A case of Mondini dysplasia with bacterial meningitis

A boy with bilateral hearing impairment developed pneumococcal meningitis at 14-month-old. Further examination revealed cerebrospinal fluid leakage due to bilateral Mondini dysplasia. He was cured by treatment with panipenem/betamiprone and dexamethasone, and then, he was performed an operation to fill the inner ear on day 30. He did not have bacterial meningitis 19 months after the operation. Children with congenital hearing impairment should be examined for malformation of the inner ear because the inner ear malformation has cerebrospinal fluid leakage and bacterial meningitis frequently.     

Multifocal Gorham-Stout disease associated with Chiari I malformation and recurrent aseptic meningitis: Case report and review of literature

Gorham-Stout disease is a rare condition of uncertain aetiology characterised by lymphatic proliferation within osseous structures and subsequent massive osteolysis. This report describes the index case of a patient with multifocal Gorham-Stout disease involving the skull base with Chiari I malformation and recurrent aseptic meningitis without fistula. A five-year-old male presented following decompression of a Chiari I malformation with headaches, vomiting, and stiff neck and cerebrospinal fluid pleocytosis without growth of a pathogenic organism. Ongoing symptoms prompted a further three presentations over several months revealing persistent aseptic cerebrospinal fluid monocytic pleocytosis. Further investigation revealed multifocal osseous cystic disease and subsequent bone biopsy suggested Gorham-Stout disease. Suboccipital decompression was not repeated despite craniocervical junction re-stenosis. A literature review demonstrated the extreme rarity of Gorham-Stout disease associated with Chiari I malformation and meningitis. Potential mechanisms of these entities occurring in concert are discussed. Consideration of Gorham-Stout disease as a secondary cause for Chiari I malformation is important amid local bone changes or cerebrospinal fluid leakage prior to pursuing suboccipital decompression considering the poor outcomes reported.     

Two cases of recurrent bacterial meningitis following traumatic cerebrospinal fluid rinorrhea

We report here two cases of recurrent bacterial meningitis following traumatic cerebrospinal fluid rhinorrhea. Case-1: an 1-year-old girl had a penetrating injury to the nasal cavity with a chopstick. From 1 day after this accident, she had suffered from recurrent bacterial meningitis. She was diagnosed as having cerebrospinal fluid rhinorrhea, and underwent surgical repair of the bone defect. Case-2: a 5-year-old girl had suffered from bacterial meningitis 4 times after head trauma. A bone defect was demonstrated by 3-D CT and repaired surgically. We consider that 3-D CT is a useful tools to detect cerebrospinal fluid fistula.     

Cerebrospinal fluid otorrhea and pseudomonal meningitis in a child with Mondini dysplasia: case report

Purpose

Mondini dysplasia is a rare congenital inner ear malformation that presents with abnormal cochlear development with accompanied vestibular dilation and vestibular aqueduct enlargement. This dysfunctional anatomy provides the potential for sensorineural hearing deficits, cerebrospinal fluid leaks, and severe cases of recurrent meningitis.

Methods

We present the case of a child with Mondini dysplasia who presented with unilateral hearing loss and cerebrospinal fluid (CSF) otorrhea that was surgically repaired through a combined middle fossa/transmeatal middle ear approach to alleviate any recurrence of infection and cerebrospinal fluid otorrhea.

Results

Postoperatively, the patient remained neurologically stable without any further CSF leakage. CSF cultures revealed a Pseudomonas aeruginosa infection, a rare occurrence within the context of Mondini dysplasia. Retrograde bacterial spread from the external ear canal into the CSF space has been theorized as the possible pathogenesis of the resulting meningitis. The patient was successfully treated with intravenous antibiotics without any neurologic complications.

Conclusions

Although Mondini dysplasia is a rare malformation, the life-threatening sequelae of meningitis that can result from the dysfunctional anatomy makes it a condition that requires elevated clinical vigilance, especially when considering children with hearing loss associated with recurrent meningitis, otorrhea, or rhinorrhea.

     

Neurotrophin-3 levels in cerebrospinal fluid from children with bacterial meningitis, viral meningitis, or encephalitis

Neurotrophin-3 levels were measured in the cerebrospinal fluid of 35 patients with bacterial meningitis, viral meningitis, or encephalitis by two-site enzyme immunoassay. Elevated cerebrospinal fluid levels of neurotrophin-3 were demonstrated in 8 of 18 patients with bacterial meningitis. Follow-up examination of the eight patients at the convalescent stage showed diminished cerebrospinal fluid levels of neurotrophin-3. In contrast, none of the 17 patients with viral meningitis or encephalitis showed an elevation of neurotrophin-3 levels in cerebrospinal fluid. No relationships were observed between neurotrophin-3 levels and cerebrospinal fluid cell numbers, cerebrospinal fluid protein levels, serum C-reactive protein concentrations, or outcome in bacterial meningitis. Since neurotrophin-3 is involved in the survival of neurons and the modulation of the immune system, neurotrophin-3 could play a neuroprotective or immunomodulatory role in bacterial meningitis.     

Total antioxidant/oxidant status in meningism and meningitis

The objective of this study was to investigate the antioxidant/oxidant status of serum and cerebrospinal fluid in children with meningismus and acute bacterial meningitis. Twenty-three children (age range, 0.75 to 9 years) with fever and meningeal signs that required analysis of the cerebrospinal fluid, but no cytologic or biochemical evidence of meningitis in their serum and cerebrospinal fluid, constituted the meningismus group. Thirty-one children (age range, 0.5 to 10 years) with acute bacterial meningitis constituted the meningitis group. Twenty-nine healthy children (age range, 0.5 to 11 years) were recruited as control subjects. Antioxidant status (ascorbic acid, albumin, thiol, uric acid, total bilirubin, total antioxidant capacity, catalase and ceruloplasmin concentrations) and oxidant status (lipid hydroperoxide and total oxidant status) were measured. The serum antioxidant status was lower, and oxidant status levels higher in both meningitis and meningismus subjects than in the control children (P < 0.001). Cerebrospinal fluid oxidant status was lower in the meningitis group than in the meningismus group (P < 0.05). These results indicate that serum antioxidant status was lower, and serum oxidant status was higher in children in the meningismus and meningitis groups, whereas cerebrospinal fluid oxidant status was higher in the meningismus group than in the meningitis group.     

Neurotrophin-4 and glial cell line-derived neurotrophic factor in cerebrospinal fluid from meningitis/encephalitis patients

The neurotrophin-4 and glial cell line-derived neurotrophic factor levels were measured in cerebrospinal fluid from 61 patients with bacterial meningitis, viral meningitis, or encephalitis, and other diseases by means of two-site enzyme-linked immunoassay. Elevated cerebrospinal fluid levels of neurotrophin-4 were demonstrated in four of the 11 patients with bacterial meningitis, and seven of the 23 patients with viral meningitis or encephalitis. None of the other patients demonstrated elevation of the neurotrophin-4 level in cerebrospinal fluid. The neurotrophin-4 levels in cerebrospinal fluid were correlated with the numbers of total and mononuclear cells in patients with viral meningitis/encephalitis. In patients with bacterial meningitis, three of the four patients with elevated neurotrophin-4 levels exhibited persistent abnormalities on computed tomography, and one revealed transient subdural effusion. On the other hand, none of the seven patients without neurotrophin-4 elevation had persistent computed tomography abnormalities, and five patients demonstrated transient computed tomography abnormalities. The glial cell line-derived neurotrophic factor levels were below the detection limit, or only slightly higher than the detection limit, in the patients with or without central nervous system infections. Although the precise roles of neurotrophin-4 and glial cell line-derived neurotrophic factor in central nervous system infections remain to be determined, neurotrophin-4 might play a neuroprotective or immunomodulatory role in central nervous system infections.     

Recurrent Streptococcus Pneumoniae Meningoencephalitis in a Patient With a Transethmoidal eningoencephalocele

We report a case of recurrent Streptococcus Pneumoniae meningoencephalitis with a transethmoidal meningoencephalocele (TEME) but without cerebrospinal fluid (CSF) leakage.A 35-year-old man was admitted with S. pneumoniae meningitis. He had suffered from four episodes of recurrent pneumococcal meningitis during the previous 4 years. A computed tomography scan of the paranasal sinus showed the TEME protruding through a bony defect of the right frontal base. However, the patient did not have symptoms that could be attributable to CSF leakage, and radioisotope cisternography did not identify a leak. Brain magnetic resonance imaging revealed cortical lesions overlying the TEME, and electroencephalography revealed epileptiform discharges in frontal regions. Appropriate antibiotics therapy without steroids was given to improve his condition.The presented case suggests that even in the absence of clinically demonstrable CSF leakage, an occult skullbase defect and its associated meningoencephalocele should be considered in patients with recurrent bacterial meningitis.     

Recurrent bacterial meningitis in a case of Crouzon syndrome after craniofacial surgery]

A 29-year-old woman, who was diagnosed as Crouzon syndrome for which two cranio-facial surgeries had been performed as a child and at the age of 19, developed high fever, headache, and confusion for two days. She was admitted to our hospital. She was diagnosed as bacterial meningitis by cerebrospinal fluid examinations, and her condition was immediately improved by antibiotics. At the age of 23, she also suffered from bacterial meningitis caused by otitis media and sinusitis, and recovered by antibiotics with no sequela. Her cranial computed tomography showed sphenoid and ethmoid sinusitis, and bone deformation and hypertrophy with no fistula connecting intracranial space and sinus. Dead space by cranio-facial surgeries might cause the development of chronic or recurrent sinusitis leading to bacterial meningitis. Our patient is the second case of recurrent bacterial meningitis with Crouzon syndrome to our knowledge. We should recognize that recurrent sinusitis with Crouzon syndrome after cranio-facial surgery is a risk of recurrent meningitis.     

经蝶窦垂体腺瘤手术中发生脑脊液漏的原因和防治

目的 探讨经蝶窦入路垂体腺瘤手术中发生脑脊液漏的常见原因及其防治方法.方法 回顾性分析260例显微镜下、274例神经内镜下经蝶窦垂体腺瘤切除术中,46例术中发生脑脊液漏,其中显微镜手术24例,神经内镜手术22例.术中多用人工干燥硬脑膜或自体脂肪及纤维蛋白胶修补,蝶窦内填明胶海绵,对于肿瘤体积巨大,鞍底骨质广泛破坏甚至消失者,用骨片填放于蝶窦内固定填塞的软组织.根据术中修补情况36例术后即刻行腰大池置管持续引流.结果 术中修补46例脑脊液漏,术后13例发生脑脊液漏,经腰大池引流3-6 d愈合;6例发生细菌性脑膜炎,经腰大池引流治愈.结论 术前应充分估计容易发生脑脊液漏的患者,手术操作格外小心,并准备好修补措施;应用自体脂肪及纤维蛋白胶修补经蝶窦垂体腺瘤术中脑脊液漏是有效的方法;如果术后发生脑脊液漏或细菌性脑膜炎,则需要腰大池置管持续引流.

Abstract：

Objective To analyze the causes and prevention and treatment of cerebrospinal fluid leak during transsphenoidal pituitary adenoma surgery.Methods Retrospective study of 260 cases of microscopic transsphenoidal surgery and 274 cases of endoscopic surgery for resection of the pituitary adenomas was performed.CSF leak occurred in 24 cases with microscopic surgery and in 22 cases with endoscopic surgery during operation.The sellar floors were sealed by artificial dried dura or free autogenic fat and fibrin glue for the CSF leakage during surgery.For giant tumors which extensively destroyed sella floor,we put bony plates in sphenoid sinus to support the crammed tissue.Lumber drainage was performed immediately after operation in 36 cases.Methods In the 46 patients with CSF leakage during surgery,13 cases occurred CSF leakage postoperatively,cured in 3-6 days after lumber drainage.6 patients occurred bacterial meningitis,cured after long term lumber drainage.Conclusion Probability of CSF leak before transsphenoidal surgery should be estimated,and preparation for the fixing up the leak is necessary.Using free autogenic fat and ebonite to repaired the sellar floors in case of CSF leakage during surgery is a effective measurement.Patients with CSF leakage or bacterial meningitis after surgery require lumber drainage.     

Fluorescence method of evaluation of the lysosomes of the lymphoid cells of the cerebrospinal fluid in meningitis and meningism

The fluorescence method of Blume et al. was found to be useful for identification and morphological evaluation of lysosomes in cerebrospinal fluid cells. The investigations were carried out in 49 patients with viral and bacterial meningitis or meningismus. It was demonstrated that the CSF cells in most patients with purulent meningitis contained no fluorescent granules in an early stage of the disease before introduction of antibacterial therapy. These granules were found in CSF cells in cases of leptospirosis, viral meningitis and meningismus of various aetiology in acute stage of the disease and even in convalescence, and in purulent meningitis in convalescence. In bacterial meningitis large lysosomal granules were observed, and in viral meningitis these granules were small. The method visualizes easily bacteria (meningococci and pneumococci) in the cerebrospinal fluid.     

TNF-alpha and IL-6 in the diagnosis of bacterial and aseptic meningitis in children

The objective of this study was to analyze the usefulness of tumor necrosis factor-alpha and interleukin-6 cerebrospinal fluid concentrations for the differential diagnosis between bacterial and aseptic meningitis in children and in the prognostic evaluation. A cross-sectional study was performed on 35 children between 1 month and 12 years of age with suspected meningitis. Cytokines determination was performed by enzyme-linked immunosorbent assay technique. The Mann-Whitney test and Spearman's correlation coefficients were used for statistical analysis. Six children presented bacterial meningitis, 13 aseptic, and 16 had no meningitis. The tumor necrosis factor-alpha concentrations were significantly higher in the bacterial meningitis group as compared with the aseptic group (P = 0.001) and among groups with and without meningitis (P = 0.000). There was correlation between tumor necrosis factor-alpha and cerebrospinal fluid leukocytes (P = 0.019), protein (P = 0.000), and glucose (P = 0.038). There was no association between cytokines and complications of the meningitis. The tumor necrosis factor-alpha concentrations in the cerebrospinal fluid were useful markers for distinguishing bacterial from aseptic meningitis and were demonstrated to be useful in evaluating the intensity of the inflammatory process in the central nervous system.     

Irreversible papillitis and ophthalmoparesis as a presenting manifestation of neurobrucellosis

A 35-year-old man presented with a meningeal syndrome and acute onset of visual blurring. Clinical investigations revealed bacterial meningitis with bilateral papillitis and ophthalmoparesis. Serum and cerebrospinal fluid serology confirmed the diagnosis of chronic active neurobrucellosis. Following therapy there was no improvement and he developed optic atrophy. Extensive literature review revealed, one case of bilateral irreversible papillitis resulting from neurobrucellosis. However no cases of neurobrucellosis have been reported with meningitis, irreversible papillitis and ophthalmoparesis. This case demonstrates that in endemic areas, acute meningitis is a potential manifestation of neurobrucellosis and that bilateral irreversible papillitis with ophthalmoparesis can be a potential serious complication.     

DNA single cell cytometry in lymphocytic pleocytosis of the cerebrospinal fluid

The value of DNA single-cell cytometry for the detection of neoplasia in Feulgen-stained cerebrospinal fluid cytological specimens was tested on 34 cases of Non-Hodgkin's lymphoma or leukemia and on 66 cases of viral or bacterial meningitis as a disease control group. The DNA content of 200 randomly chosen nuclei was measured on one pre-existing, cytologically representative slide per case, using a TV-image analysis system TAS-plus (Leitz, Germany). Neoplasia was diagnosed, if at least three nuclei with a DNA content above 5c (5cEE3) were found. The sensitivity investigating only one slide per case was 79.4% (27/34), the specificity 78.8% (52/66). Three lymphomas and 7 inflammatory cases were classified as suspicious (0<5cEE<3). In 4 lymphoma cases (11.8%) a false-negative diagnosis and in 7 cases (10.6%) of viral meningitis a false-positive diagnosis were made. No false-positive diagnosis occurred in bacterial meningitis. While the false-negative diagnoses may be due to the only slightly increased number of cells in cerebrospinal fluid, no final explanation for increased DNA values after viral infection can be given. Therefore, before using DNA single-cell cytometry to proove the malignant character of lymphocytic pleocytosis in cerebrospinal fluid, viral meningitis has to be clinically excluded.     

Large Intracranial Aneurysm after Transsphenoidal Surgery for Pituitary Macroadenoma

Uncontrolled cerebrospinal fluid (CSF) leakage after transsphenoidal surgery (TSS) for pituitary adenoma can lead to meningitis. Intracranial mycotic pseudoaneurysm is a rare complication in central nervous system infection. Large single pseudoaneurysm is more uncommon. Most mycotic aneurysms occur due to endocarditis. The present patient had no heart problem and was infected by CSF leakage after transsphenoidal surgery. We present a case of large ruptured mycotic pseudoaneurysm as a complication of cerebral infection after TSS for pituitary macroadenoma.     

Cerebrospinal Fluid TNF-α, IL-6, and IL-8 in Children With Bacterial Meningitis

ObjectiveWe evaluated the levels of cerebrospinal fluid concentrations of tumor necrosis factor-α, interleukin-6, and interleukin-8 in bacterial meningitis in children.MethodsThe study included children up to 14 years of age admitted to a pediatric ward with fever, headache, vomiting, and seizures. The diagnosis of bacterial meningitis was based on clinical features: physical examination, blood and cerebrospinal fluid cytochemical findings, Gram stain, and bacterial culture. The cerebrospinal fluid levels of tumor necrosis factor-α, interleukin-6, and interleukin-8 were measured in 57 children with bacterial meningitis, 15 with viral meningitis, and 15 controls by enzyme-linked immunosorbent assay methods.ResultsThe mean concentrations of cerebrospinal fluid, tumor necrosis factor-α, interleukin-6, and interleukin-8 were 1108 ± 183, 652 ± 287, and 442 ± 120 pg/mL, respectively, in children with bacterial meningitis and were significantly increased in those in the viral meningitis group (tumor necrosis factor-α : 711 ± 105, IL-6 : 272 ± 161, IL-8 : 175 ± 62 pg/mL; P < 0.001) or control (390 ± 37, 59 ± 17, 19 ± 13 pg/mL, respectively, P < 0.001). At optimum cutoff level based on the receiver operating characteristic curve, cerebrospinal fluid cytokines (tumor necrosis factor-α, interleukin-6, and interleukin-8) showed sensitivity and specificity of 100% for the diagnosis of bacterial meningitis. For differentiation of bacterial from viral meningitis, cerebrospinal fluid level of tumor necrosis factor-α, IL-6, and IL-8 showed sensitivity and specificity of 94.7% and 86.7%, 80.7% and 53.3%, and 89.5% and 86.7%, respectively.ConclusionThe increased concentration of cerebrospinal fluid tumor necrosis factor-α, interleukin-6, and interleukin-8 in children with meningitis suggests a role in the pathogenesis of bacterial meningitis and these levels might prove to be useful in children whose diagnosis is in question.     

Differential regulation of blood-brain barrier permeability in brain trauma and pneumococcal meningitis-role of Src kinases

Increased vascular permeability causing vasogenic brain edema is characteristic for many acute neurological diseases such as stroke, brain trauma, and meningitis. Src family kinases, especially c-Src, play an important role in regulating blood-brain barrier permeability in response to VEGF, but also mediate leukocyte function and cytokine signalling. Here we demonstrate that pharmacological inhibition of Src or c-Src deficiency does not influence cerebrospinal fluid (CSF) pleocytosis, brain edema formation, and bacterial outgrowth during experimental pneumococcal meningitis despite the increased cerebral expression of inflammatory chemokines, such as IL-6, CCL-9, CXCL-1, CXCL-2 and G-CSF as determined by protein array analysis. In contrast, inhibition of Src significantly reduced brain edema formation, lesion volume, and clinical worsening in cold-induced brain injury without decreasing cytokine/chemokine expression. While brain trauma was associated with increased cerebral VEGF formation, VEGF levels significantly declined during pneumococcal meningitis. Therefore, we conclude that in brain trauma blood-brain barrier tightness is regulated by the VEGF/Src pathway whereas c-Src does not influence brain edema formation and leukocyte function during bacterial meningitis.     

Cerebrospinal fluid Diacron-Reactive Oxygen Metabolite levels in pediatric patients with central nervous system diseases

This study assessed the validity of cerebrospinal fluid oxidative status of pediatric patients with central nervous system diseases, using the Diacron-Reactive Oxygen Metabolites test (d-Roms; Diacron International s.r.l.; Grosseto, Italy). Eighty-seven pediatric patients (8 with bacterial meningitis, 18 with aseptic meningitis, 23 with febrile seizures, 6 with rotavirus gastroenteritis-induced convulsions, 16 with epilepsy, 2 with adrenoleukodystrophy, 2 with multiple sclerosis, and 12 control subjects) were enrolled. An analysis of the infection-associated group (bacterial meningitis, aseptic meningitis, febrile seizures, and rotavirus gastroenteritis-induced convulsions) indicated that cerebrospinal fluid Diacron-Reactive Oxygen Metabolite levels in the bacterial meningitis group were significantly higher than in other infection-associated groups. In the bacterial meningitis group, the cerebrospinal fluid Diacron-Reactive Oxygen Metabolite levels obtained after improvement were significantly decreased compared with pre-improvement values. In the noninfection-associated group (epilepsy, adrenoleukodystrophy, and multiple sclerosis), the cerebrospinal fluid Diacron-Reactive Oxygen Metabolite levels in symptomatic epilepsy patients were higher than in cryptogenic epilepsy patients and control subjects, but not significantly. Progressive patients with adrenoleukodystrophy or multiple sclerosis demonstrated high Diacron-Reactive Oxygen Metabolite levels compared with another early-stage adrenoleukodystrophy patient. Oxidative stress may be associated with the pathogenesis of various pediatric central nervous system diseases. Cerebrospinal fluid Diacron-Reactive Oxygen Metabolite levels may correlate with clinical status in these diseases.     

颅脑术后表皮葡萄球菌性脑膜炎的临床分析

目的 分析颅脑术后表皮葡萄球菌性脑膜炎的临床表现、危险因素、细菌耐药性特点,探讨预防措施及治疗方法.方法 回顾性收集1999年8月至2007年8月山东大学齐鲁医院神经外科颅脑术后脑脊液培养证实表皮葡萄球菌生长患者的资料进行统计学分析.结果 6887例颅脑手术患者,术后发生表皮葡萄球菌感染27例,男16例,女11例;年龄2~72岁,平均37.5岁.颅内肿瘤手术4660例,术后感染10例;脑外伤手术787例,术后感染5例;颅内置管性手术577例,术后感染10例(脑室体外引流和脑脊液分流,其中2例为合并脑肿瘤患者);颅内其他手术863例,术后感染4例,其发生率分别为0.21%、0.64%、1.73%、0.46%.置管性手术组与其他手术组相比差异有统计学意义(P<0.05).耐甲氧西林表皮葡萄球菌总体检出率为70.4%,未有耐万古霉素菌株出现.结论 表皮葡萄球菌是颅脑术后颅内感染的主要病原菌,且耐药性逐年增强.其感染好发于颅内置管性手术及污染手术,万古霉素是治疗的首选药物.术前预防性用药疗效不确切,严格无菌术是重要预防措施.     

Increased intrathecal synthesis of fibronectin in bacterial and carcinomatous meningitis

Immunoreactive fibronectin (Fn) was quantified in paired cerebrospinal fluid (CSF) and serum samples from patients with bacterial meningitis (n = 46), tick-borne encephalitis (TBE) (n = 6), HIV infection (n = 6), Guillain-Barré syndrome (n = 5), carcinomatous meningitis (n = 11), multiple sclerosis (n = 15), disk disease (n = 11), and controls (n = 28). A highly significant elevation of CSF Fn was found in bacterial meningitis, TBE, and carcinomatous meningitis. There were no significant differences in serum Fn between any of the groups. An Fn index to estimate the rate of intrathecal Fn synthesis reached the highest value in bacterial meningitis. Our findings suggest that CSF Fn may be an indicator of adequate host reaction and tissue repair. For diagnostic purposes, the determination of CSF Fn probably does not add much to routine CSF laboratory tests.