scholarly journals Pediatric Optic Neuritis: Description of Four Cases and Review of the Literature

Children ◽  
2021 ◽  
Vol 8 (10) ◽  
pp. 855
Author(s):  
Anna Presicci ◽  
Maria Serra ◽  
Mariaclara Achille ◽  
Elvita Caputo ◽  
Lucia Margari
Keyword(s):  
Optic Neuritis ◽  
Recent Literature ◽  
Systemic Disease ◽  
Treatment Options ◽  
Igg Antibodies ◽  
Careful Evaluation ◽  
Demyelinating Disorders ◽  
Infective Etiology

Pediatric optic neuritis (PON) may be a clinically isolated and self-limiting event or may present in the context of underlying neurologic, infective, or systemic disease. PON has a high impact on the quality of life as it may or may not evolve into other acquired demyelinating syndromes (ADSs), such as multiple sclerosis (MS), neuromyelitis optica (NMO), or other syndromes related to the myelin oligodendrocyte glycoprotein IgG antibodies (MOG-IgG). These different PON phenotypes present variable clinical and radiological features, plasma and liquor biomarkers, and prognosis. We describe four pediatric cases presenting clinically with ON, with different etiopathogenetic pictures: one case had a probable infective etiology, while the others were associated with different demyelinating disorders (MS, NMO, syndrome related to MOG-IgG). We discuss the possible evolution of presenting ON in other ADSs, based on recent literature. A careful evaluation of the clinical and investigation findings and the natural course of PON is necessary to define its pathogenic pathway and evolution. Further prolonged follow-up studies are needed to highlight the predictors of PON evolution, its potential sequelae, and the best treatment options.

Decision analysis of treatment options for vestibular schwannoma

2011 ◽  
Vol 114 (2) ◽  
pp. 400-413 ◽  
Author(s):  
Robert G. Whitmore ◽  
Christopher Urban ◽  
Ephraim Church ◽  
Michael Ruckenstein ◽  
Sherman C. Stein ◽  
...  
Keyword(s):  
English Language ◽  
Treatment Options ◽  
Treatment Strategies ◽  
Complication Rates ◽  
Online Databases ◽  
Decision Analytical Model ◽  
The Mean ◽  
Meta Regression

Object Widespread use of MR imaging has contributed to the more frequent diagnosis of vestibular schwannomas (VSs). These tumors represent 10% of primary adult intracranial neoplasms, and if they are symptomatic, they usually present with hearing loss and tinnitus. Currently, there are 3 treatment options for quality of life (QOL): wait and scan, microsurgery, and radiosurgery. In this paper, the authors' purpose is to determine which treatment modality yields the highest QOL at 5- and 10-year follow-up, considering the likelihood of recurrence and various complications. Methods The MEDLINE, Embase, and Cochrane online databases were searched for English-language articles published between 1990 and June 2008, containing key words relating to VS. Data were pooled to calculate the prevalence of treatment complications, tumor recurrence, and QOL with various complications. For parameters in which incidence varied with time of follow-up, the authors used meta-regression to determine the mean prevalence rates at a specified length of follow-up. A decision-analytical model was constructed to compare 5- and 10-year outcomes for a patient with a unilateral tumor and partially intact hearing. The 3 treatment options, wait and scan, microsurgery, and radiosurgery, were compared. Results After screening more than 2500 abstracts, the authors ultimately included 113 articles in this analysis. Recurrence, complication rates, and onset of complication varied with the treatment chosen. The relative QOL at the 5-year follow-up was 0.898 of normal for wait and scan, 0.953 for microsurgery, and 0.97 for radiosurgery. These differences are significant (p < 0.0052). Data were too scarce at the 10-year follow-up to calculate significant differences between the microsurgery and radiosurgery strategies. Conclusions At 5 years, patients treated with radiosurgery have an overall better QOL than those treated with either microsurgery or those investigated further with serial imaging. The authors found that the complications associated with wait-and-scan and microsurgery treatment strategies negatively impacted patient lives more than the complications from radiosurgery. One limitation of this study is that the 10-year follow-up data were too limited to analyze, and more studies are needed to determine if the authors' results are still consistent at 10 years.

High-risk syndrome for neuromyelitis optica: a descriptive and comparative study

2011 ◽  
Vol 17 (6) ◽  
pp. 720-724 ◽  
Author(s):  
N Collongues ◽  
R Marignier ◽  
H Zéphir ◽  
F Blanc ◽  
S Vukusic ◽  
...  
Keyword(s):  
High Risk ◽  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Clinical Laboratory ◽  
Age At Onset ◽  
Igg Antibodies ◽  
Resonance Imaging ◽  
National Cohort ◽  
Magnetic Resonance Imaging Mri

Background: Neuromyelitis optica (NMO) frequently begins with a monofocal episode of optic neuritis or myelitis. A concept named high-risk syndrome (HRS) for NMO has been proposed for patients with monofocal episodes and NMO-IgG antibodies. Objective: To describe HRS patients and compare them with NMO patients. Methods: We identified 30 patients with HRS: 18 with extensive myelitis (HRM) and 12 with optic neuritis (HRON), in a database pooling patients from 25 centres in France. Clinical, laboratory/magnetic resonance imaging (MRI) data and outcome were analysed and compared with a national cohort of 125 NMO patients extracted from the same database. Results: Mean follow-up was 4.8 years. Mean age at onset was 42.8 years (range: 12.4–70) with a female:male ratio of 0.9. Asymptomatic lesions were report on visual evoked potentials in 4/8 tested HRM patients and on spinal cord MRI in 2/7 HRON patients. Three patients died, two owing to a cervical lesion. HRS and NMO patients had similar clinical/paraclinical data, except for a predominance of men in the HRS group and a later mean age at onset in the HRM subgroup. Conclusion: The description of HRS patients is compatible with a monofocal form of NMO. Asymptomatic lesions could be included in a new set of NMO diagnostic criteria.

scholarly journals Multiple Doses of Icatibant used during Pregnancy

2017 ◽  
Vol 8 (3) ◽  
pp. ar.2017.8.0210 ◽  
Author(s):  
Lauren W. Kaminsky ◽  
Theodore Kelbel ◽  
Fay Ansary ◽  
Timothy Craig
Keyword(s):  
Adverse Events ◽  
Literature Search ◽  
Medical Literature ◽  
Case Reports ◽  
Treatment Options ◽  
Multiple Treatment ◽  
Multiple Doses ◽  
Healthy Infants

Background Hereditary angioedema (HAE) is a life-long disease that often manifests by puberty. Treatment of attacks is essential to improve quality of life and to decrease morbidity and mortality. During pregnancy, treatment is limited because multiple treatment options, including icatibant, are not approved for use during pregnancy. Objective We report the outcomes of three pregnancies during which icatibant was used by a patient with HAE with normal C1-inhibitor for treatment of attacks. We also reviewed the literature for reports of icatibant use during pregnancy for outcomes and adverse events. Methods We report on a patient who treated herself with icatibant during three separate pregnancies. Postpartum follow-up verified the health of the mother and children. We also performed a complete literature search of medical literature data bases on icatibant use during pregnancy. Results The patient in our report administered multiple doses of icatibant during three pregnancies. The child born from the first pregnancy and the child from the third pregnancy were born at term and without congenital anomalies. The child from the second pregnancy was 1-month preterm. All three children were developmentally normal. The literature search identified two case reports and one abstract of limited icatibant use without adverse events during pregnancy in patients with HAE. These pregnancies resulted in the births of healthy infants. Conclusion From a search of the literature, three cases of icatibant use during pregnancy resulted in healthy infants. In addition, we report that from icatibant use in three separate pregnancies, one infant was born prematurely, but there were no birth defects. From follow-up, the children continued meeting developmental milestones. This report adds to the acquisition of knowledge for drug adverse events during postmarketing surveillance for icatibant use during pregnancy.

scholarly journals Amelogenesis Imperfecta, hypoplastic type associated with some dental abnormalities: a case report

2010 ◽  
Vol 21 (2) ◽  
pp. 170-174 ◽  
Author(s):  
Emin Murat Canger ◽  
Peruze Çelenk ◽  
Murat Yenísey ◽  
Selcen Zeynep Odyakmaz
Keyword(s):  
Quality Of Life ◽  
Treatment Options ◽  
Masticatory Muscles ◽  
Amelogenesis Imperfecta ◽  
Radiographic Examination ◽  
Temporomandibular Joints ◽  
Related Quality ◽  
Health Related

Amelogenesis imperfecta (AI) is a hereditary disorder expressing a group of conditions that cause developmental alterations in the structure of enamel. AI is a serious problem that reduces oral health-related quality of life and causes some physiological problems. The treatment of patients with AI may upgrade the quality of life and reinforce their self-esteem. Among the treatment options for AI, full-mouth metal reinforced porcelain restoration constitutes an important alternative because of its properties. This paper presents a case of AI of the hypoplastic rough type associated with a group of dental anomalies, and describes the prosthetic management of the patient. A 26-year-old female patient presented with a chief complaint of discolored teeth. Clinical and radiographic examination of the patient confirmed the diagnosis of rough pattern hypoplastic AI. The patient was treated with full-mouth metal reinforced porcelain fixed bridge. The adaptation of the temporomandibular joints and masticatory muscles was carefully observed periodically during 4 months and, after this period, the patient tolerated well her new vertical dimension. The patient received instructions on cleansing of the subpontic and interproximal areas. Follow-up visits were scheduled at 3 months and then at 6 months. No esthetic or functional problems were seen after the follow up period.

scholarly journals Stoma Complications

Acta Medica ◽  
2019 ◽  
Vol 50 (2) ◽  
pp. 47-52
Author(s):  
Timuçin Erol
Keyword(s):  
Treatment Options ◽  
Early Postoperative Period ◽  
Stoma Creation ◽  
Quick Recovery ◽  
Stoma Complications ◽  
Post Operative Period ◽  
Life Threatening ◽  
The Ideal

An improper stoma creation can cause many complications, varying from minor to life-threatening ones. Conversely, a good functioning stoma, at the ideal site improves patient’s quality of life. Most of the stoma complications occur in the early postoperative period and all clinicians must be familiar to these complications. All measures before and during operation must be taken to avoid these complications. Careful follow up after post operative period can help early diagnosis, proper treatment quick recovery of the patient. This review focus on common complications and treatment options of stoma creation.  

First Clinical Experience with Anti-myelin Oligodendrocyte Glycoprotein Antibody-Positive Optic Neuritis

2020 ◽  
Vol 237 (04) ◽  
pp. 458-463 ◽  
Author(s):  
Jan Heckmann ◽  
Margarita Todorova ◽  
Stefanie Müller ◽  
Philip Julian Broser ◽  
Veit Sturm
Keyword(s):  
Visual Acuity ◽  
Optic Neuritis ◽  
Pulse Therapy ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Individual Treatment ◽  
Igg Antibodies ◽  
Intravenous Methylprednisolone ◽  
Finger Counting ◽  
The Right

Abstract Background Antibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) have been consistently found in a range of demyelinating disorders. In this context, MOG-IgG-associated optic neuritis (ON) has been suggested as a new subset of optic neuropathy. However, clinical manifestations and distinctive characteristics have only rarely been described. Patients and Methods A retrospective case series of three patients with MOG-IgG-associated ON. Clinical morphological features using imaging techniques are presented. Results Three patients (8-year-old boy, 28-year-old female, 48-year-old male) were included. An 8-year-old boy suffered from a bilateral ON with severe visual loss. The best-corrected visual acuity (BCVA) was 0.05 in the right eye and finger counting in the left eye. The patient had a previous episode of acute disseminated encephalomyelitis (ADEM) with a right abducens nerve palsy. Visual acuity recovered after repeated cycles of intravenous methylprednisolone pulse therapy and 10 cycles of plasma exchange. During the last follow-up, BCVA was 0.9 in the right eye and 0.8 in the left eye. A 28-year-old female presented with a bilateral ON. Her BCVA was 0.5 in the right eye and 0.8 in the left eye. She fully recovered with pulse methylprednisolone therapy (1000 mg/d) with tapering after the second cycle and had a BCVA of 1.0 during the last follow-up visit. A 48-year-old male suffered from a relapsing bilateral ON. At first presentation, BCVA was 0.1 in the right eye and finger counting in the left eye. BCVA fully recovered after each pulse therapy with intravenous methylprednisolone (two cycles). Since the first relapse, the patient has been receiving long-term immunosuppression with rituximab. Despite rituximab and low-dose oral prednisone, the patient had another relapse with a left ON. After a third cycle with intravenous methylprednisolone, he partially recovered. BCVA at last follow-up was 1.0 in the right and 0.8 in the left eye. Conclusions MOG-IgG antibodies have been identified in different acquired demyelinating syndromes. The patients reported had an ADEM followed by bilateral ON, an isolated bilateral ON, and a relapsing bilateral ON. Individual treatment strategies led to substantial visual recovery in all patients. We recommend inclusion of MOG-IgG antibodies in the diagnostic workup at least after the first recurrence of ON since they can serve as a diagnostic and potential prognostic tool and might lead to specific therapeutic recommendations.

Comparative effectiveness of treatment options for pediatric craniopharyngiomas

2014 ◽  
Vol 13 (2) ◽  
pp. 178-188 ◽  
Author(s):  
Zarina S. Ali ◽  
Robert L. Bailey ◽  
Lawrence B. Daniels ◽  
Venus Vakhshori ◽  
Daniel J. Lewis ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Treatment Guidelines ◽  
Treatment Options ◽  
Surgical Approaches ◽  
Quality Adjusted Life Years ◽  
Life Years ◽  
Subtotal Removal ◽  
Quality Adjusted Life

Object No clear treatment guidelines for pediatric craniopharyngiomas exist. The authors developed a decision analytical model to evaluate outcomes of 4 surgical approaches for craniopharyngiomas in children, including attempted gross-total resection (GTR), planned subtotal removal plus radiotherapy, biopsy plus radiotherapy, and endoscopic resections of all kinds. Methods Pooled data, including the authors' own experience, were used to create evidence tables, from which incidence, relative risks, and summary outcomes in quality-adjusted life years (QALYs) were calculated for the 4 management strategies. Results Quality-adjusted life years at the 5-year follow-up were 2.3 ± 0.1 for attempted GTR, 2.9 ± 0.2 for planned subtotal removal plus radiotherapy, 3.9 ± 0.2 for biopsy plus radiotherapy, and 3.7 ± 0.2 for endoscopic resection (F = 17,150, p < 0.001). Similarly, QALYs at 10-year follow-up were 4.5 ± 0.2 for attempted GTR, 5.7 ± 0.5 for planned subtotal removal plus radiotherapy, and 7.8 ± 0.5 for biopsy plus radiotherapy (F = 6,173, p < 0.001). On post hoc pairwise comparisons, the differences between all pairs compared were also highly significant (p < 0.001). Since follow-up data at 10 years are lacking for endoscopic cases, this category was excluded from 10-year comparisons. Conclusions Biopsy with subsequent radiotherapy is the preferred approach with respect to improved overall quality of life. While endoscopic approaches also show promise in preserving quality of life at five-year follow-up, there are not sufficient data to draw conclusions about this comparison at 10 years.

scholarly journals Looking beyond the thyroid: advances in the understanding of pheochromocytoma and hyperparathyroidism phenotypes in MEN2 and of non-MEN2 familial forms

2018 ◽  
Vol 25 (2) ◽  
pp. T15-T28 ◽  
Author(s):  
Carole Guerin ◽  
Pauline Romanet ◽  
David Taieb ◽  
Thierry Brue ◽  
André Lacroix ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Adrenal Insufficiency ◽  
Adrenal Cortex ◽  
Recent Literature ◽  
Age At Diagnosis ◽  
Familial Hypocalciuric Hypercalcemia ◽  
Phenotypic Features ◽  
Optimal Quality

Over the last years, the knowledge of MEN2 and non-MEN2 familial forms of pheochromocytoma (PHEO) has increased. In MEN2, PHEO is the second most frequent disease: the penetrance and age at diagnosis depend on the mutation ofRET. Given the prevalence of bilateral PHEO (50% by age 50), adrenal sparing surgery, aimed at sparing a part of the adrenal cortex to avoid adrenal insufficiency, should be systematically considered in patients with bilateral PHEO. Non-MEN2 familial forms of PHEO now include more than 20 genes: however, only small phenotypic series have been reported, suggesting that phenotypic features of isolated hereditary PHEO must be better explored, and follow-up series are needed to better understand the outcome of patients carrying mutations of these genes. The first part of this review will mainly focus on these points. In the second part, a focus will be given on MEN2 and non-MEN2 familial forms of hyperparathyroidism (HPTH). Again, the management of MEN2 HPTH should be aimed at curing the disease while preserving an optimal quality of life by a tailored parathyroidectomy. The phenotypes and outcome of MEN1-, MEN4- and HRPT2-related HPTH are briefly described, with a focus on the most recent literature data and is compared with familial hypocalciuric hypercalcemia.

Can CSF predict the course of optic neuritis?

1998 ◽  
Vol 4 (3) ◽  
pp. 132-135 ◽  
Author(s):  
J L Frederiksen
Keyword(s):  
Optic Neuritis ◽  
Population Based ◽  
Igg Antibodies ◽  
Definite Multiple Sclerosis ◽  
Clinically Definite Multiple Sclerosis ◽  
Quantitative Analyses ◽  
Oligoclonal Igg ◽  
Paired Serum ◽  
Oligoclonal Igg Bands

To discuss the implications of CSF abnormalities for the course of acute monosymptomatic optic neuritis (AMON), various CSF markers were analysed in patients being randomly selected from a population-based cohort. Paired serum and CSF were obtained within a few weeks from onset of AMON. CSF-restricted oligoclonal IgG bands, free kappa and free lambda chain bands were observed in 17, 15, and nine of 27 examined patients, respectively. Sixteen patients showed a polyspecific intrathecal synthesis of oligoclonal IgG antibodies against one or more viruses. At 1 year follow-up five patients had developed clinically definite multiple sclerosis (CDMS); all had CSF oligoclonal IgG bands and virus-specific oligoclonal IgG antibodies at onset. Due to the relative small number studied at the short-term follow-up, no firm conclusion of the prognostic value of these analyses could be reached. CSF Myelin Basic Protein-like material was increased in only two of 29 patients with AMON, but may have potential value in reflecting disease activity, as the highest values were obtained among patients with CSF sampled soon after the worst visual acuity was reached, and among patients with severe visual impairment. In most previous studies of patients with AMON qualitative and quantitative analyses of CSF IgG had a predictive value for development of CDMS, but the results are conflicting.

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