Rheumatoid meningitis presenting with a stroke-like attack treated with recombinant tissue plasminogen activator: a case presentation.

BACKGROUND: Rheumatoid meningitis presenting with a stroke-like attack (RMSA) is a rare manifestation of rheumatoid arthritis (RA). When the patients arrive within the time-window for recombinant tissue plasminogen activator (rt-PA) infusion therapy, no diagnostic protocol has been established. CASE PRESENTATION: A 55-year-old woman was brought by ambulance to our hospital with complaints of sudden-onset dysarthria and left arm numbness. The National Institutes of Health Stroke Scale (NIHSS) score was 5, and the Alberta Stroke Program Early CT Score was 8. She was diagnosed with acute embolic stroke. At 4 h, 6 min after onset, intravenous administration of rt-PA (alteplase, 0.6 mg/kg) was started. Her neurological deficits improved rapidly, and her NIHSS score was 1. Brain MRI was then performed. There was no hemorrhagic transformation, but the MRI findings were not compatible with ischemic stroke. She had a past history of RA diagnosed 6 months earlier, and she had been treated with methotrexate (10 mg daily). She was diagnosed with RMSA, and continuous infusion of methylprednisolone 1000 mg daily was started for 3 days. The high signal intensity on the FLAIR image disappeared. CONCLUSION: CT-based decision-making for rt-PA injection is reasonable, but MRI is needed for the early diagnosis of RMSA. In this case, it is particularly important that neither adverse events nor bleeding complications were observed, suggesting the safety of CT-based thrombolytic therapy in RMSA.

Evaluation of pituitary function after infectious meningitis in childhood.

BACKGROUND: A number of studies of adults have shown that pituitary deficiencies can develop in a considerable proportion of subjects during the acute phase of meningitis or years after the infection has disappeared. The results of the very few studies of the impact of pediatric meningitis on hypothalamic-pituitary function are conflicting. METHODS: In order to determine the incidence of pituitary dysfunction in children with central nervous system infection, we evaluated pituitary function and anthropometric parameters in 19 children with meningitis of different etiologies (15 males; mean age ± standard deviation [SD] at pituitary evaluation, 5.9 ± 4.0 years; mean time from the acute event ± SD, 18 ± 10 months). RESULTS: All of the subjects had a normal stature and growth velocity for their age and gender, and none of them was obese. On the basis of Tanner's reference charts, 17 subjects (13 boys and all four girls) were pre-pubertal; two boys were in Tanner stage 2. None of the subjects had central hypothyroidism. All of the patients had normal serum of insulin growth factor (IGF)-I and prolactin. Their sex steroid and gonadotropin levels were concordant with their age and pubertal status. Early morning urine osmolality and serum electrolyte levels showed no signs of diabetes insipidus. All of the patients had normal plasma adrenocorticotropic hormone (ACTH) levels. Peak cortisol responses to the standard dose Synacthen test (SDST) were normal in all cases. CONCLUSIONS: The results showed that hypopituitarism following infectious meningitis appears to be infrequent in childhood and children's pituitary glands seem to be less vulnerable to damage than those of adults.

Extensive, circumferential, epidural spinal cord compression by IgG4-related pachymeningitis.

A rare case of IgG4-related pachymeningitis is presented. The manuscript describes the multidisciplinary treatment of a rare entity in an exceptional location, prompting emergent surgical treatment. However, the correct intraoperative hypothesis led to a minimal surgical approach with fewer risks and good disease control. Finding a balance between maximising treatment outcome and minimising risks is often difficult for clinicians worldwide who encounter the same clinical dilemma. The known cases are summarised, as are radiological characteristics and common treatments.

Association of meningitis and clival canal defect: case illustration, management, and systematic review of the literature.

OBJECTIVE: Improper embryological development of the clivus, a bony structure that comprises part of the skull base, can lead to a clival canal defect. Previously thought to be a benign condition, clival canals have been reported to be associated with meningitis and meningoceles. In this review, the authors sought to present an unpublished case of a patient with a clival canal defect associated with meningitis and to evaluate all other reported cases. METHODS: In October 2020, a search of PubMed, Web of Science, and Scopus was conducted to identify all cases of clival canals reported from January 1, 1980, through October 31, 2020. RESULTS: Including the case presented herein, 13 cases of clival canals, 11 in children (84.6%) and 2 in adults (15.4%), have been identified. Of the pediatric patients, 5 (45.5%) had an associated meningocele, and 8 (72.7%) had meningitis. Nine of the 13 patients (69.2%) had defects that were treated surgically, 5 (38.5%) by a transnasal approach and 4 (30.8%) by a transoral approach. Two patients (15.4%) were treated with drainage and antibiotics, 1 patient (7.7%) was treated solely with antibiotics, and 1 patient (7.7%) was not treated. In the literature review, 8 reports of clival canals were found to be associated with meningitis, further contributing to the notion that the clival canal may be an overlooked source of recurrent infection. In several of these cases, surgical repair of the lesion was curative, thus preventing continued episodes of meningitis. CONCLUSIONS: When a patient has recurrent meningitis with no clear cause, taking a closer look at clival anatomy is recommended. In addition, if a clival canal defect has been identified, surgical repair should be considered a safe and effective primary treatment option.

Plasma Ablation-Assisted Endoscopic Endonasal Transpterygoid Approach to Sphenoid Lateral Recess Cerebrospinal Fluid Leaks: Technique and Outcome.

BACKGROUND: Management of sphenoid lateral recess (SLR) cerebrospinal fluid (CSF) leaks present a challenge because of the location and requiring complete visualization of the defect for a successful repair. The endoscopic endonasal transpterygoid approach (EETPA) is considered the gold standard in addressing these defects. We lay out our experience in implementing this approach with plasma ablation. METHODS: This is a case series of 11 diagnosed patients of SLR CSF leaks who underwent plasma ablation-assisted EETPA repair by a single surgeon between 2011 and 2020 at our institution. Outcomes in terms of surgical field grade on the Wormald 11-point grading scale, postoperative complications, healing on nasal endoscopy and imaging, and surgical success rate were assessed. RESULTS: The etiology was spontaneous leak in 10 (90.9%) patients and secondary to temporal lobe abscess and/or meningitis in one (9.09%). Three (27%) patients were previously operated elsewhere by the transsphenoidal route, which we reoperated by this technique. As per Wormald grading, grade 1 field in 3 (27.27%), grade 2 in 6 (54.5%), and grade 3 in 2 cases (18.18%) were noted. Complications occurred in 3 patients (27%) in the form of dry eye (9%), meningitis (9%), and transient CSF rhinorrhea in the immediate postoperative period (9%). Repair sites were well healed on follow-up nasal endoscopy and imaging. The surgical success rate was 100%. CONCLUSIONS: Plasma ablation-assisted EETPA allows for a uninostril approach to the SLR, easy accessibility, and better visualization with a bloodless field, which allows appropriate repair, thus minimizing complications and preventing recurrence.

Nodular Pachymeningitis Associated With Relapsing Polychondritis and Crohn Disease Responsive to Adalimumab and Prednisone.

OBJECTIVES: To review the previous literature on the associations of pachymeningitis with Crohn disease (CD) and relapsing polychondritis (RP) and to describe a new case occurring in association with both in addition to highlighting its positive response to steroid and adalimumab treatment. METHODS: We review the patient's clinical presentation, diagnostic workup (serum and CSF testing), and MRI findings in detail and chronicle the response of the pachymeningitis to intensive immunotherapy. We contrast this case against previous reports of pachymeningitis occurring in association with RP and inflammatory bowel disease that were found on PubMed. RESULTS: Only 2 cases of ulcerative colitis and 5 cases of RP were found in association with pachymeningitis; there were no cases in association with CD. Our patient presented with symptoms isolated to a steroid-responsive headache in the setting of normal neurologic and rheumatologic examinations. Her preceding history was notable for long-standing CD and increasingly active symptoms referable to RP. Focal nodular pachymeningitis was seen overlying the left hemisphere on brain MRI. An extensive serum and CSF workup and body fluorodeoxyglucose-PET scan failed to identify an alternative etiology beyond her underlying autoimmune inflammatory disorders. After adding prednisone and adalimumab to her preexisting treatment of methotrexate, she responded dramatically both clinically and radiographically. CONCLUSIONS: Although exceptionally rare, pachymeningitis may occur as a neuroinflammatory complication of CD and RP.

[Otogenic meningitis and bilateral spontaneous dehiscences of the lateral skull base. Two case reports]. / Otogene Meningitis bei bilateralen knöchernen Dehiszenzen der Laterobasis. Zwei Fallberichte.

Meningitis in the case of spontaneous dehiscences of the lateral skull base is rare. We report on two patients with otogenic meningitis. High-resolution CT (HR-CT) showed bilateral dehiscences at the lateral skull base in both cases. Infection along the skull base was assumed. In the first case sufficient repair was achieved using a multilayered technique, while in the second case conservative treatment on an intensive care unit was successful. Conservative therapy can prove successful in exceptional cases.

Nontraumatic headaches in the Emergency Department: a systematic approach to diagnosis and controversies in two "big ticket" entities.

Headache is a very common presenting complaint in the Emergency Department (ED). While the vast majority of these have benign or self-limited etiologies, there are several life or organ threatening causes that must be excluded. This paper describes a systematic approach to the history and physical examination in these patients. Evidence-based recommendations for which patients should receive imaging in the ED are reviewed. Current diagnostic approaches and controversies in meningitis and subarachnoid hemorrhage are discussed.

Neuro-Sweet disease in a Japanese woman with Sjögren's syndrome.

Sweet disease (SD) is a multisystem inflammatory disorder characterised by fever, cutaneous erythematous plaques and aseptic neutrophilic infiltration of various organs. Neuro-Sweet disease (NSD) is a known rare central nervous system complication of SD. We describe a case of a 39-year-old Japanese woman who was diagnosed as NSD associated with Sjögren's syndrome. She was successfully treated with systemic corticosteroid therapy.

Herpes zoster oticus with meningitis masquerading as malignant otitis externa.

This is a case of an 85-year-old woman whom was admitted with otalgia and an abducens nerve palsy alongside a Pseudomonas otitis externa; she was presumed to have malignant otitis externa. However, despite optimum treatment and resolution of her otitis externa, she went on to develop an ipsilateral facial nerve palsy and sensorineural hearing loss. After further investigation, it was discovered that varicella-zoster meningitis was causing her polyneuropathy. She eventually responded to antivirals and steroids and, at follow-up, her sixth and seventh cranial nerve palsies had completely resolved, though a hearing deficit remained. This case highlights the importance of keeping a diagnosis under review, with the help of the multidisciplinary team, when the clinical course is not progressing as expected.

Rotating computed tomographic movie for evaluating partially ossified cochlea.

OBJECTIVE: To describe our newly devised method of viewing intracochlear ossification for the purpose of minimizing bone drilling during cochlear implantation for partially ossified cochleas and to evaluate its usefulness. STUDY DESIGN: Descriptive study that compares 1) conventional computed tomographic (CT) films with the newly devised CT movie and 2) preoperative evaluation using the CT movie with the findings during surgery. SETTING: University Hospital. PATIENTS: Four cochlear implantees with partial ossification of cochlea due to meningitis. INTERVENTION: Diagnostic. MAIN OUTCOME MEASURES: Reconstruction of high-resolution CT images of the temporal bone was performed using a multiplanar reformat software. By rotating the cochlear cross plane from the round window niche in the direction of the inferior segment by 5 degrees, 72 images were obtained for 360-degree rotation, which were converted to a movie using QuickTime Pro software. The preoperative evaluation and intraoperative findings were compared. RESULTS: The advantage of CT movie over sequential CT films was facility in understanding the 3-dimensional space in the cochlea. Evaluation of the extent of the ossified region on CT movie corresponded to the intraoperative findings in all 4 patients. In 1 patient, the CT movie corrected an erroneous evaluation based on conventional CT films. However, additional drilling over the extent of ossification was necessitated in another patient because of scar tissue development. CONCLUSION: Computed tomographic movie is considered an innovative new method for preoperative evaluation of ossified cochleas and is able to reduce human errors. However, additional drilling beyond ossified part can become necessary during surgery.

[Postmeningitis deafness in young children: action warranted before obliteration of the cochlea]. / Doofheid na bacteriële meningitis bij jonge kinderen: ingrijpen vóór de cochlea is geoblitereerd.

Meningitis may cause inflammation of the cochlea, which may result in deafness and also in rapid obliteration ofthe cochlea with fibrous tissue or even ossification, conditions that obstruct the placement ofa cochlear implant. In the first of two cases of postmeningitis deafness, in a boy aged 6 months and a girl aged 1 year and 9 months, ignorance about the time of audiological follow-up threatened the options for restoration of hearing. In the other case, a long diagnostic programme and an unsuccessful attempt at cochlear implantation caused a long delay in optimal restoration of hearing. Both cases illustrate the difficulties in connection with postmeningitis deafness in relation to the option of a cochlear implant operation. To increase the chances of a successful implantation, the time span between meningitis and audiological and radiological follow-up must be short. Auditory brain stem responses (ABR) and MRI are the keystones of the work-up.

[Difficulty in diagnosing pediatric tuberculosis]. / Tubercolosi pediatrica una diagnosi difficile.

Tuberculosis (TB) in children is an important warning sign in a community, as it could signal recent infection of a cavitary form in an adult. Thus, while early diagnosis is crucial for effective treatment in children, it is also imperative for the control of tuberculosis at the public health level since it allows rapid identification of contagious adult cases. Here we report four cases of difficult and delayed diagnosis of TB in children. From this experience we highlight the need for an extensive medical history of the patient during diagnostic work-up. This includes: the positive history for contact with infected adults, especially for immigrant children; exclusion of TB diagnosis for persistent respiratory symptoms (2-3 weeks) after antibiotic therapy; and the need for high-definition CT scan when the radiological picture is not specific, especially for children under 5 years of age.

Spontaneous cerebrospinal fluid fistula in the clivus.

INTRODUCTION: Spontaneous cerebrospinal fluid (CSF) fistulas are infrequent and only 10 cases in the literature have been located in the clivus. We describe two new cases of CSF fistulas in this site and review the literature. CASE REPORT: The first patient was a 52-year-old woman referred to our centre for intermittent rhinorrhea that had been diagnosed after an episode of meningitis. The second case was a 69-year-old man who was visited for rhinorrhea of one-year duration; he also developed meningitis during the preoperative study. In both cases, the spontaneous CSF fistula was diagnosed by beta-2-transferrin testing, CT scan and MRI. We performed an endonasal endoscopic transsphenoidal approach and used free grafts and vascularized flaps to close the clival defect. Treatment was successful in both cases. DISCUSSION: The physiopathology of spontaneous CSF fistulas remains unknown. Possible explanations given to date in this location are pulsatility of the basilar artery, repeated Valsalva maneuvers and Marfan's disease, the two latter also related to CSF fistulas in other locations. Closure of a CSF leak towards the nasal cavity is mandatory due to potential complications. Our results support the endoscopic transsphenoidal approach using free grafts and/or pediculated flaps as a good alternative to open surgery.

Serum carnitine and acyl-carnitine in patients with meningitis due to tick-borne encephalitis virus infection.

BACKGROUND: Hard ticks are the main vectors of tick-borne encephalitis virus (TBEV). Free carnitine (FC) and acylcarnitines (AC) have the basic role in ß-oxidation as well as the modulation of immune and nervous system. Homeostasis of carnitines in the TBE patients was not studied so far. OBJECTIVES: This study aimed to evaluate FC and AC serum concentrations in patients with meningitis due to TBEV infection before and after 14 ± 3 days of treatment. MATERIAL AND METHODS: The study was performed in 14 patients aged 48 ± 29 years that were divided a posteriori (based on their FC level before and after treatment) into 2 subgroups: 1-8 and 9-14. Diagnosis was based on the neurological, serological and pleocytosis evaluation. RESULTS: The FC level in patients 1-8 before treatment (24.1 ± 8.1) was significantly lower than in patients post-treatment (34.4 ± 8.3), lower than in the control group (40.5 ± 7.6), and lower than in patients 9-14 before treatment (40.0 ± 13.5) but not lower than in the patients 9-14 after treatment (24.7 ± 7.3 µmol/L), respectively, p < 0.05. AC concentration in the patients 1-8 before treatment (4.7 ± 2.2) was apparently lower than in patients post-treatment (9.5 ± 3.9 µmol/L) but the values were not significantly different. In patients 9-14 before treatment the AC concentration (16.3 ± 12.6) was higher than in patients after treatment (5.3 ± 4.0 µmol/L), but the difference was not statistically significant. CONCLUSIONS: FC and AC homeostasis in circulation was disturbed in the patients with meningitis due to TBEV infection patients. The mean levels of FC and AC in 60% of the patients were below the normal range but normalized after treatment whereas in 40% of the patients they were near or at a normal range and significantly decreased after treatment. Explanation of this intriguing finding and its clinical significance is not easy without further studies.

Hypertrophic pachymeningitis and undifferentiated connective tissue disease: a case report and review of the literature.

A 63-year-old man with hypertrophic pachymeningitis (HP) and an undifferentiated connective tissue disease is described. Combined therapy with prednisolone and azathioprine improved his symptoms. The association between an undifferentiated connective tissue disease and HP is discussed.

Experiences from Auditory Brainstem Implantation (ABIs) in four paediatric patients.

INTRODUCTION: Indications for auditory brainstem implants (ABIs) have been widened from patients with neurofibromatosis type 2 (NF2) to paediatric patients with congenital cochlear malformations, cochlear nerve hypoplasia/aplasia, or cochlear ossification after meningitis. We present four ABI surgeries performed in children at Uppsala University Hospital in Sweden since 2009. METHODS: Three children were implanted with implants from Cochlear Ltd. (Lane Cove, Australia) and one child with an implant from MedEl GMBH (Innsbruck, Austria). A boy with Goldenhar syndrome was implanted with a Cochlear Nucleus ABI24M at age 2 years (patient 1). Another boy with CHARGE syndrome was implanted with a Cochlear Nucleus ABI541 at age 2.5 years (patient 2). Another boy with post-ossification meningitis was implanted with a Cochlear Nucleus ABI24M at age 4 years (patient 3). A girl with cochlear aplasia was implanted with a MedEl Synchrony ABI at age 3 years (patient 4). In patients 1, 2, and 3, the trans-labyrinthine approach was used, and in patient 4 the retro-sigmoid approach was used. RESULTS: Three of the four children benefited from their ABIs and use it full time. Two of the full time users had categories of auditory performance (CAP) score of 4 at their last follow up visit (6 and 2.5 years postoperative) which means they can discriminate consistently any combination of two of Ling's sounds. One child has not been fully evaluated yet, but is a full time user and had CAP 2 (responds to speech sounds) after 3 months of ABI use. No severe side or unpleasant stimulation effects have been observed so far. There was one case of immediate electrode migration and one case of implant device failure after 6.5 years. CONCLUSION: ABI should be considered as an option in the rehabilitation of children with similar diagnoses.

Overview of a public health approach to pediatric hearing impairment in the Pacific Islands.

BACKGROUND: Childhood hearing impairment is a significant cause of disability in developing countries. Otitis media and meningitis are leading infectious causes of preventable hearing loss in children. It is estimated that the Pacific Islands have among the greatest global burden of childhood hearing impairment due to infectious causes, and yet there is currently very little in the research literature on pediatric hearing disorders in this region. OBJECTIVES: (1) To review existing research literature on pediatric hearing impairment in the Pacific Islands, and (2) to present a public health approach to the development and improvement of childhood hearing services in the Pacific Islands. DATA: The primary tool was a comprehensive literature review. MEDLINE and ScienceDirect databases were searched for relevant journal articles. There was no limit on the date of publication. Any article reporting on hearing impairment in the Pacific Region was included. RESULTS: A total of 23 journal articles were found that satisfied the above inclusion criteria. The limited information available in the literature suggests that otitis media and vaccine-preventable infections are a significant cause of avoidable childhood hearing impairment in the Pacific Islands. Pediatric audiology services are limited in this region. Further research is required to develop effective public health programs that should reduce the burden of preventable childhood hearing loss in the Pacific Islands. CONCLUSIONS: There is limited information in the research literature on pediatric hearing impairment and audiology services in the Pacific Islands. Epidemiological data based on the WHO Ear and Hearing Disorders Survey Protocol are urgently needed, and the development of audiology services within the existing public and primary health care framework should reduce the burden of preventable hearing loss in the Pacific Islands.

[Loss of cranial-nerve function caused by early syphilitic meningitis: the comeback of a pre-war syndrome]. / Hersenzenuwuitval door vroege syfilitische meningitis: terugkeer van een vooroorlogs ziektebeeld.

Two men aged 39 and 38 who had had unprotected insertive and receptive anal sexual contact with men are presented: one had paralysis of the right half of his face and the other man had erythematous macules on the palms of his hands and the soles of his feet as well as partial alopecia, earache and progressive loss of hearing in his left ear. The latter one was also HIV-seropositive and on antiretroviral medication. Syphilitic meningitis was diagnosed in both men. The 2 patients recovered after being treated with intravenous benzyl penicillin. Syphilitic meningitis is a complication seen during the early stages of a syphilis infection. Since the introduction of penicillin it has become a rare disease. Early diagnosis is of importance since syphilitic meningitis has irreversible sequelae.