Severity of Intracranial Large Artery Disease Correlates With Cerebral Small Vessel Disease.

BACKGROUND: Small vessel disease (SVD) shares common vascular risk factors with large artery disease (LAD). However, little is known about the relationship between intracranial artery stenosis and SVD burden. PURPOSE: To investigate whether SVD burden correlates with severity of intracranial LAD. STUDY TYPE: Retrospective. POPULATION: Five hundred and sixteen patients with LAD of arterial circulation were enrolled from one hospital, including 384 males (59 ± 11 years) and 132 females (60 ± 12 years). FIELD STRENGTH/SEQUENCE: 3 T. T1 -weighted fast spin echo (T1 W FSE), T2 W FSE, T2 fluid attenuated inversion recovery, diffusion-weighted imaging, susceptibility-weight imaging, and time-of-flight magnetic resonance angiography. ASSESSMENT: The LAD was divided into mild stenosis (<30%), moderate stenosis (30%-69%), and severe stenosis (≥70%). The Standard for Reporting Vascular Changes on Neuroimaging criteria was used to rate the SVD burden according to the level of white matter hyperintensity (WMH), perivascular space (PVS), cerebral microbleed (CMB), and lacunes. STATISTICAL TESTS: Lilliefors test, ANOVA, chi-squared test, Mann-Whitney U test, Wilcoxon signed rank test, Bonferroni test, Spearman's correlation, logistic regression, and Cohen's kappa test. RESULTS: The grade scores for centrum semiovale PVS (CS-PVS) were positively correlated with the degree of stenosis (R = 0.413), whereas the presence of severe basal ganglia PVS (BG-PVS) was associated with CMB (R = 0.508), lacunes (R = 0.365), and severe WMH (R = 0.478). In multivariate analysis, severe CS-PVS (adjusted odds ratio [aOR], 3.1; 95% confidence interval [CI], 1.9-4.8) and lacunes (aOR, 2.1; 95% CI, 1.3-3.4) were associated with severe stenosis of LAD. In addition, CS-PVS was related to severe stenosis in a dose-dependent manner: when CS-PVS score was 3 and 4, the aORs of severe stenosis were 1.9 and 7.7, respectively. DATA CONCLUSION: The severity of LAD in anterior circulation is associated with SVD burden, which suggests that different SVD burden may be used for risk stratification in LAD. EVIDENCE LEVEL: 3 TECHNICAL EFFICACY: Stage 3.

FLAIR vessel hyperintensities predict functional outcomes in patients with acute ischemic stroke treated with medical therapy.

OBJECTIVES: The prognostic value of fluid-attenuated inversion recovery vessel hyperintensity (FVH) remains controversial in acute ischemic stroke (AIS). The objective was to investigate whether the presence of FVH could predict long-term functional outcomes in patients with AIS receiving medical therapy. METHODS: Consecutive AIS patients with anterior circulation large vessel stenosis (LVS) in multiple centers between January 2019 and December 2020 were studied. Presence of FVH was identified and evaluated as FVH (+). Quantification of FVH was performed using an FVH-Alberta Stroke Program Early CT Score (ASPECTS) system and divided into grades: FVH-ASPECTS of 0 = grade 0; 1-2 = grade 1; 3-7 = grade 2. Poor functional outcome was defined as modified Rankin scale > 2 at 3 months. RESULTS: Overall, 175 patients were analyzed (age, 64.31 ± 13.47 years; men, 65.1%), and 78.9% patients presented with FVH. Larger infarct volume (19.90 mL vs. 5.50 mL, p < 0.001), higher rates of FVH (+) (92.0% vs. 65.9%, p < 0.001), and higher FVH grades (grade 2, 34.5% vs. 10.2%, p < 0.001) were more prone to be observed in patients with poor functional outcomes. FVH (+) with infarct volume larger than 6.265 mL (adjusted odds ratio [aOR] 6.03, 95% confidence interval [CI] 1.82-19.98) and FVH grade (grade 1, aOR 3.07, 95% CI 1.12-8.43; grade 2, aOR 5.80, 95% CI 1.59-21.11) were independently associated with poor functional outcomes. CONCLUSION: FVH (+) combined with large infarct volume and high FVH grade can predict poor long-term functional outcomes in patients with LVS who receive medical therapy. KEY POINTS: • FVH is expected to be a contrast agent-independent alternative for assessing hemodynamic status in the acute stage of stroke. • FVH (+) and high FVH grade, quantified by FVH-ASPECTS rating system and grades, are associated with large infarct volume. • The combination of FVH and DWI-based infarct volume has independent predictive value for long-term functional outcomes in AIS patients with large artery stenosis treated with medical therapy.

Neurobrucellosis.

PURPOSE: Neurobrucellosis (NB) is a rare complication of brucellosis. NB presents with avariety of clinical manifestations, and the symptoms are always atypical. Our aim was to analyze the demographic characteristics, clinical manifestations, laboratory findings, imaging findings, treatments and outcomes of patients with NB. MATERIAL AND METHOD: We retrospectively reviewed the data from 17 patients with NB hospitalized at the Chinese People's Liberation Army General Hospital between 1 January 2005 and 31 October 2016. RESULTS: The following symptoms were recorded: 10/17 (59%) patients had fever, and 9/17 (53%) patients had a disorder affecting urination and defecation. Involvement of the cranial nerves was documented in 12/17 (71%) patients. The positivity rates of the tests were as follows: serum standard tube agglutination (STA), 15/17 (88.2%); cerebrospinal fluid STA, 10/17 (59%). The radiologic findings were categorized into four types: normal, white matter changes, vascular insult and inflammatory changes. Patients were treated with different combinations of rifampicin, doxycycline, ceftriaxone sodium and sulphamethoxazole for a total of six months. Two (12%) patients deteriorated, and two (12%) patients were lost to follow-up. The remaining patients (76%) were cured, but sequelae occurred in six patients. CONCLUSIONS: NB should be kept in mind in patients with autonomic dysfunction, especially disorders of urination and defecation. Hearing loss due to vestibulocochlear nerve injury seems to be typical for NB. The high incidence of sequelae may be related to a long disease course and the involvement of the central nervous system. Early detection, diagnosis and treatment could decrease mortality and sequelae.

D-dimers (DD) in CVST.

OBJECTIVE: We were interested in further confirming whether D-dimers (DD) are indeed elevated in cerebral venous sinus thrombosis (CVST) as reported in those studies. METHODS: CVST patients who had a plasma D-dimer test (139 cases) were included and divided into two groups: elevated D-dimer group (EDG) (>0.5 µg/mL; 65 cases) and normal D-dimer group (NDG) (≤0.5 µg/mL; 74 cases). The two groups were compared in terms of demographic data, clinical manifestation, laboratory and imaging data, using inferential statistical methods. RESULTS: The chi-squared and Fisher exact test showed that, compared to the NDG (74 cases), patients with elevated D-dimer levels were more likely to have a shorter symptom duration (SD) (30 ± 83.9 versus 90 ± 58.9 d, p = 0.003), more risk factors (75.4% versus 52.7%, p = 0.006), higher multiple venous sinus involvement (75.4% versus 59.5%, p = 0.037), increased fibrinogen (43.1% versus 18.9%, p = 0.037) and higher levels of blood glucose (18.3% versus 11%, p = 0.037). According to correlation analyses, D-dimer levels were positively correlated with number of venous sinuses involvement (NVS) (r = 0.321, p = 0.009) in the EDG. Multivariate logistic regression analysis showed that SD (OR, 0.025; 95% CI, 1.324-6.043; p = 0.000), NVS (OR, 1.573; 95% CI, 1.15-2.151; p = 0.005) and risk factors (OR, 3.321; 95% CI, 1.451-7.564; p = 0.004) were significantly different between the two groups. CONCLUSION: D-dimer is elevated in patients with acute/subacute CVST.

Conventional Tumor Markers in Cerebralspinal Fluid in Patients with Elevated Serum Tumor Markers and without Central Nervous System Malignant Diseases.

Objective To explore the diffusion pattern of tumor markers (TM) from serum to cerebrospinal fluid (CSF) via the blood-brain barrier in patients with elevated serum tumor markers (TM).Methods Inpatients receiving lumbar puncture during hospitalization in our center from January 1, 2013 to December 31, 2015 were divided into study group (n=181) and control group (n=251). The study group consisted of patients with elevated serum TMs but without malignant central nervous system diseases. The control group consisted of patients with normal serum TM levels and without malignant diseases. TMs measured in the study group included elevated serum alpha-fetoprotein (AFP) (n=0), carcinoembryonic antigen (CEA) (n=26), carcinomic antigen(CA)125 (n=39), CA15- 3 (n=3),CA19- 9 (n=19), CA724 (n=47), CYFRA21- 1 (n=49), and SCC (n=17).Levels of TMs in the CSF of study group was compared with that of control group.Results Median CEA (U=0.00,P=0.00),CA19- 9 (U=0.00,P=0.00),CA15- 3 (U=0.00,P=0.04),SCC (U=0.00,P=0.00),CA125 (U=0.00,P=0.00),CA72- 4 (U=3.00,P=0.00)),and CYFRA21- 1 (U=0.00,P=0.00) in CSF were significantly lower than the corresponding serum TM levels in the study group.There was no significant difference between study group and control group for the CSF level of CEA (U=3091.00,P=0.18),CA19- 9 (U=1897.00,P=0.14), CA15- 3 (U=373.50,P=0.91)and SCC (U=1925.50,P=0.76). CSF CA125 (U=2188.00,P=0.00) and CA724 (U=1279.00,P=0.00) levels in the study group were lower than those in control group. CSF level of CYFRA21- 1 (U=1826.50,P=0.00) in study group was higher than that in control group;however, it was still lower than the upper limit of reference value. Conclusion In patients with elevated serum CEA, CA19- 9, CA15- 3, SCC, CA125, and CA72- 4 levels, transblood-brain-barrier diffusion of TMs from serum to CSF is highly unlikely.

Incidence and risk factors of pleural effusions in patients with POEMS syndrome.

Information regarding the characteristics of pleural effusions in patients with POEMS syndrome is limited. The aim of this study was to describe the incidence and risk factors of pleural effusions in patients with POEMS syndrome and characterize the pleural fluid biochemistry in those patients. A retrospective review of 96 patients with POEMS syndrome was conducted. The patients were divided into groups with and without pleural effusions. The clinical data were obtained from medical charts. Risk factors were studied with univariate and multivariate analysis. The median age at the time of diagnosis of POEMS syndrome was 45.1 years, and the median disease duration was 30.4 months. Pleural effusions were detected in 41 (42.7%) of the 96 patients. Increased serum vascular endothelial growth factor (VEGF), complement component 3 (C3), Lambda light chain, tumour necrosis factor (TNF)-α, interleukin (IL)-6 levels and low albumin as well as cardiac disease were found to be significantly correlated with pleural effusions. By multivariate logistic regression, independent risk factors for pleural effusions in POEMS syndrome were VEGF [odds ratio (OR): 2.46, 95% confidence interval (CI): 1.720-3.414, p = 0.01], TNF-α (OR: 3.64, 95% CI: 1.073-4.338, p = 0.04) and C3 (OR: 3.77, 95% CI: 1.225-3.591, p = 0.02) levels. Pleural effusions are the most common thoracic involvement findings in patients with POEMS syndrome, and all the pleural fluids are exudates. Serum VEGF, TNF-α and C3 levels are identified as important risk factors for presence of pleural effusions in POEMS syndrome.

Conventional T2-Weighted Imaging to Detect High-Grade Stenosis and Occlusion of Internal Carotid Artery, Vertebral Artery, and Basilar Artery.

BACKGROUND: To evaluate the reliability of conventional T2-weighted imaging (WI) for detecting high-grade stenosis and occlusion of cerebral arteries by disappearance of artery flow void signals. METHODS: Our hospital's neuroimaging data on patients with ischemic stroke or transient ischemic attack were collected, including only those from patients who underwent both conventional brain T2WI and three-dimensional time-of flight magnetic resonance angiography (3D-TOF MRA). Flow void signals of conventional axial T2WI were analyzed by 2 young neurologists and compared with 3D-TOF MRA as the gold standard to determine the specificity for detection of high-grade stenosis and occlusion of cerebral arteries. Interobserver agreement was evaluated by calculating kappa (κ) coefficients. RESULTS: Of the 1765 patients included, disappearance of flow void signals was detected in 445 major cerebral arteries in 320 patients on conventional axial T2WI. The specificity of disappearance of flow void signal for the diagnosis of high-grade stenosis and occlusion was 94% for internal carotid arteries, 96.2% for vertebral arteries, and 92% for basilar arteries. K coefficients were greater than .94 for all arteries. CONCLUSIONS: Disappearance of flow void signal on conventional axial T2WI is a reliable indicator of high-grade stenosis or occlusion of major cerebral arteries. Conventional axial T2WI is a useful tool in the diagnosis of cerebral artery steno-occlusive disease.

Monogenic basis of young-onset cryptogenic stroke: a multicenter study.

Background: The prevalence of stroke in young adults is increasing. We investigated the monogenic basis of young adult cryptogenic stroke patients. Methods: This multicenter study enrolled cryptogenic stroke patients under 55 years old, and individuals with nonstroke diseases were included as controls. Targeted next-generation sequencing (NGS) was applied with a custom-designed gene panel that included 551 genes. Rare variants were classified into 2 groups: pathogenic variants and variants of unknown significance. Results: A total of 153 individuals, including 30 (21 males, 70%; mean age 36.1±10.2 years) in the disease group and 123 (59 males, 48.0%; mean age 40.4±13.1 years) in the control group, were recruited. In the disease group, 32 rare variants were identified. Among these individuals, 18 pathogenic variants in 16 patients were detected, with a 53.3% (16/30) diagnostic yield of monogenic causes for cryptogenic stroke. None of these mutations were observed in the control group. Among the mutant genes, the most prevalent were Notch receptor 3 (NOTCH3), protein kinase AMP-activated noncatalytic subunit gamma 2 (PRKAG2), and ryanodine receptor 2 (RYR2). Genes associated with cardiogenic diseases showed the highest mutation frequency (10/18, 55.6%) followed by genes associated with small-vessel diseases (SVDs) and coagulation disorders. None of the patients with mutations had evident abnormalities in the heart or other systems checked by routine tests. For the imaging phenotype-genotype association analysis, infarctions in both the anterior and posterior cerebral circulation were only observed in patients with genes related to cardiogenic disease. Conclusions: In this study, pathogenic variants were identified in nearly half of the young-onset cryptogenic stroke patients, with genes related to cardiogenic diseases being the most frequently mutated. This may have implications for future clinical decision-making, including the development of finer and more sensitive examinations.

Diffusion-Weighted Magnetic Resonance Imaging in the Diagnosis of Cerebral Venous Thrombosis : A Meta-Analysis.

OBJECTIVE: A role of diffusion-weighted imaging (DWI) in the diagnosis of cerebral venous thrombosis (CVT) is not wellunderstood. This study evaluates the effectiveness of DWI in the diagnosis of CVT. METHODS: Literature search was conducted in electronic databases for the identification of studies which reported the outcomes of patients subjected to DWI for CVT diagnosis. Random-effects meta-analyses were performed to achieve overall estimates of important diagnostic efficiency indices including hyperintense signal rate, the sensitivity and specificity of DWI in diagnosing CVT, and the apparent diffusion coefficient (ADC) of DWI signal areas and surrounding tissue. RESULTS: Nineteen studies (443 patients with 856 CVTs; age 40 years [95% confidence interval (CI), 33 to 43]; 28% males [95% CI, 18 to 38]; symptom onset to DWI time 4.6 days [95% CI, 2.3 to 6.9]) were included. Hyperintense signals on DWI were detected in 40% (95% CI, 26 to 55) of the cases. The sensitivity of DWI for detecting CVT was 22% (95% CI, 11 to 34) but specificity was 98% (95% CI, 95 to 100). ADC values were quite heterogenous in DWI signal areas. However, generally the ADC values were lower in DWI signal areas than in surrounding normal areas (mean difference-0.33×10-3 mm2/s [95% CI, -0.44 to -0.23]; p<0.00001). CONCLUSION: DWI has a low sensitivity in detecting CVT and thus has a high risk of missing many CVT cases. However, because of its high specificity, it may have supporting and exploratory roles in CVT diagnosis.

Role of diffusion-weighted imaging in the diagnosis of cerebral venous thrombosis.

OBJECTIVE: To evaluate the hyperintense signal (HIS) performance on diffusion-weighted imaging (DWI) in diagnosing cerebral venous thrombosis (CVT). METHODS: Seventy-eight patients with CVT hospitalized from January 2004 to January 2015 were retrospectively studied alongside 78 controls without intracranial organic diseases. Diagnostic accuracy indices of HIS on DWI or T2-weighted imaging (T2WI) to diagnose CVT at different sites and states were analyzed. RESULTS: The overall sensitivity of HIS on DWI for the diagnosis of CVT was significantly lower than that of HIS on T2WI (34.6% vs. 79.5%). HIS on T2WI was more sensitive than HIS on DWI in detecting thrombosis, especially in the superior sagittal sinus and transverse sinus. HIS on DWI was inversely related to the time between disease onset and imaging. Compared with HIS on T2WI, combining HIS on DWI and T2WI did not increase the sensitivity for detecting CVT. HIS on DWI was not detected in the control group, but HIS on T2WI was detected in 26.3% of control individuals. The specificity of HIS on DWI for CVT was higher than that of HIS on T2WI (97.4% vs. 76.9%). CONCLUSION: HIS on DWI has a lower sensitivity, but a higher specificity, than HIS on T2WI for diagnosing CVT.

[The determination of medical reference values for tumor markers in cerebrospinal fluid].

OBJECTIVE: To determine medical reference values for tumor markers in cerebrospinal fluid. METHODS: Concentrations of CEA, CA125, CA15-3, CA19-9, CA72-4, CYFRA21-1, AFP, NSE, SCC and HCG were determined by means of double-antibody sandwich ELISA in 110 patients excluding primary tumors and meningeal carcinomatosis using Roche E170 modular immunoassay analyzer. RESULTS: The determined medical reference values for tumor biomarkers in cerebrospinal fluid were as follows: CEA<0.573 microg/L, CA125<2.591 U/ml, CA15-3<2.045 U/ml, CA19-9<2.272 U/ml, CA72-4<1.252 U/ml, CYFRA21-1<1.44 ng/ml, AFP<0.968 microg/L, NSE<57.666 ng/ml, SCC<0.5 microg/L, HCG<0.769 U/L. There was no correlation between any tumor marker and age (P>0.05). Concentrations of tumor markers were not affected by gender (P>0.05). CONCLUSION: Medical reference values for CEA, CA125, CA15-3, CA19-9, CA72-4, CYFRA21-1, AFP, NSE, SCC and HCG in cerebrospinal fluid were first determined.

A Clinical Study of Miliary Brain Tuberculomas in China.

Brain tuberculomas can exhibit many different clinical and radiological patterns. However, disseminated or miliary brain tuberculomas are very rare. Miliary brain tuberculomas have specific clinical prognostic implications. Seven patients diagnosed with miliary brain tuberculomas between December 2004 and August 2012 were evaluated retrospectively. Their clinical features, cranial magnetic resonance imaging (MRI) characteristics, treatments, and outcomes were reviewed. The median patient age was 42 years (range, 22-66 years). Six patients presented with fever, 5 with headache, 4 with papilledema, and 3 with diplopia. MRI studies revealed multiple brain lesions. MRI showed 20-50 lesions at the same level. These lesions measured approximately 2-4 mm in diameter and exhibited ring or nodular enhancement after gadolinium injection. All patients began to recover within 2 weeks of initiating antitubercular therapy (ATT). The number of lesions visible on MRI scans was halved within a month, and all lesions had healed without sequelae after 18 months of regular ATT. Miliary brain tuberculoma is a rare form of central nervous system tuberculosis. Some special characteristics of miliary brain tuberculomas are as follows: First, the presence of mild atypical clinical manifestations and almost normal laboratory findings; second, severe radiological features and 20-50 lesions at the same level on MRI scans; and third, a good response to standard ATT. Finally, they are benign; for instance, no patients died in our study. Early diagnosis and treatment can result in full recovery.

[Regulatory T cells in the treatment of autoimmune myositis in mice: efficacy and mechanism].

OBJECTIVE: To investigate effect of CD4(+) CD25(+) Foxp3(+) Tregs in the treatment of autoimmune myositis (EAM) in mice and explore the possible mechanisms. METHODS: Mouse models of EAM were divided randomly into model group and treatment group, and the latter received infusion of CD4(+) CD25(+) Foxp3(+) Tregs separated from normal mouse spleen by magnetic activated cell sorting. The changes of muscle pathology was observed, and the expression of PD-1 and CTLA-4 in spleen CD4(+) CD25(+) Foxp3(+) Tregs was analyzed using flow cytometry; peripheral blood IL-10 and TGF-ß levels were tested using double antibody sandwich ELISA. RESULTS: Compare with the model group, the mice in the treatment group showed significantly reduced muscular inflammatory cell infiltration, increased blood levels of IL-10 and TGF-ß (P<0.05), and increased expression of PD-1 and CTLA-4 in spleen CD4(+) CD25(+) Foxp3(+) Tregs (P<0.05). CONCLUSION: CD4(+) CD25(+) Foxp3(+) Tregs reinfusion produces therapeutic effect in mice with EAM by increasing peripheral blood IL-10 and TGF-ß levels and PD-1 and CTLA-4 expressions in spleen CD4(+) CD25(+) Foxp3(+) Tregs.

Voriconazole: a novel treatment option for cryptococcal meningitis.

BACKGROUND: Cryptococcal meningitis (CM) is a relatively common opportunistic infection in patients with human immunodeficiency virus (HIV) infection and can also occur in patients with no underlying disease. The aim of this study was to evaluate the clinical manifestations, laboratory findings, diagnosis and misdiagnosis, treatment, and prognosis of CM at a tertiary care hospital. METHODS: We performed a retrospective study of 55 patients at a tertiary care hospital from January 1, 1992 to December 31, 2013. All the patients had a definite diagnosis based on etiology. RESULTS: All 55 patients had a positive cerebrospinal fluid (CSF) India ink staining result. The predominant change observed on magnetic resonance imaging (MRI) was leptomeningeal liner enhancement, which is also called 'lumbriciform-enhancing.' Only 15 patients were first diagnosed with CM, indicating a misdiagnosis rate of 72.7%. At the follow-up end point, 8 patients were cured, 33 had improved, and 14 had died. The overall response rate was 74.5%. The voriconazole group had a response rate of 100%, which was significantly higher than the other two groups. CONCLUSIONS: Most CM patients in China were previously healthy without any potential risk factors. CM was easily misdiagnosed due to the lack of specificity of early clinical symptoms. Repeated CSF India ink staining should be performed to identify the pathogen. Voriconazole could be administered to the patients with CM, especially to patients who had a treatment failure with amphotericin B alone or accompanied by fluconazole.

Lamotrigine-induced severe cutaneous adverse reaction: Update data from 1999-2014.

We systematically reviewed and analyzed published patients with Stevens-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN) associated with lamotrigine therapy to identify characteristics of these reactions. We identified a total of 70 patients (42 SJS, five SJS/TEN, 23 TEN). The female to male ratio was 2.83:1 in the TEN group and 1.47:1 in the SJS group. Patients in the TEN group were younger than in the SJS group but this difference was not significant (28.35 versus 32.71 years, respectively; p=0.27). The median time to onset was 25.33 versus 18.42 days for SJS and TEN, respectively. The median dosage at onset was 36.46 versus 57.29mg, and final dosage 111.25 versus 97.92mg/day for SJS and TEN, respectively. The median final dosages did not significantly differ. Concomitant use of valproate acid was reported in 54.55% of the SJS patients and 50.00% of the TEN patients. Three fatal reactions were reported, of which two patients deteriorated rapidly and died within 12h of admission, indicating that this disease can develop rapidly before effective treatment. There was no significant difference between the SJS and TEN groups in any of the clinical factors examined which confirmed the opinion that SJS and TEN are part of a single disease spectrum.

Lamotrigine induced DIHS/DRESS: Manifestations, treatment, and outcome in 57 patients.

OBJECTIVE: To evaluate the clinical features, course, response to treatment, and outcome of lamotrigine induced drug-induced hypersensitivity syndrome (DIHS) or drug reaction with eosinophilia and systemic symptoms (DRESS). METHODS: A comprehensive PubMed and Scopus search (covering the period from January 1999 through April 2014) of the English and non-English literature (with English abstract) was conducted to identify published reports of severe cutaneous adverse reactions (SCARs) associated with lamotrigine therapy. RESULTS: This study population included 57 patients, of whom 38 (66.67%) were female and 19 (33.33%) were male. The latency period varied from 9 days to 120 days, with a mean of 27.58 ± 20.65 days. Multisystem involvement was present in 97.37% (37/38) patients. Systemic corticosteroids were administered to (61.29%) 19/31 patients. 35/38 (92.11%) patients recovered completely, one patient developed liver failure and needed liver transplant, one died from septic shock and one died from multiple organ failure. CONCLUSIONS: We found a greater predominance of women with LTG-DIHS/DRESS, and 68.42% patients were >18 years of age. The presenting symptoms in most of patients were fever, skin rash, liver involvement, hypereosinophilia, and lymphadenopathy. Lamotrigine is associated to a rather high risk of severe cutaneous adverse reactions and to the risk of dying from such reactions, likes many other anticonvulsants. Early recognition and withdrawal of the suspected agent may avoid irreversible damage to the organs will be life saving.

Diffusion-weighted MRI findings and clinical correlations in sporadic Creutzfeldt-Jakob disease.

The objective of this study is to investigate the hyperintense lesions on diffusion-weighted magnetic resonance imaging (DWI) and its clinical correlation in sporadic Creutzfeldt-Jakob disease (sCJD). Patients who suffered from sCJD and followed up at the Department of Neurology at the General Hospital of the People's Liberation Army during the period of June 1, 2007 to July 1, 2014 were reviewed. The location of the hyperintense lesions on DWI, apparent diffusion coefficient (ADC) values of the hyperintense lesions were correlated with symptoms and clinical course. A total of 58 sCJD patients and ten healthy controls were included. Hyperintense lesions on DWI were observed in all the patients. The patients with basal ganglia (BG) hyperintense lesions on DWI had shorter disease duration and higher incidence of myoclonus (92 versus 44 %) than those without BG hyperintense lesions. The patients with occipital cortex hyperintense lesions on DWI had shorter disease duration between symptom onset and akinetic mutism than those without these lesions. The lower of the BG ADC value the faster presence of akinetic mutism and the shorter disease duration the patients will have. The presence of BG and occipital cortex hyperintense lesions on DWI and BG ADC values is correlated with the clinical course and clinical symptoms.

Risk of a lamotrigine-related skin rash: current meta-analysis and postmarketing cohort analysis.

PURPOSE: We systematically reviewed studies to provide current evidence on the incidence and risk of skin rash in patients with LTG therapy. METHODS: PubMed and Scopus databases, up to 15 March 2014 were searched to identify relevant studies. Eligible studies included prospective studies, retrospective studies and postmarketing reports, which included data of skin rash in patients with LTG therapy. RESULTS: Forty-one articles met the entry criteria. A total of 4447 patients with LTG therapy from 26 prospective studies, 2977 patients from 8 retrospective studies, and 26,126 patients from 5/7 postmarketing reports were included. The overall incidence of skin rash with LTG therapy was 9.98% (444/4447) from prospective studies, 7.19% (214/2977) from retrospective studies, and 2.09% (547/26,126) from postmarketing reports. A meta-analysis of the risk of skin rash in 21 prospective studies, did not show a significant difference between patients with LTG and other drugs, including placebo, other ADEs or lithium (OR 0.99-2.41). In 6 respective studies, there was a significantly higher OR in patients with LTG compared with those with non-aromatic AEDs. However, there was no significant difference in rash risk between patients with LTG and aromatic AEDs. CONCLUSIONS: Our study showed that LTG significantly increased the risk of developing a skin rash compared to non-aromatic AEDs. Our results support the need for large prospective population-based studies and clinical trials to determine whether LTG increases the risk of developing a skin rash than compared to other drugs.

Chinese specific characteristics of sporadic Creutzfeldt-Jakob disease: a retrospective analysis of 57 cases.

OBJECTIVE: Sporadic Creutzfeldt-Jakob disease (sCJD) is a fatal and transmissible neurodegenerative disorder. However, no studies have reported Chinese specific characteristics of sCJD. We aimed to identify differences in sCJD between Chinese patients and patients from other countries. METHODS: The data from 57 Chinese sCJD patients were retrospectively analyzed, including demographic data, clinical manifestations, laboratory examinations, electroencephalograms (EEGs), diffusion-weighted imaging (DWI) scans, positron emission tomography (PET) scans, and pathological results. RESULT: The disease was pathologically confirmed in 11 patients. 39 cases were diagnosed as probable sCJD, and 7 were possible. Of the total cases, 33 were male, and 24 were female. The onset age ranged from 36 to 75 years (mean: 55.5, median: 57). Disease onset before the age of 60 occurred in 57.9% of patients. The disease duration from onset to death ranged 5-22 months (mean: 11.6, median: 11), and 51.9% of patients died 7 to 12 months after disease onset. The majority of patients presented with sub-acute onset with progressive dementia. 3 of the 9 patients who took 14-3-3 protein analysis had positive results (33.3%). The sensitivity of EEG was 79.6% (43/54). For DWI and PET examinations, the sensitivities were 94% (47/50) and 94.1% (16/17), respectively. In seven patients who did not show typical hyper-intensities on the first DWI examination, abnormalities of hypo-metabolism in the cerebral cortex were clearly detected by PET. In 13 out of the 17 patients, PET detected extra abnormal regions in addition to the hyper-intense areas observed in DWI. CONCLUSION: This is the first study to indicate that Chinese sCJD patients have a much earlier onset age and a longer disease duration than other populations, which is most likely related to racial differences. The longer disease duration may also be a probable characteristic of Asian populations. PET had high sensitivity for the diagnosis of sCJD.

Dural enhancement detected by magnetic resonance imaging reflecting the underlying causes of cerebral venous sinus thrombosis.

Dural enhancement detected by magnetic resonance imaging is a common finding in patients with cerebral venous sinus thrombosis (CVST) and is usually interpreted as a change secondary to CVST. We report two cases of CVST with intense and diffuse dural enhancement that resulted from pachymeningitis in one patient and spontaneous intracranial hypotension in another. Pachymeningitis and spontaneous intracranial hypotension were also determined to be the underlying causes of CVST. The clinical data of these two patients are described. In patients with CVST, dural enhancement is not always a secondary change to CVST. It can be a manifestation of the underlying causes of CVST. When diffuse and intense dural enhancement is revealed, sufficient ancillary tests are warranted to rule out other potential pathological changes of the dura mater those can result in CVST.