(1) Background: West syndrome is a severe, refractory, epileptic syndrome that usually appears in infancy or early childhood. ACTH is one of the more effective drugs for treating this condition. (2) Aim of the study and methods: The objective of our study was to examine short-term efficacy (during treatment schedule) and long-term outcome of intramuscular 0.02 mg/kg/day ACTH (tetracosactide) depot, used concomitantly with other antiepileptic drugs (AEDs) in patients with infantile spasms who did not achieve seizure cessation or relapse when taking only the AEDs. The drug efficacy was evaluated in retrospective and prospective analyses of 50 patients diagnosed with infantile spasms. (3) Results: Complete cessation of spasms was achieved in 42 cases (84%). EEG improvement was seen in 41 (82%) patients who responded to ACTH therapy. Information on the clinical course of 28 patients was obtained duringlong-term follow-up. In 17 (60.7%) cases, seizures were still present. Normal or near-normal development was observed in 11 out of 28 children (39%). ACTH used concomitantly with other AEDis a highly effective treatment with acceptable side effects. (4) Conclusion: Randomized controlled clinical trialswith long-term follow-up are needed to compare the effectiveness of ACTH in polytherapy and monotherapy. Dyskinesias as a potential side effect observed in our study group should be investigated in the following studies.
Background/Objectives: Chronic kidney disease (CKD) is identified as a risk factor for the occurrence of ischemic stroke. There is substantial evidence that CKD is linked to a worse prognosis and higher mortality rates in stroke patients. This study aimed to evaluate the characteristics and factors affecting favorable outcomes and mortality in patients treated using mechanical thrombectomy (MT) for ischemic stroke, with particular emphasis on patients suffering from CKD. Methods: The retrospective study included an analysis of data from 723 patients (139; 19.4% had CKD) with ischemic stroke treated with MT between March 2019 and July 2022. Results: Patients with CKD were significantly older (median age 76.5 vs. 65.65, p < 0.001) and more often female (59.7% vs. 42.6%, p < 0.001). CKD decreased the likelihood of achieving a favorable outcome (0–2 points in modified Rankin scale; OR: 0.56, CI95%: 0.38–0.81) and increased mortality (OR: 2.59, CI95%: 1.74–3.84) on the 90th day after stroke. In addition, CKD was associated with intracranial hemorrhage (ICH) in patients who underwent posterior circulation MT (13.85% vs. 50%, p = 0.022). In patients with CKD, inter alia, higher levels of C-reactive protein (OR: 0.94, CI95%: 0.92–0.99) reduced the chance of a favorable outcome. In addition, the occurrence of ICH in patients with CKD increased mortality on the 90th day after stroke (OR: 4.18, CI95%: 1.56–11.21), which was almost twice as high as in patients without CKD (OR: 2.29, CI95%: 1.54–3.40). Conclusions: Patients suffering from CKD had a lower probability of achieving a favorable outcome and had increased mortality following MT for ischemic stroke. It is crucial to understand the variations between patients with unimpaired and impaired renal function, as this could aid in predicting the outcomes of this method.
Introduction In order to better understand comorbidity rates and the associated risk of death in patients with glaucoma we retrospective analyzed two groups of subjects aged 50 years and above residing in Lombardy Region (Northern Italy) following them from January 1, 2017 to February 1, 2020 (just before the COVID-19 pandemic started in Italy). The two groups were all subjects with incident glaucoma in 2017 and a 3:1 random sample stratified by age and sex of subjects without glaucoma. Main outcome was overall survival. Other outcomes were incidence of cardiovascular diseases (heart attack, stroke, and peripheral arterial disease). Methods All data were taken from Lombardy Region administrative database and therefore the presence of glaucoma was ascertained using antiglaucoma drug prescriptions, being hospitalized or undergoing an intervention for glaucoma, and having a glaucoma exemption for healthcare co-payments. Results The study identified 14,138 incident cases of glaucoma and selected 42,414 subjects without glaucoma. The number of deaths was higher among glaucoma subjects (11.6%) compared to those without glaucoma (10.5%). The death hazard ratio (HR) for glaucoma subjects compared to controls was 1.07 (95% CI 1.01–1.14, p = 0.015) in the adjusted model. Similarly incidence of stroke was higher in glaucoma compared to non-glaucoma subjects (HR 1.10, 95% CI 1.01–1.20), while the incidence of heart attack and peripheral arterial disease during the follow-up period was similar in the two groups. HRs varied by age classes for death and peripheral arterial disease. Individuals with glaucoma showed higher comorbidity rates compared to those without glaucoma, particularly in diabetes mellitus (16.7% vs. 11.0%) and hypertension (62.2% vs. 58.1%). Conclusions Our results show an increased risk of mortality in subjects with glaucoma compared to those without glaucoma, with age being a significant factor influencing outcomes. These findings suggest the importance of monitoring and managing comorbidities in individuals with newly incident glaucoma to potentially improve their overall health outcomes and quality of life.
Encephalocraniocutaneous lipomatosis (ECCL; Haberland syndrome, #613001) is an extremely rare congenital disorder that is manifested by the involvement of the skin, eyes and central nervous system (CNS). We report two cases of children with ECCL diagnosis. First was an 8-year-old girl who presented with symptomatic epilepsy, cerebral palsy and developmental delay. In 2020, she was admitted to the hospital due to the exacerbation of paresis and intensified prolonged epileptic seizures, provoked by infection of the middle ear. Diagnostic imaging revealed radiological changes suggestive of ECCL, providing a reason for the diagnosis, despite the lack of skin and eye anomalies. The second child, a 14-year-old girl, was consulted for subtle clinical signs and epilepsy suspicion. Diagnostic imaging findings were similar, though less pronounced. Based on neuroradiological abnormalities typical for Haberland syndrome, the authors discuss possible ECCL diagnosis.
1 January 2024 in Postępy Higieny I Medycyny Doświadczalnej
Introduction: Ocular ischemic syndrome (OIS) is a rare condition associated with ocular hypoperfusion caused by carotid artery occlusive disease. This article describes the case of a patient who was admitted to the Department of Ophthalmology at UCK in Katowice, Poland, due to significant loss of visual acuity and pain in the right eye. Materials and Methods: Retrosopective case report. Results: Patient showed up in the ophthalmological emergency department complaining of significant loss of visual acuity and pain in the right eye persisting for 2 weeks. The patient had a history of type II diabetes mellitus, hypertension, status post ACS and PCI, and long-term nicotinism. Examination of the right eye revealed ocular irritation, mixed conjunctival injection, hyphema in the anterior chamber, iris neovascularization, and discrete central retinal fading. Additional examinations were performed, including an angioCT, which showed a long-standing obstruction of the right internal carotid artery, a ballooning thrombus in the lumen of the brachiocephalic trunk, and suspected critical stenosis of the left vertebral artery. A neurologist and vascular surgeon were consulted, and treatment was instituted to limit further complications from the underlying disease. The patient remains under close supervision of the vascular surgeon and neurologist. Conslusions: OIS is a rare but potentially blinding condition and should be considered in the differential diagnosis of transient visual acuity reduction or vision loss.