Peripheral nervous system involvement in HCV-related mixed cryoglobulinemia

In HCV-related mixed cryoglobulinemia (MC) a peripheral neuropathy (PN) may occur. To evaluate the prevalence and the characteristics of PN, 133 consecutive patients with HCV-MC (117 type II, 16 type III) were studied. Neurologic evaluation was performed according to the guidelines of Italian Group for the Study of Cryoglobulinemias, using a neurological disability score and a neurological symptom score. In 52/133 patients an electrophysiologic study (ENG) of ulnar, peroneal and sural nerves was performed. For 27/52 patients ENG data registered at different times (interval 12-96 months) were available. In 11 patients a sural nerve biopsy was obtained. An overt PN, mostly as sensory asymmetrical or symmetrical nerve impairment, was found in 107/133 patients (80.4%). ENG abnormalities-reduction or absence of sensory and sometimes of motor action potential, normal or slightly impaired nerve conduction velocity, consistent with axonal damage- were detected in 48/52 patients (92.3%). In 26 out of the 27 patients observed at different times an evolution of PN was found. Nerve biopsies showed a prevalent axonal damage, swollen endothelial cells in epi- and perineurial vessels and scarce mononuclear perivascular infiltrates. No leukocytoclastic vasculitis was observed. Immunoglobulins and complement in sub-perineurial vessel wall were detected. CONCLUSIONS: In HCV-MC a PN is frequent. It is mostly a sensory and progressively worsening axonopathy. Different mechanisms may be involved in the pathogenesis of this disorder and a direct role of HCV cannot be excluded.     

Central nervous system vasculitis caused by propylthiouracil therapy: a case report and literature review.

Antineutrophil cytoplasmic antibodies (ANCA) are associated with vasculitis, including vasculitis induced by drugs such as the thionamides. The affected organ systems in thionamide-induced vasculitis have been primarily renal, musculoskeletal, and dermatologic. We describe the first case of thionamide-induced central nervous system vasculitis presenting as confusion, with complete resolution after discontinuation of propylthiouracil. We review the literature and summarize 42 additional cases of thionamide-induced ANCA-positive vasculitis since 1992. Propylthiouracil was responsible in 93% of cases and the predominant ANCA pattern on immunofluorescent staining was perinuclear (p-ANCA). Clinical improvement occurred after drug discontinuation in 93%, steroid therapy was used in some cases. The mean duration of treatment with thionamides was 35 months prior to presentation. Long-term medical treatment with thionamides for hyperthyroidism may increase the risk of this severe side effect.     

艾滋病合并鸟-胞内分枝杆菌复合群中枢神经系统感染1例并文献复习

[摘要]　非结核分枝杆菌相关颅内感染相对罕见。本文报道了1例艾滋病合并鸟-胞内分枝杆菌复合群中枢系统感染病例。病例系1名45岁女性患者,因“头晕3月,加重伴头痛、失语1周”住院,入院后进行脑脊液检查,并利用二代宏基因组测序发现感染病原菌为鸟-胞内分枝杆菌复合群,从而采取相应的治疗措施,最终获得治愈。本文通过复习有关资料,深入探讨了艾滋病和非结核分枝杆菌相关性颅内感染的临床表现和处理,提示临床医生警惕非结核分枝杆菌相关性颅内感染及其严重性,及时确定病因并实施相应的干预措施。     

骨原发性淋巴瘤继发中枢神经系统浸润1例病例报告及文献复习

目的:探讨骨原发性淋巴瘤(primary bone lymphoma,PBL)继发中枢神经系统(CNS)浸润的主要临床特点、诊断及治疗。方法:分析我科收治的1例弥漫大B细胞性PBL继发CNS浸润患者的临床表现及相关检查结果,治疗方法,并复习相关文献。结果:本例患者为老年男性,主要临床表现为骨痛,经PET/CT、骨髓象、骨组织活检、免疫组化及流式细胞学等检查确诊为弥漫大B细胞性PBL。行R-CHOP化疗方案治疗,化疗后症状缓解,但出现CNS浸润。先后给予6次鞘内注射(Ara-C、MTX、DEX),并同时2次大剂量的甲氨蝶呤(HD-MTX)系统性化疗,10次腰骶部放疗后,症状缓解,但3个月后因肺部感染死亡。结论:本例患者以骨痛为首发表现,PET/CT结合MRI示多发骨损害,经骨髓象及活检、免疫组化染色,流式细胞学检查明确诊断,对PBL应重视CNS侵犯及预防。     

Central nervous system infection due toClostridium septicum: A case report and review of the literature

Summary A patient with end stage breast cancer was admitted to hospital due to fever, chills, multiply eroded discharging wounds, and sudden onset of left hemiparesis.Clostridium septicum bacteremia and brain abscess were diagnosed. The patient was treated successfully with intravenous penicillin and clindamycin and stereotactic aspiration of the abscess. Eleven cases ofC. septicum central nervous system infection are reviewed. They showed an extremely fulminant course and high fatality. Nevertheless, some relationship seems to exist between outcome and type of brain lesion. Hemolytic-uremic syndrome associated with central nervous system infection is also discussed, because all these cases in the literature were due to this organism. Early diagnosis and aggressive treatment, including surgical drainage and appropriate antibiotics, are the key to improving the prognosis. A long-term prophylactic oral antimicrobial agent is suggested for patients who survive this infection.     

Central nervous system graft-versus-host disease: report of two cases and literature review

Two patients with a plausible diagnosis of central nervous system graft-versus-host disease (CNS-GVHD) are described. Both presented with neurological manifestations 6 and 18 months following allogeneic transplant with hemiparesis, seizure, encephalopathy and magnetic resonance findings of hyperintense white matter lesions on T-2 weighed images. Brain biopsy in one and autopsy in the other revealed profound perivascular lymphocytic infiltrates composed predominantly of T-lymphocytes that were of donor origin. Although an unequivocal diagnosis of CNS-GVHD is difficult to establish, the transplantation community should be aware of this controversial entity.     

Peripheral nervous system involvement in essential cryoglobulinemia and nephropathy

The clinical and neurophysiological features of 23 patients affected by essential cryoglobulinemia (EC) have been studied. It was possible to perform sural nerve biopsy in 3 cases. Six patients were found to be affected by a peripheral neuropathy, according to the WHO criteria, while in 8 other patients clinical and neurophysiological signs of a milder peripheral nervous system (PNS) involvement were evident. The incidence of PNS involvement seems to be high (60.9%). Neurophysiological and histological studies were indicative of a mainly axonal damage.     

Central nervous system infection caused by Pseudallescheria boydii: case report and review

Pseudallescheria boydii is an uncommon cause of central nervous system (CNS) infections. Of a total of 21 cases of CNS infections caused by P. boydii (one described for the first time and 20 reported previously in the English-language literature), eight occurred in immunosuppressed patients and four in patients who nearly drowned; three were trauma related and two were iatrogenic (epidural anesthesia and ventriculoperitoneal shunt, respectively); one occurred in a patient with insulin-dependent diabetes mellitus, and three, in patients without underlying disease or predisposing conditions. The clinical manifestations included brain abscesses (13 cases), spinal pachymeningitis (two cases), chronic meningitis (two cases), intraventricular device-related ventriculitis (two cases), cranial epidural abscess (one case), and acute meningitis (one case). Seven patients showed clinical evidence of extraneural involvement by P. boydii. The outcome is known for 20 patients. Of these, the diagnosis was established while the patient was alive in 18 cases, in spite of which, 15 patients died. All five survivors were managed surgically, and two received intravenous miconazole. P. boydii should be included in the differential diagnosis of CNS infections that occur in the face of immunosuppression, near drowning, and trauma. Occasionally P. boydii can cause CNS infection in the absence of underlying disease or predisposing conditions.     

Central nervous system myelomatosis: review of the literature

Involvement of the central nervous system (CNS) in multiple myeloma (MM) is very uncommon; it has been observed in approximately 1% of the MM patients. This review summarizes the clinical and laboratory characteristics and treatment modalities of 109 patients with CNS myelomatosis (CNS MM) reported in the literature. CNS MM has a wide spectrum of neurological symptoms and signs. No guidelines for therapy of CNS MM are available, which has resulted in a large variation in the treatment schedules. Treatment options include intrathecal chemotherapy (IT), systemic chemotherapy (SC), cranial irradiation (CI) or a combination. The prognosis of CNS MM remains poor, with an overall median survival from the time of diagnosis to death of 2.0 months (range 0.1–25 months). Patients who were treated with CI had a significantly ( P  = 0.004) longer survival when compared with patients without CI.     

Central nervous system involvement in patients with mantle cell lymphoma

 In small cell lymphomas, central nervous system (CNS) involvement has been considered to be very rare. Mantle cell lymphoma (MCL) is a distinct subtype of non-Hodgkin's lymphomas consisting of small or intermediate lymphatic B-cells. It has a poorer prognosis than the other small cell lymphomas. Only a few MCL patients with CNS involvement have been reported in the literature to date. We analyzed retrospectively the incidence, clinical characteristics, and outcome of CNS involvement in 94 patients with confirmed MCL treated at one center from 1980 to 1997. Four of the 94 patients (4%) developed CNS lymphoma during the median follow-up of 51 months. The diagnosis was based on clinical, cytological and radiological findings. CNS involvement appeared at 4.6, 56, 66, or 86 months from the diagnosis of MCL. All patients had neurological symptoms and a leukemic disease; two cases were seen with a blastoid morphology. Malignant lymphatic cells were detected in spinal fluid in all cases and parenchymal infiltrations in brain in two. All patients were treated with intrathecal chemotherapy, without response. Survival time after diagnosis of CNS lymphoma ranged from 18 to 55 days. At diagnosis, no adverse prognostic factors predictive of CNS lymphoma were found. CNS involvement was associated with a progressive leukemic disease as a late event or a blastoid transformation. The prognosis of MCL patients with CNS involvement is poor. Received: July 30, 1998 / Accepted: November 12, 1998     

Central nervous system involvement in patients with rotavirus gastroenteritis.

Two children with rotavirus gastroenteritis are presented. The first case developed a fatal Reye's syndrome and the other one encephalitis with slow recovery. The rotavirus diagnosis was made in both cases by electron microscopy and a significant rise in antibody titres to Nebraska calf diarrhea virus was seen in one of the two patients.     

多发性骨髓瘤伴胸膜浸润1例

1病历资料患者女,77岁。2010年2月起反复出现胸闷、气急,无发热、咳嗽、盗汗、胸痛、心悸等不适,夜间尚可平卧。既往有高血压病史20余年,血压最高达180/110 mmHg(1 mmHg=0.133 kPa),平素服用降压药,血压控制良好。入院体检:神志清楚,消瘦,全身皮肤无黄染,无皮下出血点,球结膜无水肿,颈     

异基因造血干细胞移植治疗原发性噬血细胞综合征合并中枢神经系统病变一例报告及文献复习

目的 探讨原发性噬血细胞综合征（HLH）合并中枢神经系统病变诊断要点以及异基因造血干细胞移植（Allo-HSCT）治疗情况。 方法：对1例根据HLH-2004诊断标准确诊的原发性HLH合并中枢神经系统病变的病例临床特点进行分析,完善基因测序、免疫学指标检测和家系调查,进行Allo-HSCT。 结果：确诊11岁男性病例1例,表现为反复发热、全血细胞减少,脾大、骨髓中可见噬血现象,NK细胞活性下降（10.39%）。基因检测和家系调查显示患者携带分别来自父系和母系的PRF1基因的复杂杂合改变,两位胞姐各自携带不同突变位点;全家成员穿孔素蛋白表达量均有不同程度下降。病程中出现癫痫,头颅核磁共振提 示多发病变。确诊原发性HLH合并中枢神经系统病变。给予HLH-2004方案治疗后,接受胞姐HLA 5/10相合Allo-HSCT。目前移植后14个月,一般情况良好。 结论 对于合并中枢神经系统病变的原发性HLH,尽早进行Allo-HSCT是获得长期生存及治愈的唯一方法。     

Legionnaires' disease with pronounced cerebellar involvement:case report and literature review

正Objective To summarize the clinical course,neuroimaging and cerebrospinal fluid (CSF) analyses of cerebellar dysfunction in Legionnaires’ disease.Methods A case of Legionnaires’ disease with pronounced cerebellar involvement was reported.The related literatures published up to February 2019 were reviewed with "Legionella,legionellosis,legionnaires’ disease,cerebellum,     

Central nervous system involvement in patients with granulomatosis with polyangiitis: a single-center retrospective study

Clinical Rheumatology - The aims of this study were to estimate the frequency of central nervous system (CNS) involvement in Greek patients with granulomatosis with polyangiitis (GPA) and describe...     

Right ventricular involvement in hypertrophic cardiomyopathy: a case report and literature review

Although hypertrophic cardiomyopathy (HCM) is classically considered a disease of the left ventricle, right ventricular (RV) abnormalities have also been reported. However, involvement of the right ventricle in HCM has not been extensively characterized. The literature regarding prevalence, genetics, patterns of involvement, histologic findings, symptoms, diagnosis, and treatment of RV abnormalities in HCM is reviewed. To highlight the salient points, a case is presented of apical HCM with significant RV involvement, with an RV outflow tract gradient and near obliteration of the RV cavity, in the absence of a left intraventricular gradient. Right ventricular involvement in HCM appears to be as heterogeneous as that of the left ventricle. The spectrum extends from mild concentric hypertrophy to more unusual severe, obstructive disease. While in some cases the extent of RV involvement correlates with left ventricular (LV) involvement, predominant RV disease can be seen as well. While the genetics of RV involvement have not been well characterized, histologic findings appear to be similar to those in the left ventricle, suggesting similar pathogenesis. Significant RV involvement may result in RV outflow obstruction and/or reduced RV diastolic filling, with potentially increased incidence of severe dyspnea, supraventricular arrhythmias, and pulmonary thromboembolism. The optimal treatment for patients with significant RV disease is unknown. Medical and surgical therapies have been attempted with variable success; experience with newer techniques such as percutaneous catheter ablation has not been reported. Further characterization of RV involvement in HCM is necessary to elucidate more clearly the clinical features and optimal treatments of this manifestation of HCM.