Relapsing polychondritis-associated meningoencephalitis initially presenting as seizure: a case report and literature review.

Background and purpose: Relapsing polychondritis (RP) is a rare rheumatologic disorder that may affect the neurological system with various presentations. In this study, we present a case and summarize the clinical characteristics of RP-associated meningoencephalitis. Case presentation: A 48-year-old man presented with first-ever seizures that were well controlled by valproate. Physical examination results were unremarkable, except for binaural deformation. The initial brain magnetic resonance imaging (MRI) without contrast and electroencephalogram (EEG) findings were normal. However, the patient subsequently developed recurrent fever, scleritis, headache, lethargy, and left arm paresis. Repeated brain MRI with contrast demonstrated increased enhancement of the pia mater and abnormal diffusion-weighted imaging (DWI) signals in the bilateral auricles. The cerebrospinal fluid (CSF) analysis showed 2 leukocytes/µL, 736.5 mg/L of protein, and no evidence of infectious disease or autoimmune encephalitis. Meningoencephalitis secondary to RP was considered. The patient's condition improved significantly and quickly with the administration of dexamethasone (10 mg per day). Oral methylprednisolone was continued, and the patient remained well without relapse during the 9-month follow-up period. Conclusion: RP-associated meningoencephalitis is rare but fatal. Although symptoms vary, red or deformed ears remain the most common and suggestive features. Non-specific parenchymal changes and/or meningeal enhancement can be observed on brain MRI scans. CSF lymphocytic pleocytosis with mild protein elevation was observed in most patients.

NLRP3 inflammasome up-regulates major histocompatibility complex class I expression and promotes inflammatory infiltration in polymyositis.

OBJECTIVE: This study was designed to investigate the role of the nucleotide-binding-domain -and leucine-rich repeat -containing (NLR) family, pyrin-domain-containing 3 (NLRP3) inflammasome in the pathogenesis of polymyositis (PM). METHODS: Immunochemistry was performed to analyze the NLRP3, caspase-1 and interleukin-1 beta (IL-1ß) expression in the muscle tissue of PM patients. Rat model of PM and C2C12 cell were used to investigate the potential role of NLRP3 inflammasome in PM. RESULTS: The percentage of CD 68+ macrophages, and the expression levels of NLRP3, caspase-1 and IL-1ß in the muscle tissue were elevated in 27 PM patients. LPS/ATP treatment resulted in activation of NLRP3 inflammasome and secretion of IL-1ß as well as interferons (IFNs) and monocyte chemotactic protein-1 (MCP-1) in the Raw 264.7 macrophages. Meanwhile, LPS/ATP challenged activation of NLRP3 inflammasome induced overexpression of major histocompatibility complex class I (MHC-I), a key molecular of PM in the co-cultured C2C12 cells. The effect was decreased by treatment of NLRP3 inflammasome inhibitor MCC950 or siRNA of NLRP3 inflammasome. These findings suggested certain levels of IL-1ß rather than IFNs up-regulated MHC-I expression in C2C12 cells. IL-1ß blockade using neutralizing IL-1ß monoclonal antibody or siRNA of IL-1ß suppressed MHC-I overexpression. In vivo, NLRP3 inflammasome inhibition by MCC950 reduced the expression of NLRP3, IL-1ß and MHC-I in the muscle tissue of PM modal rats. Also, it attenuated the intensity of muscle inflammation as well as the CRP, CK, and LDH levels in the serum. CONCLUSION: NLRP3/caspase-1/IL-1ß axis may play an important role in the development of PM. Inhibition of NLRP3 activation may hold promise in the treatment of PM.

Benign paroxysmal positional vertigo in spontaneous intracranial hypotension.

OBJECTIVES: To assess the prevalence and related factors of benign paroxysmal positional vertigo (BPPV) in patients with spontaneous intracranial hypotension (SIH). METHODS: We retrospectively reviewed 156 consecutive inpatients with SIH, and collected the clinical and radiological data. These patients were divided into BPPV group and non-BPPV group according to the clinical manifestation and the results of Dix-Hallpike or supine roll tests during hospitalization period. We performed a univariate analysis and a further multiple logistic regression analysis to identify the related factors of the development of BPPV in SIH patients. RESULTS: BPPV was detected in 18 patients among the total 156 SIH patients (11.54%). The univariate analysis showed a low cerebrospinal fluid (CSF) pressure (P = 0.018), a small pontomesencephalic angle (P = 0.012) and a positive venous distension sign (VDS) (P = 0.045) were associated with the presence of BPPV. But the multivariate analysis only demonstrated a low CSF pressure was related to the presence of BPPV (OR = 1.022, 95% CI: 1.001-1.043, P = 0.044). CONCLUSION: BPPV is common in SIH patients. SIH patients with low CSF pressure may be prone to develop BPPV.

Pulmonary hypertension as a manifestation of mitochondrial disease: A case report and review of the literature.

BACKGROUND: Mitochondrial diseases are a group of multisystem heterogeneous diseases caused by pathologic dysfunction of the mitochondrial respiratory chain. A wide range of clinical expression has been described. However, pulmonary hypertension has rarely been described in association with mitochondrial disease until the past decade, and there is no currently recognized treatment for the pulmonary hypertension complicated with mitochondrial disorder. PATIENT CONCERNS: We reported the case of a 15-year-old boy who presented with shortness of breath and exercise limitation after a cold, and the diagnosis of pulmonary hypertension was confirmed by right heart catheter. Other examinations, such as blood tests, high- resolution chest computed tomography scan, and pulmonary function test, excluded other associated diseases as causes of pulmonary hypertension. DIAGNOSES AND OUTCOMES: The initial diagnosis was idiopathic pulmonary arterial hypertension and an injection of vasodilator (Treprostinil) was given. However, the dyspnea and fatigue subsequently got worsened. Tracing back his family history, together with the electromyography, nerve conduction studies, and the result of muscle biopsy, mitochondrial disease was confirmed. After treatment with vitamin E, vitamin B2, ATP, and coenzyme Q10, the patient's condition improved. CONCLUSION: Pulmonary hypertension should be considered as another potential manifestation of mitochondrial disease. Both mechanism and treatment for pulmonary hypertension complicated with mitochondrial disease are unclear. Further study is necessary.

Sensitivity and specificity of immunocytochemical staining of mycobacterial antigens in the cytoplasm of cerebrospinal fluid macrophages for diagnosing tuberculous meningitis.

The sensitivity and specificity of immunocytochemical staining of mycobacterial antigens in the cytoplasm of cerebrospinal fluid (CSF) macrophages for diagnosis of tuberculous meningitis (TBM) was prospectively compared with Ahuja criteria from 393 consecutive CSF specimens. The assay can play an important role for the diagnosis of TBM, with sensitivity of 73.5% and specificity of 90.7%.

Discrimination of myocardial infarction stages by subjective feature extraction.

Lots of studies on myocardial infarction (MI) computer assisted diagnosis are based on certain important ECG components which only account for local information. 12-Lead ECG signals which were regarded as hyper-dimensional time-series data were utilized to extract features from global information in this study. Existing feature extraction techniques for classification attempt to classify all the classes included. However sometimes it is more important to better recognize certain specific classes rather than to discriminate all the classes. A feature extraction method based on subjective-classification was proposed to discriminate the specific classes, which the classification priority was given subjectively, and each of the other classes was separated at the same time. The method includes data reduction by principal component analysis (PCA), data normalization by whitening transformation and derivation of projecting vectors for subjective-classification, etc. The data in the analysis were collected from PTB diagnostic ECG database. The results show that the proposed method can obtain a small number of effective features from 12-lead ECGs to better classify classes with priority, and the other classes can be classified at the same time.

Investigating the role of 99mTc-TRODAT-1 SPECT imaging in idiopathic Parkinson's disease.

OBJECTIVE: To investigate the role of 99mTc-TRODAT-1 SPECT in diagnosis and assessing severity of idiopathic Parkinson's disease (PD). METHODS: Thirty-eight patients with primary, tentative diagnosis of PD and eighteen age-matched normal controls were studied with 99mTc-TRODAT-1 SPECT imaging. The regions of interests (ROIs) were drawn manually on cerebellum (CB), occipital cortex (OC) and three transverse plane slice-views of striatums, the semiquantitative BG (background)/[(OC+CB)/2] were then calculated. RESULTS: A lower uptake of 99mTc-TRODAT-1 in striatums were displayed in thirty-six out of thirty-eight PD patients by visual inspection, compared to controls. In twenty-four PD cases with (Hoehn and Yahr scale) HYS stage I, a greater loss of DAT uptake was found in striatum and its subregions contralateral striatum to the affected limbs than in the same regions of the controls, although the striatal uptake was bilaterally reduced. Using Spearman correlation analysis showed that the reduction of the uptake ratios significantly correlated with the UPDRS in striatum and all its subregions in the PD group (P<0.05), a similar change was also found in the putamen by using the rating scale of Hoehn and Yahr (P<0.05). However, analysis of variance (ANOVA) did not show any relationship between the decreasing uptake of 99mTc-TRODAT-1 and increasing severity of PD patients, although the specific uptake of 99mTc-TRODAT-1 was continuously decreased in the striatum by visual inspection with the progress of PD from HYS stage I to III. CONCLUSION: 99mTc-TRODAT-1 SPECT imaging may serve as a useful method for improving the correct diagnosis of PD. In assessing the role of 99mTc-TRODAT-1 SPECT in disease severity of PD, UPDRS can offer a comprehensive index, although the Hoehn and Yahr assessment may be available in part.

[An algorithm study on telecardiogram diagnosis based on multivariate autoregressive model and two-lead ECG signals].

OBJECTIVE: In view of the time delay caused by reconstruction of signals at remote sites, a direct classification method with high accuracy suitable for telediagnosis of electrocardiogram (ECG) signals is studied. METHOD: The data for analysis and classification was obtained from MIT-BIH database, including 300 samples each of normal sinus rhythm (NSR), atria premature contraction (APC), premature ventricular contraction (PVC), ventricular tachycardia (VT), ventricular fibrillation (VF) and superventricular tachycardia (SVT). An multivariate autoregressive (MAR) model based technique that could combine the signals of two ECG leads was presented to classify the ECGs directly, including MAR modeling performed on ECGs, and quadratic discrimination function (QDF) based classification by using MAR coefficients and K-L MAR coefficients. RESULT: Besides quick and convenient diagnosis, the accuracy of the proposed classification algorithm was as high as 98.3%-100%. CONCLUSION: The MAR modeling based technique is suitable for telecardiogram diagnosis. Comparing with single-lead ECGs, better classification results can be obtained through the combination of two-lead ECG signals.