Multiple Case Series Research Articles

Overt use of a tactile/kinaesthetic strategy shifts to covert processing in rehabilitation of letter-by-letter reading

Background: Letter-by-letter readers identify each letter of the word they are reading serially in left to right order before recognising the word. When their letter naming is also impaired, letter-by-letter reading is inaccurate and can render even single word reading very poor. Tactile and/or kinaesthetic strategies have been reported to improve reading in these patients, but only under certain conditions or for a limited set of stimuli. Aims: The primary aim of the current study was to determine whether a tactile/kinaesthetic treatment could significantly improve reading specifically under normal reading conditions, i.e., reading untrained words presented in free vision and read without overt use of the strategy. Methods & Procedures: Three chronic letter-by-letter readers participated in a tactile/kinaesthetic treatment aimed at first improving letter-naming accuracy (phase 1) and then letter-by-letter reading speed (phase 2). In a multiple case series design, accuracy and speed of reading untrained words without overt use of the trained tactile/kinaesthetic strategy was assessed before phase 1, after phase 1, and again after phase 2. Outcomes & Results: All three patients significantly improved both their speed and accuracy in reading untrained words without overt use of the trained tactile/kinaesthetic strategy. All three patients required the additional practice in phase 2 to achieve significant improvement. Treatment did not target sentence-level reading, yet two of the three patients became so adept that they could read entire sentences. Conclusions: This study replicates previous findings on the efficacy of tactile/kinaesthetic treatment for letter-by-letter readers with poor letter naming. It further demonstrates that this treatment can alter cognitive processing such that words never specifically trained can be read in free vision without overtly using the trained strategy. The data suggest that an important element in achieving this level of generalisation is continuing training beyond the point of initial mastery (i.e., accurate letter naming).

P-015 Mid term and long term results with second generation Matrix2 detachable coils

PurposeBioactive polyglycolic/polylactic acid coated matrix detachable coils (Boston Scientific, Natick, Massachusetts, USA) were reported to incite intra-aneurysmal inflammation and fibrosis. Multiple large case series have shown no significant advantage with...

Sexual intercourse and cerebral aneurysmal rupture: potential mechanisms and precipitants

Aneurysmal subarachnoid hemorrhage (SAH) is a significant cause of death in young and middle-aged individuals and causes tremendous morbidity in affected patients. Despite the identification of various risk factors, the series of events leading to the formation, growth, and rupture of intracranial aneurysms is poorly understood. Cerebral aneurysm rupture has been associated with sexual intercourse and other forms of physical exercise. In fact, multiple case series reported that coitus was the immediate preceding activity in 3.8-14.5% of patients suffering from aneurysmal SAH. This may be related to the large elevations in mean arterial blood pressure that occur in both males and females during sexual intercourse (130-175 and 125-160 mm Hg, respectively). While coitus and physical exercise share important physiological similarities, each may differentially affect the probability that a preformed aneurysm will rupture. In this literature review and synthesis, the authors analyze the physiological human response to sexual intercourse in an effort to delineate those factors that may precipitate aneurysmal rupture. The authors' analysis is based on the original data collected by Masters and Johnson. To the authors' knowledge, this is the first review to address the link between sexual intercourse and intracranial aneurysmal rupture. While actual measurements of the physiological variables relevant to SAH were not performed in this article, the authors make reasonable assumptions based on the available data to help elucidate the mechanism of sexually induced aneurysmal rupture.

Enterra® Therapy: gastric neurostimulator for gastroparesis

Gastroparesis is a chronic disorder of gastric motility characterized by delayed gastric empting in the absence of mechanical obstruction, which can lead to symptoms of nausea, vomiting, bloating, abdominal pain, postprandial fullness and weight loss. Although there are many etiologies, the primary causes are diabetes or are idiopathic. The mainstay of treatment is dietary and drug therapies. However, many patients will continue to suffer intractable symptoms despite these treatments. Gastric neurostimulation with the Enterra® Therapy system has been approved for use under the Humanitarian Device Exemption by the US FDA. The device produces pulses of electrical stimulation that are delivered to the stomach continuously. One randomized clinical trial and multiple nonrandomized unblinded clinical trials and case series have documented improvement of symptoms in intractable diabetic and idiopathic gastroparesis. The purpose of this article is to introduce the Enterra Therapy gastric neurostimulator. Gastroparesis and its pathophysiology will be discussed in this clinical context to enhance the understanding of the device and its development. We will analyze the device in detail, its placement and the results of studies evaluating its efficacy.

Varicocele Repair in Patients With Nonobstructive Azoospermia: A Meta-Analysis

Multiple small case series have reported sperm in the ejaculate and spontaneous pregnancies in patients with nonobstructive azoospermia after varicocele repair. We hypothesized that men with favorable testicular histopathology on testis biopsy such as maturation arrest or hypospermatogenesis would have a higher probability of success than those with more ablative pathology, eg Sertoli-cell-only. A review of the literature on varicocele repair in patients with nonobstructive azoospermia was performed and 11 publications from the previous 20 years were evaluated. Histopathological data were presented in 8 publications, and were categorized as Sertoli-cell-only, maturation arrest and hypospermatogenesis. Maturation arrest was further differentiated by 4 publications. Early maturation arrest was defined as maturation ending at the secondary spermatocyte and late maturation arrest was defined as maturation ending at the spermatid without spermatozoa present. Success after repair was defined as having sperm in the ejaculate or spontaneous pregnancy. A total of 233 patients were analyzed. After varicocele repair 91 (39.1%) patients had motile sperm in the ejaculate and 14 spontaneous pregnancies were reported. Success rates in patients with maturation arrest (42.1%) or hypospermatogenesis (54.5%) were significantly higher than in those with Sertoli-cell-only (11.3%, p <0.001 in both groups). Patients with late maturation arrest had a higher probability of success (45.8%) than those with early maturation arrest (0%, p = 0.007). Infertile men with nonobstructive azoospermia can have improvement in semen analysis and achieve spontaneous pregnancy after repair of clinical varicoceles. This meta-analysis demonstrates that men with late maturation arrest and hypospermatogenesis have a higher probability of success and, therefore, histopathology should be considered before varicocele repair in men with nonobstructive azoospermia.

Opioids for palliation of refractory dyspnea in chronic obstructive pulmonary disease patients

Dyspnea, a distressing symptom, in chronic obstructive pulmonary disease patients is often unrelieved. The purpose of this article is to examine the efficacy of opioids administered orally, in nebulized form and other routes in dyspnea relief. Additionally, factors that inhibit the prescription of opioids and use of opioids are explored. Although there are multiple case reports and case series, there is a paucity of well designed, prospective, randomized controlled trials with large enough number of chronic obstructive pulmonary disease patients. One review of randomized controlled trials and another randomized controlled trial found opioids effective in relieving dyspnea without causing major adverse effects. Opioid is an effective palliative drug in chronic obstructive pulmonary disease patients with distressing dypnea that is refractory to standard modalities of treatment.

Low-Energy Femoral Fractures Associated With Long-Term Bisphosphonate Use in a Rehabilitation Setting: A Case Series

Bisphosphonate therapy for the treatment of postmenopausal osteoporosis is common, with more than 22 million prescriptions for alendronate (Merck) alone in 2005. The safety of this class of medications has been supported by long-term trials [1,2]. However, during the last several years multiple small case series link the long-term use of bisphosphonates to unusual complications such as avascular necrosis of the jaw and low-energy nonvertebral fractures that in some cases surprisingly demonstrate severely suppressed bone turnover [3] .I t may be that the suppressed turnover prevents effective repair of stress fractures. Recently reported cases of nonvertebral fractures include multiple small series describing an unusual fracture of the femur, either subtrochanteric or diaphyseal, in patients taking long-term alendronate [4-7]. This fracture may be preceded by vague complaints of pain in the thigh in the setting of “low or no trauma.” There may be local cortical thickening and a fracture pattern that is simple and horizontally situated [4]. Biopsies in some of these patients have demonstrated a frozen bone pattern [3]. This pattern reveals depression of bone formation. At the authors’ freestanding rehabilitation hospital in the last 7 years, 1 male and 3 postmenopausal female patients with 5 atypical low-impact or no-impact femoral fractures were identified. One was subtrochanteric and 4 were diaphyseal. Two of these patients were receiving extended alendronate therapy; one had received zoledronic acid infusions as an adjunctive therapy for multiple myeloma. One patient was found to be hypogonadal and osteopenic. These cases add to the data that suggest the long-term use of bisphosphonates is associated with a risk of low-energy femoral fractures. CASE 1 A 53-year-old white man with a history of osteoporosis, hypertension, coronary artery disease, gastroesophageal reflux, asthma, psoriatic arthritis, and obsessive-compulsive disorder complained of right-sided hip and knee pain. While walking, he felt a sudden pain in his right leg and fell to the floor. He was taken for immediate plain radiographs, which revealed a right transverse mid-femur fracture. He underwent an intramedullary nailing. He was transferred to acute inpatient rehabilitation and then discharged home without an assistive device. Endocrine consultation revealed a testosterone level of less than 50 ng/dL (normal 212-755 ng/dL) and a luteinizing hormone level of less than 0.1 mIU/mL (normal 20-140 U/mL). He was diagnosed with hypogonadism, which was thought to contribute to his insufficiency fracture. He was treated with topical androgen (Solvay) for his hypogonadism and alendronate for his osteopenia. A DEXA scan obtained at the time of the fracture had a T score of the spine of 0.98, and the hip T score was 0.3 (the lower limit of normal). This fracture did not achieve a primary union, and required a reoperation to achieve a union.

Ablation of Barrett's esophagus using the HALO radiofrequency ablation system

The incidence of esophageal adenocarcinoma is rising at an epidemic rate. Barrett's esophagus (BE) is its only known precursor lesion and is an ideal target for endoprevention of esophageal adenocarcinoma. Recent advances in endoscopic therapy, especially radiofrequency ablation (RFA) using the HALO system have renewed interest in ablative therapies for the management of BE. RFA uses a bipolar electrode array to deliver thermal energy to the metaplastic and dysplastic Barrett's tissue for large-area circumferential and small-area focal ablation. Multiple prospective uncontrolled trials, case series, and registries have consistently shown safety and effectiveness of RFA in treating all stages of BE. A large multicenter sham-controlled trial showed that in patients with either low-grade or high-grade dysplasia, RFA is effective in ablating esophageal intestinal metaplasia and dysplasia and preventing progression to higher grades of dysplasia and cancer. In addition, cost-effectiveness models have shown ablation may be a cost-effective strategy for the management of all stages of BE. This article is a review of technical background of RFA using the HALO system and is a summary of the data on its current applications for the treatment of BE.

Role of endosonography in drainage of fluid collections and other NOTES procedures

Endosonography (EUS) has become the accepted procedure for drainage of pancreatic fluid collections in the past decade. EUS was shown to be safe and effective and it has been the first-line therapy for uncomplicated pseudocysts. Where walled-off pancreatic necrosis was originally thought to be a contraindication for endoscopic treatment, multiple case series have now shown that these fluid collections also can be treated endoscopically with low morbidity and mortality. Analogous to the treatment of pancreatic fluid collections, others, such as abscesses in the lower and upper abdomen, have also been treated successfully, although there is limited literature in this regard, EUS appears to be a useful technique in natural orifice transluminal endoscopic surgery (NOTES) procedures as well.

Clinical Effects of Colonic Cleansing for General Health Promotion: A Systematic Review

The practice of colonic cleansing to promote general health and well-being continues to generate interest among the lay population. These practices are widely touted as adjuncts to improve vitality and as therapeutic modalities to minimize the symptoms, or prevent the actual development, of a variety of chronic disease states. The data supporting colonic cleansing and body "detoxification" have not been studied well in a systematic manner. This report describes a systematic review of the published literature of both the traditional and complementary and alternative medicine arenas that was performed in an attempt to qualify and quantify the value of colonic cleansing. The investigators concluded that there are no methodologically rigorous controlled trials of colonic cleansing to support the practice for general health promotion. Conversely, there are multiple case reports and case series that describe the adverse effects of colonic cleansing. The practice of colonic cleansing to improve or promote general health is not supported in the published literature and cannot be recommended at this time.

Evaluation of Renal Artery Stenosis in Dialysis Patients

Atherosclerotic renal artery stenosis (RAS), recognized as a contributor to chronic kidney disease (CKD), may be present in a substantial fraction of dialysis patients. It is generally unknown what proportion of end-stage renal disease patients on dialysis could recover kidney function if RAS were treated. Patients with CKD are often inadequately screened for RAS because of technical limitations of various screening modalities. Multiple small case series and studies have evaluated the role of revascularization for renal salvage in dialysis patients with RAS; these studies are reviewed. Large prospective, randomized multicenter trials of intervention for RAS exclude patients with advanced CKD. The risks of intervention must be weighed against the potential for renal recovery, even when predictors of success are not known with certainty.

The use of drug-eluting stents in ST-elevation myocardial infarction

Drug-eluting stents (DES) dramatically lower rates of restenosis in patients undergoing percutaneous coronary interventions. However, their use has extended beyond the initial approved indications into off-label scenarios such as ST-elevation myocardial infarction (STEMI). Multiple retrospective case series and 10 prospective randomised controlled trials have been published on the use of DES in STEMI patients, and these generally show reduced rates of target vessel revascularisation with a neutral effect on mortality. Three meta-analyses show similar findings with the composite endpoint of death, recurrent myocardial infarction, or re-intervention significantly lower in the DES group, although this was largely driven by a decrease in target vessel revascularisation. These studies also demonstrate that DES use in the setting of STEMI is not associated with increased risk of stent thrombosis compared to bare metal stents. In summary, current data support the efficacy of DES in reducing restenosis and re-intervention in patients who present with STEMI. From a safety standpoint, the use of DES in STEMI does not increase the risk of stent thrombosis, and there is no difference in overall mortality. The use of DES in STEMI patients should be individualised on a case-by-case basis given the importance of compliance with dual antiplatelet therapy.

Advantages of the multiple case series approach to the study of cognitive deficits in autism spectrum disorder

In the neuropsychological case series approach, tasks are administered that tap different cognitive domains, and differences within rather than across individuals are the basis for theorising; each individual is effectively their own control. This approach is a mainstay of cognitive neuropsychology, and is particularly suited to the study of populations with heterogeneous deficits. However it has very rarely been applied to the study of cognitive differences in autism spectrum disorder (ASD). Here, we investigate whether this approach can yield information beyond that given by the typical group study method, when applied to an ASD population. Twenty-one high-functioning adult ASD participants and 22 IQ, age, and gender-matched control participants were administered a large battery of neuropsychological tests that would represent a typical neuropsychological assessment for neurological patients in the United Kingdom. The data were analysed using both group and single-case study methods. The group analysis revealed a limited number of deficits, principally on tests with a large executive function component, with no impairment in more routine abilities such as basic attending, language and perception. Single-case study analysis proved more fruitful revealing evidence of considerable variation in abilities both between and within ASD participants. Both sub-normal and supra-normal performance were observed, with the most defining feature of the ASD group being this variability. We conclude that the use of group-level analysis alone in the study of cognitive deficits in ASD risks missing cognitive characteristics that may be vitally important both theoretically and clinically, and even may be misleading because of averaging artifact.

Intensive (Daily) Behavior Therapy for School Refusal: A Multiple Baseline Case Series

The following multiple baseline case series examines school refusal behavior in 4 male adolescents. School refusal symptom presentation was ascertained utilizing a functional analysis from the School Refusal Assessment Scale (Kearney, 2002). For the majority of cases, treatment was conducted within a 15-session intensive format over a 3-week period. Treatment elements included cognitive-behavioral therapy with the adolescent, parent training sessions, or a combination of these strategies. Treatment was effective for 3 of 4 cases in the short term. At 3-year follow-up, all 3 of the acute treatment responders had switched to alternative educational programs, although parents rated them as significantly improved and less impaired compared to pretreatment. Obstacles to treatment, and recommendations for program improvement, are discussed.

Von Willebrand disease and other bleeding disorders in women: consensus on diagnosis and management from an international expert panel

Reproductive tract bleeding in women is a naturally occurring event during menstruation and childbirth. In women with menorrhagia, however, congenital bleeding disorders historically have been underdiagnosed. This consensus is intended to allow physicians to better recognize bleeding disorders as a cause of menorrhagia and consequently offer effective disease-specific therapies.

The Results of Laboratory Evaluation of Acute Knee Effusion After ACL Reconstruction: What is Normal? (SS-52)

The Results of Laboratory Evaluation of Acute Knee Effusion After ACL Reconstruction: What is Normal? (SS-52)

Ictal asystole—Late manifestation of partial epilepsy and importance of cardiac pacemaker

Ictal asystole (IA) is a life-threatening complication of epilepsy and is a potential mechanism of sudden unexplained death in epilepsy (SUDEP). This entity has been proven by multiple case reports and small case series. The management of the patients with IA is still in early phase of discussion. We report a patient with medically intractable cryptogenic partial epilepsy for 27 years who presented with new onset drop attacks. During the epilepsy monitoring unit stay he was found to have a left fronto-temporal partial onset seizures which triggered brady-arrhythmia followed by asystole for 20 s. A cardiac pacemaker was implanted and the patient was followed for 2 years. He continued to have simple and complex partial seizures but did not have drop attacks anymore. He still occasionally feels the activation of his pacemaker during simple partial phase of his seizures but the characteristic loss of muscle tone never happened again which made him highly satisfied. Our case demonstrates that IA can even happen decades after the onset of epilepsy. Cardiac pacemaker should be considered in all patients with IA as it prevents ictal falls and possibly SUDEP.

Baseline Pain and Disability in the Investigational Vertebroplasty Efficacy and Safety Trial

Multiple case series of vertebroplasty outcomes have been published, though no large, placebo controlled trial has yet been performed. Our aim was to report baseline characteristics for the Investigational Vertebroplasty Efficacy and Safety Trial (INVEST), a randomized blinded controlled study of vertebroplasty. We compared baseline demographics, pain scores, and scores on the modified Roland-Morris Disability Scale (RMDS), a back pain-specific metric, between 2 groups. One group included subjects enrolled at the lead INVEST site (n = 27 to date). The second group consisted of eligible patients seen concurrently at the lead INVEST site, who declined enrollment (n = 70). Comparisons were made by using 2-sample t tests. Mean ages were similar between groups, averaging approximately 74 years among study participants and 77 years among nonenrolled eligible patients (P = .17). Approximately 75% of subjects were female in both groups. RMDS scores of enrolled patients at the lead site (18.0 +/- 4.2) were not statistically different from those of eligible nonenrolled patients at the lead site (18.6 +/- 3.6, P = .49). Pain scores in the enrolled subjects were measured as "average intensity over the prior 24 hours" with mean scores of 7.6 +/- 2.1 among enrolled patients at the lead site. Pain scores in eligible nonenrolled patients were measured as "pain at rest," with mean score of 3.4 +/- 3.3, and "pain with activity," with mean score of 8.5 +/- 2.0. Patient demographics among subjects enrolled in the INVEST are similar to those in a cohort of eligible nonenrolled patients. Back pain-specific disability was similar between subjects enrolled in the INVEST study and eligible nonenrolled patients at the lead site.

Autoimmune pancreatitis

Autoimmune pancreatitis (AIP) is an increasingly recognized clinical condition. Our objective is to provide a concise review of the advances in the past year in our understanding of AIP. In a hospital survey from Japan, the prevalence of AIP was estimated at 0.82 per 100,000 individuals. The pathogenesis of AIP remains unclear but a recent report noted that T helper type 2 and T regulatory cells predominantly mediate the immune reaction in AIP. Genetic associations that may predispose to relapse of AIP were reported. Multiple case series further described the clinical profile of AIP and its extrapancreatic manifestations. A large series on immunoglobulin G4 (IgG4)-associated cholangitis noted that patients with IgG4-associated cholangitis presented with obstructive jaundice and had increased serum IgG4 levels and IgG4-positive cells in bile duct biopsy specimens. Tissue IgG4 staining is likely to be a useful adjunct to serological diagnosis. AIP is steroid-responsive but maintaining remission continues to remain challenging. Presently low-dose steroids or immunomodulators are being used but efficacy of these medications remains to be determined. There has been significant progress in understanding the clinical profile of AIP but knowledge of pathogenesis remains limited. Treatment practices vary widely and management of refractory disease continues to be challenging.

Congenital auditory meatal atresia: a numerical review

Congenital auditory meatal atresia (CAMA) is an uncommonly encountered disorder. Though a rare condition, CAMA poses multiple problems for affected children. Recent management of CAMA in developed countries rests on osseo-integrated prostheses and bone-anchored hearing aids. The situation is different in developing countries where aesthetic and otologic surgeries are the available lines of management. Surgical management of CAMA has been staged into reconstructive surgery for auricular deformity followed by external and middle ear reconstruction either via anterior or transmastoid approaches. Multiple case series describing the outcomes and complications of both approaches have been published, but no authors have attempted to compare either. We have attempted to compare the outcomes and complications of both approaches by analyzing published medical articles concerning surgical management of CAMA identified by searching the Medline database using "congenital aural atresia" and "external auditory canal atresia" as keywords. A total of 923 ears were reported by 13 articles included in this study. The transmastoid approach is shown, by multiple linear regression, to have better postoperative hearing gain and less likely restenosis.