Cognitive functions in idiopathic intracranial hypertension.

OBJECTIVE: Cognitive problems in idiopathic intracranial hypertension (IIH) is generally overlooked in the presence of disabling headache and threat to visual function. The aim of this study was to search for cognitive deficits in patients with IIH using neuropsychologic tests in addition to P300 potential recordings to assess cognition related brain activity. METHODS: Fifty IIH patients were examined using Montreal Cognitive Assessment Test, Stroop Test and Visual Aural Digit Span Test to measure different domains of cognition at the time of diagnosis. P300 potentials were recorded by using an oddball paradigm. Hospital Anxiety and Depression Scale was used to determine anxiety and depression. Quality of life (QoL) was assessed by SF-36. The results were compared with fifty healthy controls with matching age, gender and body mass index. RESULTS: Neuropsychologic tests revealed wide cognitive impairment including attention, working memory, executive function, naming, language, delayed recall and orientation in IIH patients. In addition, quality of life was affected in the sub-parameters of general health perceptions, emotional role functioning, vitality, mental health and bodily pain. P300 potential latencies were long and the amplitudes were reduced indicating deficits in attention and working memory. Anxiety scores were high, and health-related QoL was low mainly involving vitality, emotional and mental health. Cognitive dysfunction was not correlated with the levels of anxiety and the correlation with headache severity was mild. CONCLUSION: A multidomain cognitive decline mainly involving attention and working memory was recorded in IIH patients. It was not correlated with anxiety and only a mild correlation with headache severity was present which may indicate a casual relationship between raised intracranial pressure and cognitive deficits. Screening is important as neuropsychological rehabilitation might be relevant in these patients.

Calcitonin gene-related peptide (CGRP) levels in peripheral blood in patients with idiopathic intracranial hypertension and migraine.

BACKGROUND: Calcitonin gene-related peptide (CGRP) plays a dominant role in migraine. This prospective study was designed to investigate CGRP levels in patients with idiopathic intracranial hypertension (IIH) and compare the results of migraine patients and healthy controls (HC). As a second objective, CGRP levels obtained from IIH patients defining sustained headache after the resolution of papilledema were compared with those not defining post-IIH headache. METHODS: Thirty-six patients with IIH, 36 with episodic migraine (EM), 18 with chronic migraine (CM), and 36 HC were included in the study. CGRP levels were studied from blood samples obtained from the antecubital vein by using a commercial ELISA kit. RESULTS: Serum CGRP levels of the patient groups were significantly higher than the HC (p < 0.001). As compared with controls, both CM (p Adj<0.001) and IIH (p Adj=0.039) had significantly increased levels of CGRP. Levels recorded from EM patients did not differ from the HC (p Adj=0.661). In 16 IIH patients, persistent headache was reported after the normalization of intracranial pressure (ICP). Twenty patients did not report post-IIH headaches. Comparison of serum CGRP levels of these two groups revealed significantly higher CGRP levels in patients with sustained headaches obtained from blood samples both at the initial and control visit (p Adj <0.001). CONCLUSIONS: CGRP levels of the patient groups were higher than the HC. High levels recorded in patients with IIH indicates the role of CGRP in IIH related headache and even higher levels in patients with sustained headache after normalization of ICP strengthens this finding.

Printability for additive manufacturing with machine learning: Hybrid intelligent Gaussian process surrogate-based neural network model for Co-Cr alloy.

AM has revolutionized the manufacturing industry, involving several operating parameters that may affect the properties of the final manufactured part. In AM, LPBF has proved its reliability in producing dense components; however, process development for every material necessitates extensive testing. Even the tiniest change can negate all the data for the same material. It is vital to have a P-P correlation that can train itself following a change in powder or machine to achieve defects-free parts and optimal properties. These goals cannot be met alone by multi-physics. One of the ways to address this issue is to apply ML, but it requires a huge data set for training and testing purposes. A framework has been developed for Co-Cr S-S curves to resolve this issue. Twenty-two experimental S-S curves have been generated to produce YS, TS, and EL data points. In combination with DNN, these data points have been applied to the validated and tested GPS-surrogate model to develop a smart processing window to achieve desired YS, TS, and EL. LP, LSS, HD, and PLT have been selected during the whole framework as inputs, while YS, TS, and EL have been classified as outputs. The output of the smart window was verified experimentally. It is found that the highest YS (1110.91 MPa) is attained using LP = 180 W, LSS = 600 mm/s and HD = 70 µm, while least YS (645.05 MPa) is identified using LP = 160 W, LSS = 900 mm/s and HD = 70 µm. For TS, the maximum (165.91 MPa) and minimum (689.73 MPa) values have been achieved using LP = 180 W, LSS = 900 mm/s and HD = 70 µm, and LP = 180 W, LSS = 1000 mm/s and HD = 70 µm, respectively. In the case of EL, LP = 180 W, LSS = 700 mm/s and HD = 70 µm, and LP = 180 W, LSS = 600 mm/s and HD = 70 µm, resulted 23.04% and 0.789% EL, respectively. Using CC, LP and HD did not significantly affect the TS, YS, and EL, while a negative relationship has been found for LSS with TS, YS, and EL. The smart processing window showed that the YS and TS could be achieved at low-high LP and low LSS at the cost of EL. This study provides a technique for framework development in the case of P-P relation based on the provided inputs and the corresponding outputs, leading toward process smartification.

Assessment of effectiveness of anakinra and canakinumab in patients with colchicine-resistant/unresponsive familial Mediterranean fever.

INTRODUCTION: Familial Mediterranean fever (FMF) is a hereditary auto-inflammatory disease characterized by recurrent fever and serosal inflammation. Anti-interleukin-1 (Anti-IL-1) treatments are recommended in colchicine resistant and/or intolerant FMF patients. This study aims to evaluate the efficacy of anakinra and canakinumab in FMF patients that are resistant/intolareted to colchicine or complicated with amyloidosis. METHODS: Between January 2014 and March 2019, 65 patients following-up at Sivas Cumhuriyet University (Medical Faculty Rheumatology-Internal Medicine Department) who were diagnosed with FMF according to the criteria of Tel-Hashomer were included in the study. The laboratory values and clinical features of patients and disease activities were recorded at least every 3 months, and these data were analyzed. RESULTS: Forty-one (63.1%) patients used anakinra (100 mg/day) and 24 (36.9%) patients used canakinumab (150 mg/8 week). The median duration of anti-IL-1 agents use was 7 months (range, 3-30). Fifteen (23.1%) cases were complicated with amyloidosis. Seven (10.8%) patients had renal transplantation. Overall, the FMF 50 score response was 96.9%. In the group that had a glomerular filtration rate (GFR) ≥ 60 ml/min/m2, the median proteinuria decreased from 2390 mg/day (range, 1400-7200) to 890 mg/day (range, 120-2750) (p = 0.008). No serious infections were detected, except in one patient. CONCLUSIONS: Anti-IL-1 agents are effective and safe in the treatment of FMF patients. These agents are particularly effective at reducing proteinuria in patients with GFR ≥ 60 ml/min/m2, but less effective in cases with FMF associated with arthritis and sacroiliitis. Large and long follow-up studies are now needed to establish the long-term effects of these treatments.

Osteoprotegrin interacts with biomarkers and cytokines that have roles in osteoporosis, skin fibrosis, and vasculopathy in systemic sclerosis: A potential multifaceted relationship between OPG/RANKL/TRAIL and Wnt inhibitors.

Objectives: We explored the interactions of osteoprotegerin (OPG) with biomarkers of bone turnover and cytokines, including soluble receptor activator for nuclear factor kappa beta ligand (sRANKL), tumor necrosis factor-related apoptosis-induced ligand (TRAIL), and Wnt inhibitors in osteoporosis, vasculopathy and fibrosis related to systemic sclerosis (SSc). Methods: The study included 46 SSc patients and 30 healthy controls. Skin thickness, pulmonary fibrosis and/or hypertension, digital ulcers, and calcinosis cutis of SSc patients were assessed. We determined bone mineral density (BMD), and OPG, sRANKL, TRAIL, secreted frizzled-related protein 1 (sFRP-1), Dickkopf-related protein 1 (DKK-1), sclerostin in the serum of both patients and controls. Results: OPG, sclerostin, and sFRP-1 levels were similar between patients and controls (P > 0.05). Femoral neck and lumbar spine BMD and vitamin D levels were lower, and the OC, NTX, sRANKL, DKK1 and TRAIL levels were significantly higher, in patients than in controls (p < 0.05). In subgroup analysis, patients with higher modified Rodnan skin score (mRodnan) had higher DKK-1, sclerostin, and TRAIL levels (p < 0.05); those with diffuse SSc subtype had lower BMD values than those with limited SSc (p < 0.05). Skin and pulmonary fibrosis linked negatively with BMD measures. Conclusion: we showed that sRANKL levels were higher and correlated with bone turnover markers. It may be related to osteoporosis in SSc. The OPG level was unaltered in SSc patients. Higher TRAIL levels associated with skin thickness may indicate vascular dysfunction or injury. Higher DKK-1 and sclerostin levels may be related to a reactive increase in cells and be prominently linked to fibrosis in SSc.

Bilateral orbital pseudotumor in a patient with Takayasu arteritis: a case report and review of the literature.

Inflammatory orbital pseudotumor is often associated with rheumatologic disorders. It has been reported commonly with ANCA-associated vasculitides, especially granulomatosis with polyangiitis (Wegener's granulomatosis). There are also a few cases of large vessel vasculitis such as giant cell arteritis and Behcet's disease. Here, we report a patient with undiagnosed Takayasu arteritis presenting with proptosis and diplopia, with later diagnosis of an inflammatory pseudotumor of the orbit. In this case, we believe extensive involvement of blood vessels, including bilateral pulmonary artery stenosis, and elevated inflammatory markers that show disease activity may be related to pseudotumor formation in Takayasu arteritis. Since this is an unusual and unreported presentation of the disease, better estimation of a causal relationship may be possible in the future with further information. In conclusion, although uncommon, this case highlights that orbital pseudotumor may be an important finding in Takayasu arteritis. For early diagnosis, better treatment, and good prognosis, it should be considered in patients presenting with ocular symptoms similar to the other vasculitides.