An 11-year-old girl with recurrent bacterial meningitis due to liquorrhea caused by bone malformation of the skull base

The patient is a healthy 11-year-old girl with no history of trauma or hearing impairment. She developed pneumococcal meningitis three times, at ages 7, 10, and 11. Intracranial examination revealed, pore expansion and cerebrospinal fluid leakage in the right internal acoustic foramen, which were attributed to a bone malformation of the base of the skull. A procedure was performed to close the cerebrospinal fluid leakage; no relapse has been observed thus far. Previous case reports indicate that repetitive bacterial meningitis is often caused by internal ear malformation, trauma, tumors, or surgical operation. This case suggests the possibility that underlying disorders may not be apparent in cases of repetitive bacterial meningitis and, more proactive investigations are required to prevent further recurrence of meningitis.     

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.

     

Bacterial meningitis in adults: demonstration of inner ear involvement using high-resolution MRI

OBJECTIVE: To visualize the sites involved in audiovestibular dysfunction during bacterial meningitis in adults and to relate these findings to the extent of hearing impairment and vestibular dysfunction. BACKGROUND: Hearing impairment is among the most frequent complications of bacterial meningitis. METHODS: High-resolution MRI (HR-MRI) of the inner ear was performed in seven adult patients with hearing loss as a complication of bacterial meningitis. Results: Five patients had unilateral (n = 1) or bilateral (n = 4) contrast enhancement of vestibulocochlear structures. The structures most frequently involved were the cochlear nerve (n = 9), the first cochlear turn (n = 9), the vestibulum (n = 9), and the semicircular canals (n = 7). There was a significant correlation between clinical and MRI findings: all nine ears with cochlear enhancement were deaf (hearing loss >90 dB), whereas none of the five ears with normal MRI findings had hearing losses of more than 90 dB (range, 30 to 70 dB; p = 0.0005). Vestibular dysfunction as revealed clinically and by quantitative vestibular function testing was found in six of seven patients (11 of 14 ears). Five of these patients (nine ears) also demonstrated enhancement of the vestibular organ on high-resolution MRI of the inner ear. CONCLUSIONS: High-resolution MRI can visualize the involvement of vestibulocochlear structures in bacterial meningitis in both cooperative and consciously impaired patients. These findings suggest a correlation between abnormalities on MRI and the extent of cochlear dysfunction.     

Sudden bilateral hearing loss with vertigo due to vertebral artery occlusion]

We report a case of sudden bilateral hearing loss associated with an occlusion of the right vertebral artery. A 66-year-old man was admitted to hospital suffering from sudden onset vertigo, deafness, and vomiting. He could not walk due to truncal ataxia. There was positional nystagmus to the right; spontaneous and gaze-evoked nystagmus were absent. He had no facial nerve palsy, dysarthria, pyramidal tract signs, limb ataxia, and sensory impairment. Pure tone audiometry demonstrated a profound sensorineural deafness of both ears. A tentative diagnosis of sudden deafness was made. He was treated with intravenous infusion of corticosteroid; hearing loss of the left ear slightly improved. Cranial MR imaging demonstrated fresh small infarcts in the right cerebellar tonsil, the left cerebellar medulla, and the left middle cerebellar peduncle. MR angiography demonstrated an occlusion of the right vertebral artery. It is possible that reduced perfusion of the anterior inferior cerebellar arteries and internal auditory arteries on both sides resulted in multiple "border zone" infarcts and damage to the inner ear. Clinicians should be aware of the possibility of vertebrobasilar occlusive disease in case of sudden bilateral hearing impairment, even when brainstem or cerebellar signs are absent.     

Cystic cochleovestibular anomaly presenting with congenital deafness and recurrent bacterial meningitis in childhood

Recurrent bacterial meningitis (RBM) in many instances is associated with identifiable anatomical defects. Presence of congenital deafness with recurrent meningitis should alert clinician for presence of middle and inner ear malformation. These defects can be demonstrated by various neuro imaging techniques and can be surgically corrected. In this case report we describe a child seen at our institute with congenital deafness and recurrent meningitis, discuss the approach to RBM and briefly describe inner ear malformation associated with the same and how to differentiate them.     

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.     

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.     

Mondini dysplasia as a cause for recurrent bacterial meningitis: an early diagnosis

Mondini dysplasia is a rare but an important cause for recurrent pyogenic meningitis in children and requires a high index of clinical suspicion for early diagnosis. We present the case of a 7-year-old boy, who presented with 2 episodes of pyogenic meningitis within a span of 1 month. There was no obvious history of hearing abnormalities, but pure tone audiometry suggested profound mixed hearing loss in the left ear. High-resolution computed tomographic scan and magnetic resonance imaging of temporal bones confirmed the diagnosis of Mondini dysplasia in the left ear. Computed tomographic cisternography failed to demonstrate any obvious cerebrospinal fluid leak. The child was managed conservatively and has been asymptomatic since then. Thus, in our patient, Mondini dysplasia as a cause for recurrent pyogenic meningitis was diagnosed (early) during the second episode of meningitis. The need for an early diagnosis of Mondini dysplasia has been stressed in this report.     

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.     

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.     

Mycoplasma meningitis resulting in increased production of cerebrospinal fluid: case report and review of the literature

PURPOSE: We report a case of increased cerebrospinal fluid (CSF) production in a child with concomitant mycoplasma meningitis. MATERIALS AND METHODS: This 4-year-old boy presented with a 2-week history of body aches, malaise, and headaches. He developed sudden onset of obtundation, apnea, left eye deviation, and bilateral dilated and unreactive pupils. A ventriculostomy was placed initially for a poor neurologic examination in the setting of likely meningitis. Initial intracranial pressure was high, and CSF production was supraphysiologic for the first few days of empiric, broad-spectrum treatment. Mycoplasma meningitis was diagnosed. The ventriculostomy was weaned after adequate treatment for mycoplasma meningitis. RESULTS AND CONCLUSIONS: At 4 months follow-up, the child remains shunt-free with only mild cognitive-linguistic impairment. Untreated mycoplasma meningitis may cause raised intracranial pressure (possibly as a result of increased CSF production) and result in a poor neurological examination. In this setting, CSF diversion in the form of an external ventricular drain may be beneficial to preserve neurologic function during treatment with antibiotics.     

A case of aseptic meningitis caused by relapsing polychondritis]

We report a patient of relapsing polychondritis (RP) with antecedent aseptic meningitis. A 65-year-old man has developed headache and fever. Neurological examination showed meningeal signs, and cerebrospinal fluid (CSF) examination revealed meningeal inflammation which contained 450 polymorphonuclear cells/microl, 302 mononuclear cells/microl, and 0 red cells/microl, with 79 mg protein/dl. Serologic testing for autoimmune disease as well as the culture and cytology of CSF were negative. He admitted our hospital as having aseptic meningitis and experienced antibiotic therapy. However, his pyrexia continued and he developed repeating visual and hearing impairment reacting to steroid. Three months later, he became behaviorally deaf, and bilateral auricular chondritis occurred with nonerosive seronegative inflammatory polyarthritis. The result of condral biopsy was consistent with the diagnosis of RP showing cartilage surrounded by an intense inflammatory cell response with a decreased number of chondrocytes. A clinical diagnosis was made and prednisolone 60 mg/day was begun with the result of resolution of the auricular chondritis, and slight improvement of his deafness. Aseptic meningitis is a rare complication of RP. Only one report detailed RP patient who had preceding meningitis. RP is a potentially lethal disease resulting from suffocation by airway collapse, the complications of a cardiac large vessel, and so on. For improvement of a life prognosis, an early diagnosis and treatment are indispensable. Although RP is a rare discovery, it is necessary that RP should be taken into consideration and be differentiated as a cause of relapsing aseptic meningitis.     

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.     

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.     

The paintfill method as a tool for analyzing the three-dimensional structure of the inner ear

The mammalian inner ear is a complex epithelial tube designed to detect sound, angular and linear acceleration, as well as gravity. The major parts of the ear include three orthogonal semicircular canals, a central vestibule, a coiled cochlea, and an endolymphatic duct and sac allowing fluid balance with the cerebrospinal system. Located throughout this tubular system are six separate sensory areas composed of hair cells and support cells that are essential for the transduction of hearing and balance information. Deafness and vestibular dysfunction are extremely common sensory disorders in the human population, with one in every 1000 children born profoundly hearing impaired and many progressive forms diagnosed later in life. Approximately 20-30% of patients with congenital sensorineural hearing loss demonstrate radiographic abnormalities of the inner ear, indicating that malformations of the inner ear make a significant contribution to the high frequency of deafness and balance disorders. Unfortunately, the very complexity that makes the inner ear such an exquisite structure has also made it an extremely difficult organ to tackle for researchers. Here, I describe a histological method involving paintfilling of the inner ear that provides easy analysis of the three-dimensional structure of this complex organ. The paintfill method can be used to quickly assess inner ear morphology and can help identify defects that may cause or contribute to deafness and/or vestibular dysfunction.     

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.     

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.     

Bacterial meningitis associated with a complex ischiorectal abscess

We describe a case of bacterial meningitis in a 37 year old man resulting from the direct extension of an spontaneously occurring ischiorectal abscess into the intradural space. The patient presented with back pain and urinary retention and this was followed by the development of headache, photophobia and a left VIth nerve palsy. The patient was not diabetic or immunocompromised. He was treated with broad spectrum antibiotics and with drainage of the ischiorectal abscess; no organism was grown from blood, abscess contents or cerebrospinal fluid. He went on to make a full recovery.     

Absolute neutrophil count in aseptic and bacterial meningitis related to time of lumbar puncture

Previous studies have demonstrated that during the first 24 hours of illness caused by aseptic meningitis, polymorphonuclear cells predominate in cerebrospinal fluid and decline afterward. To test the hypothesis that the absolute neutrophil count and percentage of polymorphonuclear cells in cerebrospinal fluid of bacterial meningitis patients are elevated and sustained after 24 hours compared with that of patients with aseptic meningitis. Seventy-two patients with aseptic meningitis and 13 with bacterial meningitis participated. All patients with aseptic meningitis and four patients with bacterial meningitis were followed-up prospectively. The patients were enrolled in four groups according to the interval between onset of symptoms and performance of the lumbar puncture (<12 hours, 12-24 hours, 24-36 hours, and >36 hours). In aseptic meningitis the mean absolute neutrophil count was 182, 164, 79, and 68 cells/mm³, respectively (P = 0.025). In bacterial meningitis the absolute neutrophil count was 28, 1,466, 5,853, and 235 cells/mm³, respectively. The mean percentage of polymorphonuclear cells in aseptic meningitis was 49%, 46%, 40%, and 26%, respectively (P = 0.038); in bacterial meningitis, 70%, 83%, 81%, and 58%, respectively. The findings suggest that the absolute neutrophil count and the polymorphonuclear cell percentage in cerebrospinal fluid have different kinetics in aseptic vs. bacterial meningitis.     

Inadvertent intracranial insertion of a soft rubber tube in a patient with Treacher-Collins syndrome: case report and review of literature

Case report An inadvertent insertion of a soft rubber tube into the intracranial compartment in a patient with Treacher-Collins syndrome is reported. The neonate was delivered vaginally at 34 weeks gestation, had bilateral choanal atresia and presented with apnea at birth. An emergency orotracheal intubation was performed, and ventilatory support was instituted. Computed tomography scan of the facial bones showed a bilateral bony choanal atresia, with a thick posterior nasal septum, and complete absence of maxillary sinuses, external auditory canal, middle ear and ossicles, and roof of ethmoid bone. There was a central cleft in the frontoethmoidal area. The inner ear and the semicircular canals were present. The patient underwent successful transpalatal repair with nasal stenting. During surgery, two unsuccessful attempts to negotiate a soft rubber tube through the occluded choana were made. Postoperative magnetic resonance imaging revealed a linear hemorrhagic tract created by the soft rubber tube coursing through the anteroinferior basal frontal lobe, thalamus, basal ganglia and terminating in the occipital lobe. The patient developed bacterial meningitis, communicating hydrocephalus followed by cerebrospinal fluid rhinorrhea. A transcranial repair of the anterior cranial fossa was performed followed by ventriculoperitoneal shunt insertion. Subsequently, he underwent shunt revision for suspected shunt malfunction and developed peritoneal abscess for which the shunt was exteriorized and later removed. An endoscopic third ventriculostomy was performed, and the patient is recovering well. Discussion The potential implications of an inadvertent foreign body penetration into the intracranial compartment in such a rare event are discussed and the relevant literature is briefly reviewed.