed in relation with those published in the literature. The ictal bradycardia syndrome is probably underdiagnosed, while its recognition is of utmost importance because of potential life threatening complications such as asystole. Up to now, its aetiology is poorly understood, its ictal semeiology is often described insufficiently and its therapy is still discussed.

Fine analysis of cytolytic and natural killer T lymphocytes in the CSF in multiple sclerosis and other neurologic diseases.

Cytolytic T lymphocyte precursors (CTL-p) and natural killer precursors (NK-p) in the CSF of 15 MS patients and 11 patients with other neurologic disorders (OND) were quantitatively assessed, using a T-lymphocyte microculture system that allows clonal expansion of all human T cells. CSF CTL-p and NK-p frequencies were higher in patients with OND of inflammatory nature than in patients with noninflammatory OND. In all MS patients, these frequencies were higher in the CSF than in their peripheral blood. Surprisingly, in all patients studied, the CSF contained a substantial number of CTL-p with a helper (CD4+) phenotype.

HLA-DQ1 associated with reflex sympathetic dystrophy.

Reflex sympathetic dystrophy (RSD) is a relatively common disabling disorder of unknown pathophysiology. From a cohort of 52 patients, carefully selected to fulfill the recently formulated diagnostic criteria for RSD, venous blood samples were taken for typing of class I and II major histocompatibility antigens. The frequency of HLA-DQ1 was found to be significantly increased compared with control frequencies. The association provides an indication for an organic basis of RSD.

In vitro functional blocking of myelin basic protein-specific cytolytic human T lymphocyte clones by immunosuppressive drugs and monoclonal antibodies.

The in vitro effects of cyclosporin A, prednisolone, and anti-CD4 monoclonal antibody WW.T4 on myelin basic protein-specific human CD4+ cytolytic T lymphocyte clones were studied. Functional assays of antigen-specific proliferation, induction of specific lysis, cytolysis itself, and interferon-gamma production were done. Prednisolone decreased secretion of interferon-gamma by the clones and blocked specific proliferation; the latter could, however, be overcome by the addition of exogenous interleukin 2. It did not influence cytolytic properties. In contrast, cyclosporin A and WW.T4 blocked the four antigen-specific functions of the autoimmune myelin basic protein-specific human T cell clones measured.

Myelin basic protein-specific CD4+ cytolytic T-lymphocyte clones isolated from multiple sclerosis patients.

In the present report we describe the isolation and characterization of stable, long-term, human T-lymphocyte clones specific for myelin basic protein (MBP) from multiple sclerosis (MS) patients. Isolation of these clones appeared possible only by seeding peripheral blood mononuclear cells into a limiting dilution microculture system containing MBP, autologous irradiated cells and Interleukin-2 (IL-2), thereby minimizing effects of putative suppressor cell populations. All clones obtained were of the CD4+ phenotype. The majority was capable of MBP-specific cytolysis, tested with 51Chromium-labeled autologous Epstein-Barr virus (EBV)-transformed B-cells, coated with MBP, as targets. A few other clones had natural killer (NK) function. All clones produced Interleukin-2 (IL-2) upon adequate stimulation.

Prospective study of home morphine infusion in 62 terminally ill patients.

The project "Infusion Therapy at Home" studied the practice of parenteral infusion therapy at home. Among the treatment modalities was the administration of morphine infusions to terminally ill patients. With infusion of morphine, patients were pain-free on 73% of the treatment days. Quality of care, quality of life, and costs were acceptable.

Electrical spinal cord stimulation in reflex sympathetic dystrophy: retrospective analysis of 23 patients.

OBJECT: The aim of the study was to assess retrospectively the clinical efficacy and possible adverse effects of electrical spinal cord stimulation (SCS) for the treatment of patients with reflex sympathetic dystrophy (RSD). METHODS: Twenty-three patients who suffered severe pain due to RSD were included in the study. The SCS system was implanted only after a positive 1-week test period. The visual analog scale (VAS) score for pain (1-10) was obtained in all patients prior to treatment, 1 month postimplantation, and at last follow up. At final follow-up examination, patients were asked to rate the effect of their treatment on the 7-point global perceived effect scale. Eighteen (78%) of 23 patients treated between 1991 and 1997 reported improvement during the test period. Permanent implantation of SCS system was not performed in the other five patients. Complications occurred in nine (50%) of 18 patients. The system was removed in three patients after implantation (17%). At the end of follow up (mean 32 months) 15 patients still had an implanted system. The mean pain score had decreased from 7.9 to 5.4 (p<0.001). In the other eight patients the pain score had not changed significantly. In 13 patients (57%) in whom the SCS system was implanted, clinical status had much improved or improved; these cases were regarded as successful. CONCLUSIONS: In this retrospective series, the majority of patients with RSD reported a subjective improvement after implantation of an SCS system.

Radiofrequency lesions of the stellate ganglion in chronic pain syndromes: retrospective analysis of clinical efficacy in 86 patients.

OBJECTIVE: Stellate ganglion (SG) blockade is used for the treatment of chronic pain syndromes in which the sympathetic nervous system is hypothesized to be involved. A possible treatment modality to achieve long-term pain reduction is blockade of the SG by means of a radiofrequency lesion (RF-SG). To evaluate the outcome of RF-SG as a therapy for different chronic pain syndromes, we reviewed 86 RF-SG procedures. DESIGN: Medical records containing treatment information were reviewed systematically. A systematic MEDLINE literature review search on SG blockade was also performed. RESULTS: In our clinic, 39.5% of 221 patients who received a prognostic SG block subsequently underwent RF-SG. Of these patients, 40.7% noted a more than 50% reduction of pain, 54.7% reported no effect on pain, and 4.7% showed worsening of pain. The mean follow-up interval was 52 weeks. The computer-assisted literature search resulted in 31 studies: 12 about complications and 19 about the efficacy of SG blockade. A review of these studies showed partial pain relief in 41.3% of patients, complete pain relief in 37.8%, and no pain relief in 20.9%. CONCLUSIONS: The efficacy of RF-SG blockade seems to be in line with that of other SG blockade procedures reported in the literature. Our retrospective study shows that an RF-SG block is most likely to be of benefit for patients suffering from complex regional pain syndrome type 2, ischemic pain, cervicobrachialgia, or postthoracotomy pain. Clinical efficacy remains to be proven in a randomized controlled trial, however.

Screening of patients with complex regional pain syndrome for antecedent infections.

OBJECTIVE: This study was designed to investigate whether Complex Regional Pain Syndrome type I (CRPS I) could be linked to any previous infection. PATIENTS: Fifty-two patients with CRPS I of one extremity were screened for the presence of antibodies against mostly neurotropic microorganisms. RESULTS: Of these 52 patients, none had antibodies against Treponema pallidum, Borrelia burgdorferi, or HTLV-1. Only four patients were positive for Campylobacter jejuni. For cytomegalovirus, Epstein-Barr virus, herpes simplex virus, and Toxoplasma gondii, seroprevalences were similar to control values. The total seroprevalence of Parvovirus B 19 in our CRPS population was 77%, which was significantly higher than in an independent Dutch population group (59%). Seroprevalence in lower extremity CRPS 1 (94%) was significantly higher than in upper extremity CRPS I patients (68%). In this study all patients were seropositive for varicella zoster virus (VZV) antibodies, but a high prevalence of VZV antibodies is similar to its prevalence in a normal population (>90%). CONCLUSIONS: In this study we found a significantly higher seroprevalence of Parvovirus B19 in CRPS I and this is most striking in lower extremity CRPS I patients. Further serologic research in other geographic areas is needed to provide additional information about a potential role of Parvovirus B 19 or other microorganisms in the etiopathogenesis of CRPS I.

Pain catastrophizing predicts pain intensity, disability, and psychological distress independent of the level of physical impairment.

OBJECTIVE: The aim of the current study was to examine the relation between catastrophizing and pain intensity, pain-related disability, and psychological distress in a group of patients with chronic pain, controlling for the level of physical impairment. Furthermore, it was examined whether these relations are the same for three subgroups of chronic pain patients: those with chronic low back pain, those with chronic musculoskeletal pain other than low back pain, and those with miscellaneous chronic pain complaints, low back pain and musculoskeletal pain excluded. DESIGN: Correlational, cross-sectional. PATIENTS AND SETTING: Participants in this study were 211 consecutive referrals presenting to a university hospital pain management and research center, all of whom had a chronic pain problem. RESULTS: Overall, chronic pain patients who catastrophize reported more pain intensity, felt more disabled by their pain problem, and experienced more psychological distress. Regression analyses revealed that catastrophizing was a potent predictor of pain intensity, disability, and psychological distress, even when controlled for physical impairment. No fundamental differences between the three subgroups were found in this respect. Finally, it was demonstrated that there was no relation between physical impairment and catastrophizing. CONCLUSIONS: It was concluded that for different subgroups of chronic pain patients, catastrophizing plays a crucial role in the chronic pain experience, significantly contributing to the variance of pain intensity, pain-related disability, and psychological distress. These relations are not confounded by the level of physical impairment. Some clinical implications of the results are discussed. Finally, the authors concluded that these results support the validity of a cognitive-behavioral conceptualization of chronic pain-related disability.

Cervicogenic headache: techniques of diagnostic nerve blocks.

Ther term cervicogenic headache (CEH) was introduced by Sjaastad and co-workers in 1983. In 1990 Sjaastad et al. published diagnostic criteria for CEH. In 1998 refinements of these criteria were published, emphasising the use of diagnostic nerve blocks in patients with CEH as important confirmatory evidence. However, the standardisation of diagnostic nerve blocks in the diagnosis of CEH remains to be defined. Herein we present an overview of diagnostic nerve blocks in the cervical area. Suggestions as to their role in the diagnosis of CEH are given.

Randomized trial of radiofrequency lumbar facet denervation for chronic low back pain.

STUDY DESIGN: A prospective double-blind randomized trial in 31 patients. OBJECTIVES: To assess the clinical efficacy of percutaneous radiofrequency denervation of the lumbar zygapophysial joints in reducing pain, functional disability, and physical impairment in patients with back pain originating from the lumbar zygapophysial joints. SUMMARY OF BACKGROUND DATA: Chronic low back pain is a major health problem in the industrialized world. A treatment option is percutaneous radiofrequency denervation of the lumbar zygapophysial joints. Its clinical efficacy has never been formally tested in a controlled trial. METHODS: Thirty-one patients with a history of at least 1 year of chronic low back pain were selected on the basis of a positive response to a diagnostic nerve blockade and subsequently randomly assigned to one of two treatment groups. Each patient in the radiofrequency treatment group (15 patients) received an 80 C radiofrequency lesion of the dorsal ramus of the segmental nerve roots L3, L4, and L5. Patients in the control group (n = 16) underwent an the same procedure but without use of a radiofrequency current. Both the treating physician and the patients were blinded to the group assignment. Before treatment, physical impairment, rating of pain, the degree of disability, and quality of life were assessed by a blinded investigator. RESULTS: Eight weeks after treatment, there were 10 success patients in the radiofrequency group (n = 15) and 6 in the sham group (n = 16). The unadjusted odds ratio was 3.3 (P = 0.05, not significant), and the adjusted odds ratio was 4.8 (P < 0.05, significant). The differences in effect on the visual analog scale scores, global perceived effect, and the Oswestry disability scale were statistically significant. Three, 6, and 12 months after treatment, there were significantly more success patients in the radiofrequency group compared with the sham group. CONCLUSIONS: Radiofrequency lumbar zygapophysial joint denervation results in a significant alleviation of pain and functional disability in a select group of patients with chronic low back pain, both on a short-term and a long-term basis.

Randomized controlled trial of percutaneous intradiscal radiofrequency thermocoagulation for chronic discogenic back pain: lack of effect from a 90-second 70 C lesion.

STUDY DESIGN: A prospective double-blind randomized trial in 28 patients. OBJECTIVES: To assess the clinical effect of percutaneous intradiscal radiofrequency thermocoagulation for reducing pain, functional disability, and physical impairment in patients with chronic discogenic low back pain. SUMMARY OF BACKGROUND DATA: Chronic discogenic low back pain is a challenging problem in western countries. A treatment option is radiofrequency heating of the affected disc. Its clinical efficacy has never been formally tested in a controlled trial. METHODS: Twenty-eight patients with a history of at least 1 year of chronic low back pain were selected on the basis of a diagnostic anesthetization of the lower intervertebral discs. Only patients with one putative painful level were selected and randomly assigned to one of two treatment groups. Each patient in the radiofrequency treatment group (n = 13) received a 90-second 70 C lesion of the intervertebral disc. Patients in the control group (n = 15) underwent the same procedure, but without use of radiofrequency current. Both the treating physician and the patients were blinded to the group assignment. Before treatment, physical impairment, rating of pain, the degree of disability, and quality of life were assessed by a blinded investigator. RESULTS: Eight weeks after treatment, there was one success in the radiofrequency group (n = 13) and two in the control group (n = 15). The adjusted and unadjusted odds ratio was 0.5 and 1.1, respectively (not significant). Also, visual analog scores for pain, global perceived effect, and the Oswestry disability scale showed no differences between the two groups. CONCLUSIONS: Percutaneous intradiscal radiofrequency thermocoagulation (90 seconds, 70 C) is not effective in reducing chronic discogenic low back pain.

Primary intracranial hypotension and abnormal radionuclide cisternography. Report of a case and review of the literature.

We report a case of spontaneous posture-dependent headache associated with primary intracranial hypotension (PIH). The spinal fluid pressure was 0 cm H2O. Radioisotope cisternography showed that the cerebrospinal fluid flow was normal at the level of the spinal cord, but was suggestive for a hyperabsorption or leakage of CSF below the craniocervical junction.

Parkinsonism in alcohol withdrawal: case report and review of the literature.

A case of severe, acute parkinsonism occurring in a 60-year-old man after cessation of chronic alcohol consumption, is reported. He recovered completely in 3 months without specific therapy. The literature on alcohol withdrawal parkinsonism including nine other cases, is reviewed.