ISRN Neurology最新文献

Increasingly, spasticity is managed with surgically implanted Intrathecal Baclofen pumps. Intrathecal Baclofen pump revision surgery unrelated to programmable pump end-of-life is not uncommon, requiring special attention during pre-, intra-, and postoperative management. We aimed to identify and describe complications of Intrathecal Baclofen pump as well as to report avoidance and management of complications. Methods and Materials. Through 2002-2006, at the department of neurosurgery, Henry Ford and Oakwood Health Systems, Intrathecal Baclofen pumps were implanted in 44 patients: 24 children versus 20 adults; 30 "primary-implant-patients"; 14 "revision-only patients". We evaluated reasons for revision surgeries and diagnostic workup requirements. Results. Eight primary-implant-patients required 14 revisions and 7 of revision-only patients needed 13 procedures. Seven patients with slowly increasing baclofen-resistant spasticity had either (i) unsuspected pump-catheter connector defects, (ii) an X-ray-documented pump-catheter connector defect, (iii) X-ray-demonstrated fractured catheter with intrathecal fragment. Implant infections occurred in 4 cases. Scintigraphy revealed occult CSF leakage N=1 and intrinsic pump failure N=1. Conclusion. Intrathecal Baclofen pumps, although very gratifying, have a high, technique-related complication incidence during implant life. Meticulous technique, high clinical suspicion, appropriate workup, and timely surgical management can reduce surgical complications of Intrathecal Baclofen pump implantation.

The causal gene(s) for familial adult myoclonic epilepsy (FAME) remains undetermined. To identify it, an exome analysis was performed for the proband in a Japanese FAME family. Of the 383 missense/nonsense variants examined, only c.5720G>A mutation (p.Arg1907His) in the UBR5 gene was found in all of the affected individuals in the family, but not in the nonaffected members. Such mutation was not found in any of the 85 healthy individuals in the same community nor in any of the 24 individuals of various ethnicities. The present study demonstrated an FAME-associated mutation in the UBR5 gene, which is located close to the reported locus linked to Japanese FAME families.

Constipation and fecal incontinence are common in patients with neuromuscular diseases. Despite their high prevalence and potential impact on overall quality of life, few studies have addressed anorectal dysfunction in patients with multiple sclerosis (MS). The goal of this paper is to define the prevalence, pathophysiology, impact, and potential treatment of constipation and incontinence in MS patients. Methods. The PubMed database was searched for English language publications between January 1973 and December 2011. Articles were reviewed to assess the definition of the study population, duration, type and severity of MS, sex distribution, prevalence, impact, results of physiologic testing, and treatments. Results. The reported prevalence of constipation and fecal incontinence ranged around 40%. Anorectal dysfunction significantly affected patients with nearly 1 in 6 patients limiting social activities or even quitting work due to symptoms. Caregivers listed toileting as a common and significant burden. The only randomized controlled trial showed a marginal improvement of constipation with abdominal massage. All other reports lacked control interventions and only demonstrated improvement in individuals with milder symptoms. Conclusion. Anorectal dysfunction is a common manifestation in MS that significantly affects quality of life. Therapies are at best moderately effective and often cumbersome, highlighting the need for simple and more helpful interventions.

The author hypothesized that multiple sclerosis (MS) is a humoral autoimmune disease, caused by faulty interplay between myelin-specific, dimeric IgE, specifically competing non-IgE antibodies and IgE-triggered degranulating mast cells. The principal fault was believed to be insufficient quantity of protective, specific non-IgE antibodies. Also conjectured was the possibility of an unexpected and adverse immune suppression caused by none-MS pharmaceuticals being consumed by patients for their MS or for other conditions. To test both hypotheses, a mimotopic, peptide antigen-based, serum immunoassay was developed to measure dimer-bound IgE excess among MS patients, wherein the IgE specifically complexes with two or more myelin surface epitopes at an interval of 40-100 Angstroms, a separation critical for mast cell degranulation and cell damaging effect. MS test sensitivity and specificity, when analyzing five previously untreated patients for dimeric IgE presence, was 100%. In direct comparison, twenty age- and gender-matched female and male control subjects were test negative. Analysis of 35 multiple sclerosis patients, who were concomitantly being treated with potentially immunosuppressive pharmaceuticals, appeared to show the substances' negative effect upon MS causation, progression, or specific immunoassay performance. Therefore, MS is likely an autoimmune disease caused by IgE-mediated mast cell degranulation possibly in conjunction with immunosuppressive agents.

The present study examines the ability of melatonin to protect striatal dopaminergic loss induced by 6-OHDA in a rat model of Parkinson's disease, comparing the results with L-DOPA-treated rats. The drugs were administered orally daily for a month, their therapeutic or dyskinetic effects were assessed by means of abnormal involuntary movements (AIMs) and stepping ability. At the cellular level, the response was evaluated using tyrosine hydroxylase immunoreactivity and striatal ultrastructural changes to compare between L-DOPA-induced AIMs and Melatonin-treated rats. Our findings demonstrated that chronic oral administration of Melatonin improved the alterations caused by the neurotoxin 6-OHDA. Melatonin-treated animals perform better in the motor tasks and had no dyskinetic alterations compared to L-DOPA-treated group. At the cellular level, we found that Melatonin-treated rats showed more TH-positive neurons and their striatal ultrastructure was well preserved. Thus, Melatonin is a useful treatment to delay the cellular and behavioral alterations observed in Parkinson's disease.

A total of 25 consecutive patients suffering from degenerative cervical disc disease who underwent three-level anterior cervical discectomy and fusion (ACDF) including polyetheretherketone (PEEK) cages packed with allograft were followed up for at least two years. The fusion rate reached 72% (18/25), and asymptomatic pseudarthrosis was seen in 6 patients but without mobility on flexion-extension radiographs, and revision surgery was not needed. Cage subsidence occurred at one level (C67), but it was not progressive, and reoperation was not necessary. A significant increase (P < 0.001) in fused segment angle (FSA) and fused segment height (FSH) was observed postoperatively. Similarly, a significant clinical improvement (P < 0.001) was demonstrated postoperatively in terms of Japanese Orthopedic Association (JOA) score and visual analog scales (VASs) score. PEEK cages alone with allograft proved to be a safe and effective surgical option in the treatment of three-level degenerative cervical disc disease. Although the fusion rate was not high, this technique may offer improvement of symptomatology and maintenance of cervical spine's sagittal profile.

Although therapy switch is common among patients with multiple sclerosis (MS), sometimes the initial prescribed treatment is maintained for a long period with clinical stability, low disability, and nonsignificant side effects. We aim to describe demographic and clinical characteristics of patients treated in our MS clinic with the same disease-modifying drug (DMD) lasting for >12 years. From the cohort of 51 patients followed in our MS clinic with relapse-remitting MS who started an DMD between 1996 and 1999, we found a high percentage (51%) of patients who were efficiently treated with the first DMD. These patients were mainly females, with low annualized relapse rate and Multiple Sclerosis Severity Score (MSSS). Our results may be related to the open and multidisciplinary model of our MS clinic organization. Identifying characteristics associated with therapy persistence may be useful in developing strategies to improve therapy effectiveness.

Introduction. The objective of this study was to perform an economic analysis in terms of annual national human capital resource loss from young stroke mortality in Fiji. The official retirement age is 55 years in Fiji. Method. Stroke mortality data, for working-age group 15-55 years, obtained from the Ministry of Health and per capita national income figure for the same year was utilised to calculate the total output loss for the economy. The formula of output loss from the economy was used. Results. There were 273 stroke deaths of which 53.8% were of working-age group. The annual national human capital loss from stroke mortality for Fiji for the year was calculated to be F$8.85 million (US$5.31 million). The highest percentage loss from stroke mortality was from persons in their forties; that is, they still had more then 10 years to retirement. Discussion. This loss equates to one percent of national government revenue and 9.7% of Ministry of Health budget for the same year. The annual national human capital loss from stroke mortality is an important dimension in the overall economic equation of total economic burden of stroke. Conclusion. This study demonstrates a high economic burden for Fiji from stroke mortality of young adults in terms of annual national human capital loss.