P.055 Clinical and Electrophysiological characteristics of anti-nodal/paranodal antibodies in chronic inflammatory demyelinating polyradiculoneuropathy patients

Background: CIDP is an autoimmune polyneuropathy. Antibodies against the Node of Ranvier have been described, NF155,NF140/186 and contactin-1. Methods: A retrospective review of patients with CIDP who tested positive for anti-nodal/paranodal antibodies via Western blot were evaluated. We have included 20 sero-negative CIDP patients. All patients met definite or probable EFNS criteria. clinical, electrophysiological data and response to treatment were obtained. Results: Forty-five patients tested positive for the antibodies. Sixteen were positive for NF155, 11 for NF140, 5 for CNTN1,11 were double positive for NF155 and NF140, and 3 were triple positive for NF155, NF140 and CNTN1. Age of onset was similar in both seronegative (53.9 ± 3.1 yrs.) versus seropositive (52.3 ± 2.4 yrs.). Chronic presentation manifested in 85% of seronegative, 80% of seropositive patients.Intrestingly,all triple-positive patients presented with a more acute presentation (i.e,<8 wks.) 7/20 seronegative (35%),1/16 NF155, 6/11 NF140,1/5 contactin, 2/11 of double-positive, 3/3 of triple-positive (28%,13/46) responded to IVIg. Conclusions: No major clinical or electrophysiological differences between groups. triple-positive patients showed 100% response to IVIg.These results cast doubt on the specificity of the Western blot as a clinico-electrophysiologic discriminator. Future testing with cell-based assays will likely provide a robust measure that will guide treatment decision.

Primary Brainstem Hemorrhage: A Review of Prognostic Factors and Surgical Management

Primary brainstem hemorrhage (PBSH) is the most fatal subtype of intracerebral hemorrhage and is invariably associated with poor prognosis. Several prognostic factors are involved, of which the two most predominant and consistent are the initial level of consciousness and hemorrhage size. Other predictors, such as age, hyperthermia, and hydrocephalus, are generally not dependable indicators for making prognoses. Scoring systems have now been developed that can predict mortality and functional outcomes in patients suffering from PBSH, which can thus guide treatment decision-making. A novel grading scale, entitled “the new primary pontine hemorrhage (PPH) score,” represents the latest approach in scoring systems. In this system, patients with a score of 2–3 points appear to benefit from surgical management, although this claim requires further verification. The four main surgical options for the treatment of PBSH are craniotomy, stereotactic hematoma puncture and drainage, endoscopic hematoma removal, and external ventricular drainage. Nevertheless, the management of PBSH still primarily involves conservative treatment methods and surgery is generally not recommended, according to current practice. However, the ongoing clinical trial, entitled Safety and Efficacy of Surgical Treatment in Severe Primary Pontine Hemorrhage Evacuation (STIPE), should provide additional evidence to support the surgical treatment of PBSH. Therefore, we advocate the update of epidemiological data and re-evaluation of PBSH treatment in a contemporary context.

Right Sided Bochdalek Hernia with Ureteric Involvement – A Case Report and Review of Management Options

Ureteric herniation through a diaphragmatic defect in adults (Bochdalek hernia – BH) is rare, with less than 15 cases reported in the literature. Most patients present with non-specific abdominal symptoms with or without worsening of renal function, and therefore, the clinical picture can be masked [1]. Management of ureteric involvement ranges from conservative to reconstructive surgery. We report a case of an elderly (>80-year-old) patient who presented acutely with symptoms mimicking an acute abdomen and on imaging was found to have a BH associated with herniation of right ureter causing hydro-ureter. We managed the patient conservatively, and she improved symptomatically, which was evident from her follow-ups. We provide a detailed case report of this situation as well as discuss the literature on the topic to guide treatment decision-making.

Ethical issues in geriatric cranial neurosurgery

The global demographic shift to an older population has led to the emergence of the new field of geriatric neurosurgery. Beyond the complexities of disease states and multimorbidity, advanced age brings with it intricate ethical issues pertaining to both the practice and provision of medical and surgical care. In this paper, the authors describe the central ethical themes seen across the spectrum of common neurosurgical conditions in the elderly and highlight the use of foundational ethical principles to help guide treatment decision-making.

SAT-423 Anti-thyroid Drug Response in Graves’ Disease: Predictors of Biochemically Persistent Disease

Introduction: Anti-thyroid Drugs (ATD) have become the most frequently used treatment for Graves’ disease (GD) in the United States. However, the response to this therapy is variable. Factors that predict biochemically responsive vs. biochemically persistent disease remain unknown. Identifying predictors of disease poorly responsive to ATD can help guide treatment decision making, follow up planning and prognosis. Methods: From a database of patients with GD treated with ATD and receiving care at an academic medical center between 2009–2019, we selected adults with incident GD treated with ≥14 days of ATD. Results: 172 patients (from a database of 730 patients with GD on ATD) were sampled for the purpose of this pilot and 97 of these met inclusion criteria. Patients had a median age of 50 (18–90); female, 70.1%; never smokers, 64.9%; median goiter size of 40 g (15–100); and median TRAb on presentation of 8.1 mIU/L (1.0- 60). Graves’ orbitopathy (GO) was present in 13.4% at baseline. Patients (100%) were started on methimazole at a median dose of 20 mg (2.5–60). The median time from presentation until biochemical improvement (defined as the first instance of FT4 ≤1.7 ng/dL) was 120.9 days (18–1525), and to biochemical euthyroidism (normal TSH & FT4) was 251 days (41–1259) including a median of 3 (0–17) dose adjustments. In a univariate analysis, response to ATD was divided into two groups; biochemically responsive and biochemically persistent disease (based on reaching biochemical improvement in ≤6 months, or &gt;6 months respectively). Biochemically persistent disease was more common in those with GO at presentation (38.5% vs.11.1%) (p .024). There was a trend towards greater prevalence of biochemically persistent disease in those with TRAb ≥ 8.0 mIU/L (46.2% vs. 27.8%) (p .204), and goiter estimated 30 grams or above by physical examination (30.8% vs. 19.4%) (p .460). Biochemically responsive disease was associated with higher frequency of hypothyroidism during treatment (p .047). Conclusion: Our preliminary results illustrate the spectrum of response to ATD and predictors of biochemically persistent disease. We aim to expand this analysis utilizing a large database. As use of ATD increases, clinicians and patients can apply this data to estimate response to therapy, and identify patients that may require more aggressive therapy, thereby tailoring management plans.

An update on serum biomarkers to assess axial spondyloarthritis and to guide treatment decision

Axial spondyloarthritis (axSpA) is a group of debilitating, chronic, rheumatic conditions characterized by inflammation and new bone formation, mainly involving the spine and the sacroiliac joints. The lack of biomarkers in axSpA is well known. Despite significant treatment advances in recent years thanks to the introduction of drugs with a new mode of action, such as new biologic and targeted synthetic disease-modifying antirheumatic drugs, no relevant improvement in the identification of disease biomarkers has been achieved. Common parameters, such as erythrocyte sedimentation rate and C-reactive protein, which are routinely used to measure systemic inflammation, are the sole markers available to date and are not adequate to assess disease activity in all patients. The aim of this study is to review the most promising serum biomarkers that may help treatment decision in axSpA via a proper assessment of disease activity and identification of negative prognostic factors.