Encephalopathy is a term for any diffuse disease of brain that alters brain function or structure with very different etiologies and prognoses. There are some reports that steroids have good effects on some encephalopathies with nonvasculitic autoimmune causes. We describe a 63-year-old woman with a 3-month history of progressive gait disturbance, cognitive decline and increasing confusion in whom steroid treatment resulted in dramatic clinical recovery.
14-3-3 Proteins ; Brain ; Female ; Gait ; Humans ; Middle Aged ; Prognosis ; Steroids

Background: This study aims to identify the incidence and risk factors of periprosthetic infections following endoprosthetic reconstruction of femoral metastatic bone disease (MBD). In this population with MBD, the marked impact of infection on the patient’s systemic treatment highlights the importance of understanding both the incidence and associated risk factors. Methods: This retrospective cohort study included a total of 140 patients who underwent endoprosthetic reconstruction for femoral MBD at a tertiary referral hospital in South Korea between 2009 and 2019. Infection-free survival was estimated using the Kaplan-Meier method, and Cox proportional hazards model analyses were performed to evaluate the risk factors associated with periprosthetic infection. Results: The incidence of periprosthetic infection in patients who underwent endoprosthetic reconstruction for femoral MBD was 9% (12 out of 140 patients). Risk factors for periprosthetic infection were hepatocellular carcinoma (HCC) as the primary tumor (hazard ratio [HR], 6.08; 95% CI, 1.63–22.6; p = 0.007) and low preoperative absolute neutrophil count (HR, 6.99; 95% CI, 1.79–27.4; p = 0.005). Conclusions Patients with femoral MBD had a 9% risk of developing a periprosthetic infection. Given their limited life expectancy, this translated to a substantial rate of 58.9 infections per 1,000 person-joint-years. Possible risk factors for periprosthetic infection were low preoperative absolute neutrophil count and HCC as the primary tumor. The high incidence of periprosthetic infection and its associated risk factors should be considered in patients undergoing endoprosthetic reconstruction for femoral MBD.

Background: This study aims to identify the incidence and risk factors of periprosthetic infections following endoprosthetic reconstruction of femoral metastatic bone disease (MBD). In this population with MBD, the marked impact of infection on the patient’s systemic treatment highlights the importance of understanding both the incidence and associated risk factors. Methods: This retrospective cohort study included a total of 140 patients who underwent endoprosthetic reconstruction for femoral MBD at a tertiary referral hospital in South Korea between 2009 and 2019. Infection-free survival was estimated using the Kaplan-Meier method, and Cox proportional hazards model analyses were performed to evaluate the risk factors associated with periprosthetic infection. Results: The incidence of periprosthetic infection in patients who underwent endoprosthetic reconstruction for femoral MBD was 9% (12 out of 140 patients). Risk factors for periprosthetic infection were hepatocellular carcinoma (HCC) as the primary tumor (hazard ratio [HR], 6.08; 95% CI, 1.63–22.6; p = 0.007) and low preoperative absolute neutrophil count (HR, 6.99; 95% CI, 1.79–27.4; p = 0.005). Conclusions Patients with femoral MBD had a 9% risk of developing a periprosthetic infection. Given their limited life expectancy, this translated to a substantial rate of 58.9 infections per 1,000 person-joint-years. Possible risk factors for periprosthetic infection were low preoperative absolute neutrophil count and HCC as the primary tumor. The high incidence of periprosthetic infection and its associated risk factors should be considered in patients undergoing endoprosthetic reconstruction for femoral MBD.

Background: This study aims to identify the incidence and risk factors of periprosthetic infections following endoprosthetic reconstruction of femoral metastatic bone disease (MBD). In this population with MBD, the marked impact of infection on the patient’s systemic treatment highlights the importance of understanding both the incidence and associated risk factors. Methods: This retrospective cohort study included a total of 140 patients who underwent endoprosthetic reconstruction for femoral MBD at a tertiary referral hospital in South Korea between 2009 and 2019. Infection-free survival was estimated using the Kaplan-Meier method, and Cox proportional hazards model analyses were performed to evaluate the risk factors associated with periprosthetic infection. Results: The incidence of periprosthetic infection in patients who underwent endoprosthetic reconstruction for femoral MBD was 9% (12 out of 140 patients). Risk factors for periprosthetic infection were hepatocellular carcinoma (HCC) as the primary tumor (hazard ratio [HR], 6.08; 95% CI, 1.63–22.6; p = 0.007) and low preoperative absolute neutrophil count (HR, 6.99; 95% CI, 1.79–27.4; p = 0.005). Conclusions Patients with femoral MBD had a 9% risk of developing a periprosthetic infection. Given their limited life expectancy, this translated to a substantial rate of 58.9 infections per 1,000 person-joint-years. Possible risk factors for periprosthetic infection were low preoperative absolute neutrophil count and HCC as the primary tumor. The high incidence of periprosthetic infection and its associated risk factors should be considered in patients undergoing endoprosthetic reconstruction for femoral MBD.

Background: This study aims to identify the incidence and risk factors of periprosthetic infections following endoprosthetic reconstruction of femoral metastatic bone disease (MBD). In this population with MBD, the marked impact of infection on the patient’s systemic treatment highlights the importance of understanding both the incidence and associated risk factors. Methods: This retrospective cohort study included a total of 140 patients who underwent endoprosthetic reconstruction for femoral MBD at a tertiary referral hospital in South Korea between 2009 and 2019. Infection-free survival was estimated using the Kaplan-Meier method, and Cox proportional hazards model analyses were performed to evaluate the risk factors associated with periprosthetic infection. Results: The incidence of periprosthetic infection in patients who underwent endoprosthetic reconstruction for femoral MBD was 9% (12 out of 140 patients). Risk factors for periprosthetic infection were hepatocellular carcinoma (HCC) as the primary tumor (hazard ratio [HR], 6.08; 95% CI, 1.63–22.6; p = 0.007) and low preoperative absolute neutrophil count (HR, 6.99; 95% CI, 1.79–27.4; p = 0.005). Conclusions Patients with femoral MBD had a 9% risk of developing a periprosthetic infection. Given their limited life expectancy, this translated to a substantial rate of 58.9 infections per 1,000 person-joint-years. Possible risk factors for periprosthetic infection were low preoperative absolute neutrophil count and HCC as the primary tumor. The high incidence of periprosthetic infection and its associated risk factors should be considered in patients undergoing endoprosthetic reconstruction for femoral MBD.

We report herein a patient with aphasic status epilepticus (ASE) who underwent serial brain magnetic resonance imaging (MRI) including diffusion-weighted imaging (DWI), perfusion imaging (PI), and magnetic resonance angiography (MRA) in the peri-ictal phase. The initial DWI revealed a high signal intensity in the left medial temporal area and PI demonstrated hyperperfusion in the left temporoparietal area. MRA revealed an increased flow signal in the ipsilateral intracranial arteries. PI signal changes had normalized by the follow-up MRI. The DWI, PI, MRA, and clinical manifestation of ASE are described herein.
Arteries ; Brain ; Follow-Up Studies ; Humans ; Magnetic Resonance Angiography ; Magnetic Resonance Imaging ; Perfusion ; Perfusion Imaging ; Status Epilepticus

No abstract available.
Disulfiram*

BACKGROUND: Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited microangiopathy that is caused by mutations in the Notch3 gene. Typical findings from magnetic resonance imaging (MRI) include multiple subcortical lacunae, extensive white-matter change, and multiple cerebral microbleeds (CMBs). CMBs are indicative of bleeding-prone microangiopathy. The rate of intracerebral hemorrhage in CADASIL is higher in Asian patients than in Caucasian patients. However, CMBs have not been thoroughly evaluated in Asian patients. We performed a detailed analysis of the frequency and distribution pattern of CMBs and assessed whether vascular risk factors exert an independent effect on CMBs in Asian CADASIL patients. METHODS: The study population comprised 60 patients who underwent brain MRI, including T2*-weighted gradient-echo sequences. Demographic factors, vascular risk factors, and MRI findings were compared between CADASIL patients with and without CMBs. The impact of vascular risk factors on CMBs, lacunae, and white-matter hyperintensities (WMHs) was assessed by logistic regression analysis. RESULTS: CMBs, which were detected in 34 (56.7%) patients, exhibited a significant predilection for the thalamus (46.7%), subcortical-cortical region (35.0%), and basal ganglia (31.7%). Hypertension, lacunae, and white-matter lesions were more common in patients with CMBs. Hypertension was an independent risk factor for CMBs, lacunae, and WMHs in patients with CADASIL. CONCLUSIONS: This study found that CMBs tended to occur in hypertensive patients with CADASIL. Further studies should focus on elucidating the association between reduced blood pressure and the number of CMBs.
Asian Continental Ancestry Group ; Basal Ganglia ; Blood Pressure ; Brain ; CADASIL* ; Cerebral Hemorrhage ; Demography ; Humans ; Hypertension* ; Leukoencephalopathies ; Logistic Models ; Magnetic Resonance Imaging ; Risk Factors ; Thalamus

BACKGROUND AND PURPOSE: Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited microangiopathy caused by mutations in the Notch3 gene. Although previous studies have shown an association between lacunar infarction and cognitive impairment, the relationship between MRI parameters and cognition remains unclear. In this study we investigated the influence of MRI parameters on cognitive impairment in CADASIL. METHODS: We applied a prospective protocol to 40 patients. MRI analysis included the normalized volume of white-matter hyperintensities (nWMHs), number of lacunes, and number of cerebral microbleeds. Cognition was assessed with the aid of psychometric tests [Mini-Mental State Examination (MMSE), Alzheimer's Disease Assessment Scale-cognition (ADAS-cog), Trail-Making Test, and Stroop interference (Stroop IF)]. RESULTS: A multivariate regression analysis revealed that the total number of lacunes influenced the performance in the MMSE, ADAS-cog, and Stroop IF, while nWMHs had a strong univariate association with ADAS-cog and Stroop IF scores. However, this association disappeared in the multivariate analysis. CONCLUSIONS: These findings demonstrate that the number of lacunes is the main predictive factor of cognitive impairment in CADASIL.
Alzheimer Disease ; CADASIL ; Cognition ; Humans ; Leukoencephalopathies ; Prospective Studies ; Psychometrics ; Stroke, Lacunar

Although paraganglioma (PGL), an extra-adrenal retroperitoneal pheochromocytoma (PHEO), is a rare catecholamine-secreting neuroendocrine tumor, it can cause severe hypertensive crisis during anesthesia or surgery if undiagnosed preoperatively. Extraluminal perigastric masses may be presumed to be gastrointestinal stromal tumors (GISTs) or soft tissue sarcomas even when histologic confirmation is not possible. Therefore, without a histologic diagnosis or symptoms of excessive catecholamine secretion, PGL may be mistaken for GIST. We report a case of preoperatively undiagnosed PGL which caused hypertensive crisis during anesthesia for retroperitoneal mass excision.
Anesthesia ; Gastrointestinal Stromal Tumors ; Neuroendocrine Tumors ; Paraganglioma ; Pheochromocytoma ; Sarcoma ; Stromal Cells