Out of hours magnetic resonance imaging for suspected cauda equina syndrome: lessons from a comparative study across two centres.

BACKGROUND: Cauda equina syndrome, a rare but disabling spinal surgical emergency, requires prompt investigation, ideally using magnetic resonance imaging as patients may require decompressive surgery. Out of hours access to magnetic resonance imaging is only routinely available in major trauma centres and neurosurgical units. Patients in regional hospitals with suspected cauda equina syndrome may require transfer for diagnostic imaging. We retrospectively studied the proportion of patients referred with suspected cauda equina syndrome who required out of hours transfer for magnetic resonance imaging and decompressive surgery. MATERIALS ANDS METHODS: Retrospective cohort study of patients referred using online referral platforms with suspected acute cauda equina syndrome and transferred out of hours between 6pm to 8am on weekdays and all day on weekends to two of the largest neurosurgical units in the UK in Birmingham and Cambridge. RESULTS: A total of 441 patients were referred across both sites with a suspicion of acute cauda equina syndrome; 339 patients were transferred for diagnostic scans and only 16 of them (4.7%) were positive for cauda equina compression, necessitating prompt decompressive surgery. Of the patients with negative magnetic resonance scans, 50% had their discharge or transfer back to referring hospitals delayed by more than 24 hours. CONCLUSIONS: Over 95% of patients who were transferred for imaging did not undergo emergency decompression. The authors propose a greater role for the provision of out of hours magnetic resonance imaging in all hospitals admitting emergency patients to streamline management.

Irradiation influence on the phenoloxidase pathway and an anti-oxidant defense mechanism in Spodoptera litura (Lepidoptera: Noctuidae) and its implication in radio-genetic 'F 1 sterility' and biorational pest suppression tactics.

The present study was conducted to appraise the ontogenic radio-sensitivity of a serious tropical pest, Spodoptera litura (Fabr.). The molecular responses pertaining to the phenoloxidase (PO) pathway and an anti-oxidant defense mechanism were evaluated in order to understand its implication in pest control at pre-harvest and post-harvest intervals. Irradiation exhibited an inverse relationship with age with respect to impact on developmental and transcriptional responses. Transcript abundance of PO cascade enzymes, prophenoloxidase (slppo-2), its activating enzyme (slppae-1) and free-radical scavenging enzymes, superoxide dismutase (slsod) and catalase (slcat) was evaluated upon gamma irradiation alone and the dual-stress of radiation plus microbial challenge. The slppo-2, slppae-1, slsod and slcat transcripts were significantly up-regulated in F 1 L6 larvae (6th-instar) resulting from 100 Gy sub-sterilized male adults and unirradiated female moths. The extent of upregulation was relatively higher in comparison with L6 survivors (6th-instar larvae) developed from irradiated neonates (L1) treated with 100 Gy. Upon Photorhabdus challenge, the transcripts were down-regulated in irradiated L1 suggesting increased larval susceptibility to bacterial infections. Radioresistance increased with the age of the insect, and molecular responses (transcript abundance) of insect defense mechanism were less influenced when older age (F 1 progeny) were irradiated. These findings will help to optimize the gamma dose to be employed in inherited sterility technique for (pre-harvest) pest suppression and (post-harvest) phytosanitation and quarantine, and suggest compatible integration of biorational tactics including nuclear technology.

Mobile Right Atrial Thrombi in a Patient with the Hemoglobin SC Disease.

The formation of Intracardiac thrombi is rare in the absence of structural heart disease or atrial fibrillation. We describe a case of spontaneous right atrial thrombus formation that occurred in a patient with a hypercoagulable condition who had been sub optimally anticoagulated.

Coronary microvascular dysfunction in male patients with Anderson-Fabry disease and the effect of treatment with alpha galactosidase A.

OBJECTIVE: To measure coronary flow reserve (CFR), an index of microvascular function, in Anderson-Fabry disease (AFD) at baseline and after enzyme replacement therapy (ERT). METHODS AND RESULTS: Mean (SD) myocardial blood flow (MBF) at rest and during hyperaemia (adenosine 140 microg/kg/min) was measured in 10 male, non-smoking patients (53.8 (10.9) years, cholesterol 5.5 (1.3) mmol/l) and in 24 age matched male, non-smoking controls (52.0 (7.6) years, cholesterol 4.5 (0.6) mmol/l) by positron emission tomography (PET). Resting and hyperaemic MBF and CFR (hyperaemic/resting MBF) were reduced in patients compared with controls (0.99 (0.17) v 1.17 (0.25) ml/g/min, p < 0.05; 1.37 (0.32) v 3.44 (0.78) ml/g/min, p < 0.0001; and 1.41 (0.39) v 3.03 (0.85), p < 0.0001, respectively). This coronary microvascular dysfunction was independent of cholesterol concentrations. PET was repeated in five patients after 10.1 (2.3) months of ERT; resting and hyperaemic MBF and CFR were unchanged after ERT (0.99 (0.16) v 0.99 (0.16) ml/g/min; 1.56 (0.29) v 1.71 (0.3) ml/g/min; and 1.6 (0.37) v 1.74 (0.28), respectively; all not significant). CONCLUSIONS: The results of the present study show that patients with AFD have very abnormal coronary microvascular function. These preliminary data suggest that ERT has no effect on coronary microvascular dysfunction. Further work is necessary to determine whether treatment at an earlier stage in the course of the disease may improve coronary microvascular function in patients with AFD.

Prevalence and clinical significance of systolic impairment in hypertrophic cardiomyopathy.

OBJECTIVES: To determine the frequency of systolic impairment (SI) and its impact on the natural history of hypertrophic cardiomyopathy (HCM). METHODS: 1080 patients (mean (SD) age 43 (15) years, 660 men) with HCM were evaluated. Initial assessment included history, examination, 48 hour Holter monitoring, cardiopulmonary exercise testing, and echocardiography; SI was defined as a fractional shortening (FS) < or = 25%. Survival data were collected at clinic visits or by direct communication with patients and their general practitioners. The results of serial echocardiography in 462 patients with normal FS at presentation are also reported. RESULTS: 26 (2.4%) patients (49 (14) years, 18 men) had SI at the initial visit. During follow up (58 (49) months), nine (34.6%) died or underwent cardiac transplantation compared with 108 (10.2%) patients with normal FS (p = 0.01). Five year survival from death (any cause) or transplantation was 90.1% (95% confidence interval (CI) 87.8 to 92.4) in patients with normal systolic function versus 52.4% (95% CI 25.2 to 79.6, p < 0.0001) in patients with SI. In patients who underwent serial echocardiography, 22 (4.8%, aged 41 (15) years) developed SI over 66 (40) months; the annual incidence of SI was 0.87% (95% CI 0.54 to 1.31). On initial evaluation patients who developed SI had a higher frequency of syncope (67 (15.2%) v 10 (45.5%) of those who did not develop SI, p = 0.001), non-sustained ventricular tachycardia (91 (20.6%) v 11 (50%), p = 0.002), and an abnormal blood pressure response on exercise (131 (29.7%) v 15 (68.2%), p = 0.001). Patients with SI had greater wall thinning (p = 0.001), left ventricular cavity enlargement (p < 0.0005), and deterioration in New York Heart Association functional class (p = 0.001) during follow up. Thirteen (59.1%) patients who progressed to SI died or underwent transplantation compared with 38 (8.6%) patients who maintained normal systolic function. CONCLUSIONS: SI is an infrequent complication of HCM but, when present, is associated with a poor prognosis.

Serum ferritin levels in West Nile encephalitis.

West Nile encephalitis (WNE) presents clinically as aseptic meningitis, meningoencephalitis, encephalitis, or acute flaccid paralysis. Non-specific laboratory findings, e.g., leukopenia and thrombocytopenia, accompany WNE. Lymphopenia is marked and prolonged with WNE. Three patients with WNE were found to have elevated serum ferritin levels. Severity seemed to be directly related to serum ferritin levels. Although preliminary, the results suggested that serum ferritin levels >or= 500 ng/mL (normal range 5-187 ng/mL) occur late with WNE, and not in a control group of patients with viral meningitis or encephalitis.

Pregnancy related complications in women with hypertrophic cardiomyopathy.

OBJECTIVES: To determine whether pregnancy is well tolerated in hypertrophic cardiomyopathy. SETTING: Referral clinic. DESIGN: The study cohort comprised 127 consecutively referred women with hypertrophic cardiomyopathy. Forty (31.5%) underwent clinical evaluation before pregnancy. The remaining 87 (68.5%) were referred after their first pregnancy. All underwent history, examination, electrocardiography, and echocardiography. Pregnancy related symptoms and complications were determined by questionnaire and review of medical and obstetric records where available. RESULTS: There were 271 pregnancies in total. Thirty six (28.3%) women reported cardiac symptoms in pregnancy. Over 90% of these women had been symptomatic before pregnancy. Symptoms deteriorated during pregnancy in fewer than 10%. Of the 36 women with symptoms during pregnancy, 30 had further pregnancies. Symptoms reoccurred in 18 (60%); symptomatic deterioration was not reported. Heart failure occurred postnatally in two women (1.6%). No complications were reported in 19 (15%) women who underwent general anaesthesia and in 22 (17.4%) women who received epidural anaesthesia, three of whom had a significant left ventricular outflow tract gradient at diagnosis after pregnancy. Three unexplained intrauterine deaths occurred in women taking cardiac medication throughout pregnancy. No echocardiographic or clinical feature was a useful indicator of pregnancy related complications. CONCLUSIONS: Most women with hypertrophic cardiomyopathy tolerate pregnancy well. However, rare complications can occur and therefore planned delivery and fetal monitoring are still required for some patients.

Septal myotomy-myectomy and transcoronary septal alcohol ablation in hypertrophic obstructive cardiomyopathy. A comparison of clinical, haemodynamic and exercise outcomes.

AIMS: Surgical myectomy has been successfully used to treat patients with symptomatic obstructive hypertrophic cardiomyopathy (HCM). More recently, alcohol septal ablation has been advocated as a less invasive, but equally effective alternative therapy. The aim of this non-randomized cohort study was to compare subjective and objective outcomes in patients undergoing these therapies. METHODS: Forty-four patients (25 male; age 41+/-15 years) with symptomatic drug-refractory obstructive HCM were studied. Twenty-four patients underwent surgical myectomy and 20 alcohol septal ablation. All patients underwent clinical evaluation, echocardiography and upright maximal cardiopulmonary exercise testing using a cycle ergometer before and following their intervention. RESULTS: Peak gradient was reduced to a similar extent by both modalities (myectomy: 83+/-23 to 15+/-10 mmHg (P<0.000001); ablation: 91+/-18 to 22+/-14 mmHg (P<0.000002);P =0.48 for myectomy vs ablation) and led to similar improvements in NYHA class (myectomy: 2.4+/-0.6 to 1.5+/-0.7 (P<0.00001); ablation: 2.3+/-0.5 to 1.7+/-0.8 (P<0.0001);P=0.3 for myectomy vs ablation). Myectomy resulted in a greater improvement in peak oxygen consumption (myectomy: 16.4+/-5.8 to 23.1+/-7.1 ml.kg(-1) min(-1) (P<0.00002); ablation: 16.2+/-5.2 to 19.3+/-6.1 ml.kg(-1) min(-1) (P<0.05);P <0.05 for myectomy vs ablation) and work rate achieved (myectomy: 130+/-57 to 161+/-60 watts (P<0.04); ablation: 121+/-53 to 137+/-51 watts (P=0.11);P <0.05 for myectomy vs ablation). CONCLUSION: Surgical myectomy and alcohol septal ablation are equally effective at reducing obstruction and subjective exercise limitation in appropriately selected patients. However, the superior effect of surgical myectomy on exercise test parameters suggests that surgery remains the gold standard against which new treatment modalities should be compared.

Prevalence of Anderson-Fabry disease in male patients with late onset hypertrophic cardiomyopathy.

BACKGROUND: Although studies have suggested that "late-onset" hypertrophic cardiomyopathy (HCM) may be caused by sarcomeric protein gene mutations, the cause of HCM in the majority of patients is unknown. This study determined the prevalence of a potentially treatable cause of hypertrophy, Anderson-Fabry disease, in a HCM referral population. METHODS AND RESULTS: Plasma alpha-galactosidase A (alpha-Gal) was measured in 79 men with HCM who were diagnosed at > or =40 years of age (52.9+/-7.7 years; range, 40-71 years) and in 74 men who were diagnosed at <40 years (25.9+/-9.2 years; range, 8-39 years). Five patients (6.3%) with late-onset disease and 1 patient (1.4%) diagnosed at <40 years had low alpha-Gal activity. Of these 6 patients, 3 had angina, 4 were in New York Heart Association class 2, 5 had palpitations, and 2 had a history of syncope. Hypertrophy was concentric in 5 patients and asymmetric in 1 patient. One patient had left ventricular outflow tract obstruction. All patients with low alpha-Gal activity had alpha-Gal gene mutations. CONCLUSION: Anderson-Fabry disease should be considered in all cases of unexplained hypertrophy. Its recognition is important given the advent of specific replacement enzyme therapy.

Isolated cardiac manifestations in Fabry disease: the UK experience.

UNLABELLED: Cardiac abnormalities are common in patients with Fabry disease, and may be the only clinical manifestation of the disease in some patients. At St George's Hospital Medical School, a national referral centre for hypertrophic cardiomyopathy in the UK, a study of 153 consecutively referred male patients revealed that 4% had Fabry disease. This increased to 6% in patients over 40 years of age. All these patients had electrocardiographic (ECG) abnormalities, most of which were consistent with those found in patients with classic Fabry disease: left ventricular hypertrophy, repolarization abnormalities and a prolonged QRS complex. A study of heterozygote females with Fabry disease also showed ECG and echocardiographic abnormalities in most patients. CONCLUSIONS: Fabry disease should be considered in the differential diagnosis of otherwise unexplained cardiac disease.