eNeurologicalSciPub Date : 2023-06-01DOI: 10.1016/j.ensci.2023.100468
Hany A. Zaki , Haris Iftikhar , Mavia Najam , Maarij Masood , Nood Dhafi R. Al-Marri , Mohamed Abdelgadir M. Elgassim , Mohamed Fayed , Eman E. Shaban
{"title":"Plasma exchange (PE) versus intravenous immunoglobulin (IVIG) for the treatment of Guillain-Barré syndrome (GBS) in patients with severe symptoms: A systematic review and meta-analysis","authors":"Hany A. Zaki , Haris Iftikhar , Mavia Najam , Maarij Masood , Nood Dhafi R. Al-Marri , Mohamed Abdelgadir M. Elgassim , Mohamed Fayed , Eman E. Shaban","doi":"10.1016/j.ensci.2023.100468","DOIUrl":"10.1016/j.ensci.2023.100468","url":null,"abstract":"<div><h3>Background and purpose</h3><p>Guillain- Barré syndrome (GBS) is a neuropathic condition that leads to the rapid development of impairments and is characterized by weakness and numbness or tingling sensation in the legs and arms and sometimes loss of movement and feeling in the legs, arms, upper body, and face. Currently, the cure for the disease is yet to be developed. However, treatment options such as intravenous immunoglobulin (IVIG) and plasma exchange (PE) have been used to minimize the symptoms and duration of the disease. Therefore, this systematic review and meta-analysis compared the efficacy of IVIG and PE in treating GBS patients with severe symptoms.</p></div><div><h3>Methodology</h3><p>Six electronic databases, including PubMed, Embase, Scopus, ScienceDirect, Medline, and Google scholar, were scoured for articles related and relevant to our research. Additionally, more studies were obtained through the reference lists of the studies retrieved from these electronic databases. Quality assessment and statistical data analysis were conducted using Review Manager software (RevMan 5.4.1).</p></div><div><h3>Results</h3><p>The search for relevant articles resulted in 3253 articles, of which only 20 were included for review in the current study. A sub-group analysis indicated no significant difference in the curative effect (Hughes score reduces by at least one score 4 weeks after GBS treatment; OR: 1.00; 95% CI: 0.66–1.52; <em>p</em> = 1.00 and Achieving grade 0 or 1 on Hughes scale; OR: 1.03; 95% CI: 0.27–3.94; <em>p</em> = 0.97). Similarly, the statistical showed that the difference in length of hospitalization and duration of mechanical ventilation was insignificant between the IVIG and PE group (Standard Mean Difference (SMD): -0.45; 95% CI: −0.92, 0.02; I<sup>2</sup> = 91%; <em>p</em> = 0.06 and SMD: -0.54; 95% CI: −1.67, 0.59; I<sup>2</sup> = 93%; <em>p</em> = 0.35, respectively). Moreover, the meta-analysis did not find any significant difference in the risk of GBS relapse (RR: 0.47; 95% CI: 0.20–1.14; <em>p</em> = 0.10) and risk of complications related to the treatment regimens (RR: 1.03; 95% CI: 0.71–1.48; <em>p</em> = 0.89). However, the statistical analysis of outcomes from 3 studies showed that the risk of discontinuation was significantly lower in the IVIG group than in the PE group (RR: 0.22; 95% CI: 0.06–0.88; <em>p</em> = 0.03).</p></div><div><h3>Conclusion</h3><p>Our study suggests that IVIG and PE have similar curative effects. Similarly, IVIG seems easier to use and thus can be preferred for treating GBS.</p></div>","PeriodicalId":37974,"journal":{"name":"eNeurologicalSci","volume":"31 ","pages":"Article 100468"},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10242495/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9597813","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
eNeurologicalSciPub Date : 2023-06-01DOI: 10.1016/j.ensci.2023.100457
Sabrina Mata , Matteo Bussotti , Monica Del Mastio , Alessandro Barilaro , Patrizia Piersanti , Maria Lombardi , Massimo Cincotta , Sara Torricelli , Deborah Leccese , Martina Sperti , Gabriele Rosario Rodolico , Benedetta Nacmias , Sandro Sorbi
{"title":"Epidemiology of amyotrophic lateral sclerosis in the north east Tuscany in the 2018–2021 period","authors":"Sabrina Mata , Matteo Bussotti , Monica Del Mastio , Alessandro Barilaro , Patrizia Piersanti , Maria Lombardi , Massimo Cincotta , Sara Torricelli , Deborah Leccese , Martina Sperti , Gabriele Rosario Rodolico , Benedetta Nacmias , Sandro Sorbi","doi":"10.1016/j.ensci.2023.100457","DOIUrl":"10.1016/j.ensci.2023.100457","url":null,"abstract":"<div><h3>Background</h3><p>The incidence of Amyotrophic Lateral Sclerosis (ALS) varies among different geographical areas and seems to increase over time. This study aimed to examine the epidemiologic data of ALS in the north-east Tuscany and compare the results with those of similar surveys.</p></div><div><h3>Methods</h3><p>Data from ALS cases diagnosed in Florence and Prato Hospitals were prospectively collected from 1st June 2018 to 31st May 2021.</p></div><div><h3>Results</h3><p>The age- and sex-adjusted incidence rate of ALS in cases per 100,000 population was 2.71 (M/F ratio: 1.21), significantly higher as compared to that reported in the 1967–1976 decade in the same geographical area (0.714). The age- and sex-adjusted incidence rate among resident strangers was similar to that of the general population (2.69). A slightly higher incidence rate (4.36) was observed in the north-east area of Florence province, which includes the Mugello valley. The mean prevalence was of 7.17/100,00. The mean age at diagnosis was 69.7 years, with a peak between 70 and 79 years among men and a smoother age curve among women.</p></div><div><h3>Conclusions</h3><p>ALS epidemiological features in north-east Tuscany are in line with other Italian and European Centers. The dramatic increase of the local disease burden over the last decades probably reflects better ascertainment methods and health system.</p></div>","PeriodicalId":37974,"journal":{"name":"eNeurologicalSci","volume":"31 ","pages":"Article 100457"},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10063398/pdf/main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9241701","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Fulminant Guillain–Barré syndrome secondary to Campylobacter coli infection: An autopsy case report","authors":"Fumiya Kutsuna , Momoko Soeda , Aiko Hibino , Masahiro Tokuda , Shiro Miura , Hiroshi Iwanaga","doi":"10.1016/j.ensci.2023.100454","DOIUrl":"https://doi.org/10.1016/j.ensci.2023.100454","url":null,"abstract":"<div><p>The most common infection preceding Guillain–Barré syndrome (GBS) is <em>Campylobacter jejuni</em> enteritis, although a few patients present with <em>Campylobacter coli</em>. We report a case of <em>C. coli</em>–induced fulminant GBS. A 61-year-old woman presented with bilateral limb weakness. Nerve conduction studies revealed a reduction of amplitude and <em>C. coli</em> was isolated from a fecal specimen, leading to the diagnosis of GBS. Although the patient was immediately administered immunoglobulin, her symptoms rapidly worsened and she died. Peripheral nerve autopsy revealed myelin ovoid, and infiltration of CD68-positive macrophages into nerves. More effective treatments for fulminant GBS need to be developed.</p></div>","PeriodicalId":37974,"journal":{"name":"eNeurologicalSci","volume":"31 ","pages":"Article 100454"},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"50201413","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
eNeurologicalSciPub Date : 2023-06-01DOI: 10.1016/j.ensci.2023.100460
Antonio Cruz-Culebras
{"title":"A rare challenge: A patient with progressive fibrodysplasia ossificans and acute ischemic stroke treated with mechanical thrombectomy.","authors":"Antonio Cruz-Culebras","doi":"10.1016/j.ensci.2023.100460","DOIUrl":"10.1016/j.ensci.2023.100460","url":null,"abstract":"<div><p>Fibrodysplasia ossificans progressiva (FOP) is a rare, disabling genetic condition characterized by congenital malformations of the great toes and progressive heterotopic ossification. Here, we briefly describe the case of a 56-year-old male with known FOP and acute ischemic stroke that underwent mechanical thrombectomy with conscious sedation. Treating physicians should be aware of special medical considerations to prevent flare-ups and inflammation that result from any tissue injuries in this disease. Mechanical thrombectomy is a challenging scenario because general anesthesia and injections should be avoided in these patients. The treatment is still preventive and supportive, but this is the first report of the procedure in a patient with FOP.</p></div>","PeriodicalId":37974,"journal":{"name":"eNeurologicalSci","volume":"31 ","pages":"Article 100460"},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10121383/pdf/main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9391095","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
eNeurologicalSciPub Date : 2023-06-01DOI: 10.1016/j.ensci.2023.100469
Moustafa A. Mansour , Yehia Moawad , Hassan Ali
{"title":"Beta-propeller protein-associated neurodegeneration: A clinical update with a case report","authors":"Moustafa A. Mansour , Yehia Moawad , Hassan Ali","doi":"10.1016/j.ensci.2023.100469","DOIUrl":"10.1016/j.ensci.2023.100469","url":null,"abstract":"","PeriodicalId":37974,"journal":{"name":"eNeurologicalSci","volume":"31 ","pages":"Article 100469"},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10311142/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9742922","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
eNeurologicalSciPub Date : 2023-06-01DOI: 10.1016/j.ensci.2023.100467
Isabella Fabbri , Fabio Betti , Roberto Tedeschi
{"title":"Gait quality after robot therapy compared with physiotherapy in the patient with incomplete spinal cord injured: A systematic review","authors":"Isabella Fabbri , Fabio Betti , Roberto Tedeschi","doi":"10.1016/j.ensci.2023.100467","DOIUrl":"10.1016/j.ensci.2023.100467","url":null,"abstract":"<div><h3>Background</h3><p>Spinal cord injury results in the interruption of neuronal conduction in the spinal cord, a condition that occurs in 0.1% of the world's population. This results in severe limitations in autonomy including locomotor function. Its recovery can be pursued through conventional isolated physiotherapeutic rehabilitation (overground walking training - OGT) or associated with Robot-assisted gait training - RAGT (<em>e.g.</em>: Lokomat ®).</p></div><div><h3>Aim</h3><p>The aim of this review is to compare the effectiveness of RAGT combined with conventional physiotherapy.</p></div><div><h3>Methods</h3><p>The databases consulted, from March 2022 to November 2022, were PubMed, PEDro, Cochrane Central Register of Controlled Trials (Cochrane Library) and CINAHL. RCT studies of people with incomplete spinal cord injuries treated with RAGT and/or OGT with the aim of improving walking were analysed.</p></div><div><h3>Results</h3><p>Among the 84 RCTs identified, 4 were included in the synthesis, with a total of 258 participants. The outcomes analysed concerned both locomotor function through lower limb muscle strength and the need for assistance in walking, using the WISCI-II scale and the LEMS. Robotic treatment stimulated the greatest improvements in the four studies; however, they were not always statistically significant.</p></div><div><h3>Conclusion</h3><p>A rehabilitation protocol combining RAGT with conventional physiotherapy is more effective than isolated OGT in improving ambulation in the subacute phase.</p></div>","PeriodicalId":37974,"journal":{"name":"eNeurologicalSci","volume":"31 ","pages":"Article 100467"},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/e6/9c/main.PMC10248036.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9992983","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
eNeurologicalSciPub Date : 2023-06-01DOI: 10.1016/j.ensci.2023.100450
Marian Irene C. Escasura , Jose C. Navarro , Stroke Data Bank Investigators
{"title":"Predictive factors for clinical outcomes in Filipino patients with watershed infarction: A single center study","authors":"Marian Irene C. Escasura , Jose C. Navarro , Stroke Data Bank Investigators","doi":"10.1016/j.ensci.2023.100450","DOIUrl":"10.1016/j.ensci.2023.100450","url":null,"abstract":"<div><h3>Background and purpose</h3><p>Watershed infarcts denote ischemic lesions involving the distal territories of two major arteries. For years, hypotheses on its pathophysiological mechanisms have been proposed. Yet, the cause is still widely debated. This study aimed to determine the mechanism of watershed strokes and compare their clinical outcomes to acute ischemic stroke from other causes and predict the factors affecting clinical outcomes in patients with watershed infarcts.</p></div><div><h3>Methods</h3><p>This single-center, comparative, six-years retrospective cohort study included patients with a diagnosis of Acute Ischemic Stroke. Patients were classified under watershed group or acute ischemic stroke based on their neuroimaging findings. Stroke mechanisms were determined between groups as well as the factors associated with clinical outcomes in watershed strokes.</p></div><div><h3>Results</h3><p>Among the 424 patients included in the study, large artery atherosclerosis was seen in greater frequency in patients with watershed infarcts regardless of the type (EWIs: <em>n</em> = 68, 73% vs IWIs: <em>n</em> = 89, 75%). No differences observed in the clinical outcomes between groups. Multiple variable analysis showed that age, female sex, high NIHSS score and presence of underlying malignancy were associated with clinical outcomes.</p></div><div><h3>Conclusion</h3><p>Clinical outcomes between watershed infarcts and acute ischemic strokes were similar. Hemodynamic compromise in the setting of severe stenosis is the underlying mechanism for both types of watershed strokes thus, the goal of treatment is to maintain adequate perfusion. High baseline NIHSS score, increased age, female gender and underlying malignancy were all poor predictors of clinical outcomes in patients with watershed strokes.</p></div>","PeriodicalId":37974,"journal":{"name":"eNeurologicalSci","volume":"31 ","pages":"Article 100450"},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/e7/c6/main.PMC9995929.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9102669","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"HTLV-1-associated demyelinating neuropathy: A case report and review of the literature","authors":"Keiko Tamaki , Takayasu Mishima , Yuji Tateishi , Hidekazu Mera , Hiromu Ogura , Jun Tsugawa , Shinsuke Fujioka , Yasushi Takamatsu , Yoshio Tsuboi","doi":"10.1016/j.ensci.2023.100453","DOIUrl":"10.1016/j.ensci.2023.100453","url":null,"abstract":"<div><p>A 78-year-old man developed paresthesias in the extremities. He was referred to our hospital because of positive anti-human T-cell leukemia virus type 1 (HTLV-1) antibodies in the serum and the presence of abnormal lymphocytes. He was diagnosed as chronic-type adult T-cell leukemia/lymphoma. Neurological examination revealed sensory impairment in the distal parts of the extremities with loss of deep tendon reflexes. Nerve conduction study showed motor and sensory demyelinating polyneuropathy, indicating a diagnosis of HTLV-1-associated demyelinating neuropathy. Corticosteroid therapy followed by intravenous immunoglobulin therapy improved his symptoms. Since demyelinating neuropathy associated with HTLV-1 infection is not well recognized, we here report its characteristics and clinical course through our case report and literature review.</p></div>","PeriodicalId":37974,"journal":{"name":"eNeurologicalSci","volume":"31 ","pages":"Article 100453"},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/c6/c8/main.PMC9986509.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9137734","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
eNeurologicalSciPub Date : 2023-06-01DOI: 10.1016/j.ensci.2023.100461
Satomi Hiya, Hajime Yoshimura, Michi Kawamoto
{"title":"Successful treatment with subcutaneous ofatumumab in an adolescent patient with refractory myelin oligodendrocyte glycoprotein-immunoglobulin G-associated disease (MOGAD)","authors":"Satomi Hiya, Hajime Yoshimura, Michi Kawamoto","doi":"10.1016/j.ensci.2023.100461","DOIUrl":"10.1016/j.ensci.2023.100461","url":null,"abstract":"<div><p>Preventing relapse of myelin oligodendrocyte glycoprotein-immunoglobulin G-associated disease (MOGAD) with steroids and immunosuppressants is sometimes difficult. There is no standard treatment for refractory cases. We present the case of a 17-year-old female patient with longitudinally extensive myelitis, asymptomatic bilateral optic neuritis, and positive serum MOG-IgG. While taking steroids and several immunosuppressants during the following 14 months, she suffered from two symptomatic relapses in the cerebrum and spinal cord, and multiple asymptomatic relapses in the cerebrum. The patient was negative for MOG-IgG at the second relapse of myelitis. Subcutaneous ofatumumab has suppressed relapse for 13 months. Ofatumumab can be considered a therapeutic option for refractory MOGAD.</p></div>","PeriodicalId":37974,"journal":{"name":"eNeurologicalSci","volume":"31 ","pages":"Article 100461"},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/9a/a2/main.PMC10139969.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9392583","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}