Multiple Intracranial Aneurysms from Coccidioidal Meningitis: Case Report Featuring Aneurysm Formation and Spontaneous Thrombosis with Literature Review.

BACKGROUND: Coccidioidal meningitis can progress to vasculitis with aneurysm formation. Although aneurysmogenesis is rare, it carries exceptionally high mortality. Except in one instance, prior case reports have documented universally fatal consequences. CASE DESCRIPTION: A 26-year-old man developed disseminated coccidioidomycosis with formation of multiple aneurysms throughout the anterior intracranial vasculature bilaterally. This report is unique in that it chronicles the formation and subsequent spontaneous thrombosis of several aneurysms over a 4-week period. In total 10 aneurysms were documented in the same patient-the highest reported to date. The patient was eventually discharged from the hospital for what has heretofore been a universally fatal disease process. Neurologic examination and vascular imaging 1 month after discharge demonstrated stable findings. CONCLUSIONS: Coccidioidal aneurysms carry a high mortality. The mainstay of therapy remains lifelong triazole antifungal therapy with the addition of liposomal amphotericin in cases of treatment failure. Steroid use is controversial but should be considered whenever there is vascular involvement. Although watchful waiting is reasonable in light of the possibility of spontaneous thrombosis with medical management, dynamic changes in aneurysm size or configuration should prompt timely endovascular or operative interventions.

Long-term clinical and radiographic stability after clip reconstruction of a midbasilar dissecting mycotic aneurysm.

OBJECTIVE: Mycotic aneurysm rupture has been described as a rare, universally fatal complication of central nervous system Coccidioides immitis meningitis. Recently, however, we reported the successful surgical management of a midbasilar dissecting mycotic aneurysm related to C. immitis meningitis in a 24-year-old pregnant woman with acquired immune deficiency syndrome. METHODS: A 24-year-old pregnant woman with known acquired immune deficiency syndrome and a history of C. immitis meningitis presented to our institution with subarachnoid and intraventricular hemorrhage. Diagnostic cerebral angiography revealed a dissecting, midbasilar aneurysm involving the right anterior inferior cerebellar artery and causing stenosis of the proximal basilar artery. Through a retrosigmoid craniotomy, clip reconstruction of the basilar artery under hypothermic circulatory arrest was performed. The patient was neurologically intact at discharge (postoperative day 19) with the exception of mild disconjugate gaze and headache. RESULTS: At her 53-month follow-up, the patient was neurologically intact with only mild disconjugate gaze on upgaze. There was no evidence of local aneurysm recurrence or regional aneurysm formation, and the basilar artery was patent. Her C. immitis meningitis was well controlled. The patient is maintained on oral highly active antiretroviral therapy and an antifungal regimen. CONCLUSIONS: Our patient represents the only survivor of a C. immitis mycotic aneurysm rupture. In her case, long-term clinical and radiographic stability were achieved through a vigilant multidisciplinary approach.

[Invasive aspergillosis with extrapulmonary involvement: pathogenesis, clinical characteristics and prognosis]. / Aspergilosis invasora con afectación extrapulmonar: patogenia, características clínicas y pronóstico.

BACKGROUND: The incidence of invasive aspergillosis has increased worldwide. Information regarding the clinical characteristics of patients with extrapulmonary involvement is scarce. OBJECTIVE: We aimed to describe the pathogenesis, characteristics and outcomes of patients with invasive aspergillosis and extrapulmonary disease. METHODS: A retrospective study conducted in a university hospital in Barcelona, Spain (1995-2011). RESULTS: A total of 12 cases of invasive aspergillosis and extrapulmonary involvement were found. The most common clinical manifestations were invasive sinusitis, early postoperative prosthetic valve endocarditis, fungaemia, postoperative meningitis, multiple brain abscesses and lumbar spondylitis with epidural abscess. Sinusitis occurred frequently in patients without immunosuppression and had invasive brain involvement in one case. Endocarditis was associated with multiple septic metastases. Concomitant lung involvement was documented in 5 cases. The strains isolated were Aspergillus fumigatus (5), Aspergillus flavus (3), and Aspergillus niger (2). The species of Aspergillus was not established for 3 isolates. All patients were treated with antifungals and surgery was performed in 8 cases. Outcome was related with the source of infection; all patients with invasive sinusitis survived, while the remaining patients had a high mortality rate (88%). CONCLUSIONS: Invasive aspergillosis with extrapulmonary involvement is rare. The most common presentation is invasive sinusitis, which has a lower mortality. Other clinical forms with extrapulmonary involvement were associated with severe immunosuppression or previous surgery, and had a poor outcome.

Terminal ventriculostomy as an adjuvant treatment of complex syringomyelia: a case report and review of the literature.

Complex syringomyelia is multifactorial, and treatment strategies are highly individualized. In refractory cases, sectioning of the filum terminale, also known as terminal ventriculostomy, has been described as a potential adjuvant treatment to alleviate syrinx progression. A 10-year-old boy with a history of arachnoiditis presented with complex syringomyelia, progressive lower extremity motor weakness, and spasticity. Previously, he had failed spinal cord detethering and direct syrinx shunting. Imaging studies demonstrated a holocord syrinx extending to the level of his conus medullaris and into the filum terminale. The patient underwent an uncomplicated lumbar laminectomy and transection of the filum terminale. Operative pathologic specimens demonstrated a dilated central canal within the filum. Postoperative imaging demonstrated significant reduction in the diameter of the syrinx. At follow-up, the patient's motor symptoms had improved. Terminal ventriculostomy may be a useful adjuvant in treating caudally placed syringes refractory to other treatments. This procedure carries low neurological risk and involves no hardware implantation. In select cases, terminal ventriculostomy may help preserve neurological function in the face of otherwise progressive syringomyelia.

[Coccidioidomycosis revealed by specific cutaneous lesions occurring after placement of a ventricular cardiac shunt for chronic meningitis]. / Coccidioïdomycose révélée par des lésions cutanées spécifiques survenues après une dérivation ventriculo-cardiaque pour méningite chronique.

INTRODUCTION: Coccidioidomycosis is a deep mycosis rare in France. CASE: We report the case of a patient with disseminated coccidioidomycosis and meningitis after travel to California. It initially suggested tuberculosis. The ensuing hydrocephalus required a ventricular peritoneal shunt, replaced by a ventricular cardiac shunt after an obstruction occurred. Diagnosis was made 18 months later when the patient developed specific plantar ulcerations. Dramatic improvement was observed under fluconazole and maintained after prolonged follow-up. COMMENTS: Coccidioidomycosis should be considered in any patient who presents with meningitis suggestive of tuberculosis who has traveled to endemic zones. Cutaneous dissemination secondary to a ventricular cardiac shunt is rare.

Multiple Aspergillus brain abscesses in immuno-competent patient with severe cranio-facial trauma.

Aspergillosis of the central nervous system (CNS) is a rare, but well described disease in immuno-competent patients. We present a 65-year-old patient who developed neuro-aspergillosis 10 months after severe cranio-facial trauma (Le Fort III). He was treated successfully with surgery including stereotactic drainage and, with Amphotericin B, Liposomal Amphotericin B, and Itraconazol.

Intracranial aspergillosis involving the internal auditory canal and inner ear in an immunocompetent patient.

We report the MR imaging findings in a case of intracranial aspergillosis involving the internal auditory canal (IAC) and inner ear in an immunocompetent patient. The presence of rim enhancement of the vestibulocochlear nerve, abnormal signal intensity involving the labyrinth, and adjacent meningeal enhancement might help clinicians to make a correct diagnosis in patients with a mass in the IAC and previous history of ear surgery.

Craniocerebral aspergillosis of sinonasal origin in immunocompetent patients: clinical spectrum and outcome in 25 cases.

OBJECTIVE: Craniocerebral aspergillosis of sinonasal origin has been reported mainly in immunocompromised patients with high mortality, and it has been described very infrequently in immunocompetent hosts. This retrospective study focuses on clinical outcome in relation to anatomic locations of invasive aspergillosis of sinonasal origin in immunocompetent patients with emphasis on our preliminary experience with use of preoperative orally administered itraconazole. METHODS: Medical records of patients treated in two tertiary care hospitals from 1991 to 2003 were reviewed retrospectively. All patients had radiological evidence of disease in the paranasal sinuses with or without intracranial extension. The study cohort was divided into three types on the basis of area of involvement revealed by computed tomographic or magnetic resonance imaging scans of brain. All patients underwent surgical intervention and treatment with antifungal therapy. Preoperative orally administered itraconazole therapy was used in four patients on the basis of neuroradiological features. Clinical outcome was assessed with the Glasgow Outcome Scale, and univariate analysis of prognostic factors was performed with 95% confidence interval (P = 0.05). RESULTS: Mean patient age was 36.5 years (range, 14-74 yr) with a male preponderance (male-to-female ratio, 23:2). Nasal stuffiness (n = 13), headaches (n = 10), proptosis (n = 9), and nasal discharges (n = 7) were major presenting clinical features. Radiological data were obtained by computed tomographic (n = 25) and magnetic resonance imaging (n = 20) scans of the brain, and diagnoses were established by histopathological analysis (n = 20) or/and fungal cultures (n = 15). Preoperative orally administered itraconazole was given in four patients with intracerebral aspergillosis. Overall mortality was 28% and was highest in patients with Type 1 aspergillosis (66.7%). Type 3 aspergillosis and use of preoperative itraconazole remained statistically significant prognostic factors. CONCLUSION: Craniocerebral aspergillosis in immunocompetent hosts has three patterns of presentation that seem to correlate with clinical outcomes. Intracerebral aspergillosis (Type 1) is associated with the worst clinical outcome. Patients with orbital and cranial base aspergillosis (Type 3) had good recovery. Intracranial extradural aspergillosis (Type 2) remained intermediate on the Glasgow Outcome Scale. Preoperative orally administered itraconazole therapy may improve clinical outcome in patients with intracerebral aspergillosis. Prospective clinical studies are required to make firm clinical therapeutic recommendations.

Cerebral phaeohyphomycosis caused by a dematiaceous scopulariopsis species.

This report describes a rapidly fatal case of cerebral phaeohyphomycosis in a 33-year-old immunocompetent male. The infection presented as a single large lesion in the deep white matter of one temporal lobe, which was then removed surgically. Histologic features observed in the lobectomy specimen were characterized by perivascular sleeves of mononuclear cells accompanied by hemorrhages. These were reminiscent of acute hemorrhagic leukoencephalitis except for the presence of rare fungal organisms and sparse multinucleated giant cells similar to those occurring in AIDS. During the four days following surgery, a large focus of cerebritis with massive invasion of fungi developed in each centrum semiovale around the ventriculostomy sites. Fungal culture of the brain obtained at autopsy grew an organism consistent with a Scopulariopsis species.

Hydrocephalus in coccidioidal meningitis: case report and review of the literature.

OBJECTIVE AND IMPORTANCE: Coccidioidomycosis was once confined to the southwest United States and northern Mexico. It has become a larger concern because of the concentration of military bases in these areas, the increasing mobility of populations, and the rising population of immunocompromised persons. Outside endemic areas, the diagnosis is rarely considered. Patients with coccidioidomycosis may develop occult basilar meningitis progressing to communicating hydrocephalus and death. CLINICAL PRESENTATION: A 60-year-old white man presented with a 1-month history of vertigo, falls, and vomiting. Computed tomography of the head revealed mild hydrocephalus. Lumbar puncture results were remarkable for 1065 mg/dl protein; acid-fast bacillus stain, Gram's stain, and culture results were negative. Postgadolinium magnetic resonance imaging demonstrated enhancement of basilar and cervical meninges, suggesting inflammation, and communicating hydrocephalus. For 48 hours, the patient's level of consciousness decreased progressively. INTERVENTION: A ventriculoperitoneal shunt was placed, and antifungal agents were initiated on an emergent basis. CONCLUSION: Coccidioidomycosis should be considered in the differential diagnosis of occult basilar meningitis. The diagnosis is established by the discovery of a high (>1:2) titer of complement-fixing antibody in the cerebrospinal fluid. Communicating hydrocephalus is a common complication of untreated coccidioidal meningitis, and it may develop during appropriate treatment (oral fluconazole, 200-400 mg/d, continued indefinitely). Patients with hydrocephalus and evidence of increased intracranial pressure require a shunt.

Aspergillosis in the frontal sinus with intracranial extension: report of 1 case and analysis of the literature

The authors report a case of the 53 year old man carrier of aspergillosis in the both ethmoid and frontal sinus without association with the HIV virus. Aspergillosis in its focal and disseminated form is frequently associated with immune dysfunction, especially with patients carriers of the acquired immunodeficiency syndrome (AIDS), and it constitutes, when present in the central nervous system (CNS), a serious infection with difficult control and high morbidity index. The habitual treatment for these cases consists of the resection of the necrotic tissue and, so much as possible, of the lesions and also the administration and lingering use of liposomal amphotericin B.A lot of times, the invasion of the orbit and of the paranasal sinus can justify an aggressive conduct with extensive cranium-facial surgeries. The patient in subject was submitted to the partial resection of the lesion, which invaded the base of the anterior floor and the base of the frontal lobe to the orbitary cone and mesial face of the orbit. There was as postoperative complication, a liquoric fistula of difficult treatment due to the fragility of the dura mater in the base of the cranium infiltrated by the lesion. It received, for about 12 months, amphotericin in high doses and he comes maintaining himself stable of the clinical-neurological point of view. The authors discuss the etiopathogeny, the diagram of flow of the diagnostic and therapeutics conducts and they point out the need to include aspeergillosis of the SNC as disease of badly prognostic, deserving aggressive therapeutics in its initial phase

Cerebral candidiasis presenting as a mass lesion.

The authors report of a case of pseudotumour caused by Candida species without evidence of any underlying disease. No portal of entry of the infection was found. Total removal followed by treatment with flucanazole resulted in a favorable outcome. We discuss the differential diagnosis of a huge calcified intracranial mass lesion without any soft tissue component.

Imaging in phaeohyphomycosis of the brain: case report.

A case of multiple, conglomerated brain abscesses due to Xylohypha bantiana, with unique imaging features, is presented. This is the first culture-proven case of brain infection due to this fungus in the neuroimaging literature.

Paracoccidioidomycosis of the central nervous system: CT findings.

A retrospective analysis of six cases of central nervous system paracoccidioidomycosis, all but one proven by biopsy and surgery, was carried out to study the CT and clinical data and pathological correlation. Most of the patients were from the country. Headache, vomiting, seizures and hemiparesis were the most frequent symptoms. Papilloedema was present in four patients with raised intracranial pressure. Five patients had chronic lung disease and two with advanced systemic disease, skin and mucous membrane lesions were also observed. The neurological disturbance was sometimes the presenting features and the diagnosis was discovered incidentally after surgery. Both solitary and multiple parenchymal lesions were observed and the cerebral hemispheres were more commonly involved in four patients. Local meningeal involvement was observed in one with a single cortical granuloma. We emphasise the usefulness of CT, showing a rounded or lobulated mass with an isodense or radiolucent centre after contrast enhancement, surrounded by an irregular wall of varying thickness. There was always moderate oedema, extending peripherally. Other infections or neoplastic diseases may present similar findings. Preoperative diagnosis should rest on integration of clinical data, chest films, laboratory and neuroimaging studies.

Intracranial mycotic infections in neurosurgical practice.

Intracranial mycotic infections requiring neurosurgical intervention are being diagnosed more frequently. This study is a review of 17 cases of intracranial mycotic infections that were treated in a neurosurgical unit in Saudi Arabia over an 8-year period. A primary focus of infection was identified in 41% of patients while 18% of patients had a predisposing factor. Forty-seven percent of patients presented with a brain abscess (solitary 29%, multiple 18%) while 35% had a granuloma. 18% meningitis and ventriculitis and 12% hydrocephalus. The Aspergillus species and Ramichloridium machenziei were the commonest pathogens. Following the appropriate surgical and antimicrobial treatment, the mortality rate was 41% and there was evidence of residual disease at follow-up in 18%. The reason for a fatal outcome was failure to consider a fungal aetiology and to obtain a tissue diagnosis early-because of late referral (2 cases), as well as failure to respond to antimycotic therapy (4 cases) and rupture of the internal carotid artery due to Aspergillus arteritis (one case). It is concluded that an early tissue diagnosis is crucial in the management of intracranial mycotic infection so that the appropriate surgical and antimycotic treatment can be started early.

Rhodotorula spp. fungemia in an immunocompromised boy after neurosurgery successfully treated with miconazole and 5-flucytosine: case report and review of the literature.

A case of Rhodotorula fungemia in a 13-year-old boy after neurosurgery successfully treated with miconazole and 5-flucytosine is reported. Intravascular catheter insertion, broad-spectrum anti-miocrobials, surgery, and immunosuppression are the main risk factors for fungemia.

[A case of aspergillosis presenting marked granulomatous pachymeningitis].

The authors report a rare case of intracranial aspergillosis presenting marked granulomatous pachymeningitis. A 58-year-old male who had a three-year history of diabetes and chronic bronchitis was referred to us because of progressive left hemiparesis and dysarthria. Postcontrast CT scan showed a ring-enhancing lesion with marked perifocal edema in the right parietal lobe, and right subdural enhancing mass. MR image revealed hypertrophic dura mater in the right convexity. On June 9, 1992, partial removal of the intra-axial cystic mass and granulomatous dura mater was performed. A number of characteristic aspergillus hyphae were recognized in the resected cyst and granulomatous dura mater. Postoperatively, the patient was treated with amphotericin-B and fluconazole. But granulomatous pachymeningitis became progressively enlarged and eventually created a large mass effect again. On January 23, 1993, the patient died of pneumonia. Cerebral aspergillosis is getting common but preoperative diagnosis is still difficult because of its causing several clinical features such as brain abscess, granuloma, intracerebral hemorrhage, cerebral infarction, meningitis, and encephalitis. Several comments were made about the pathogenesis of these features, and the necessity of early diagnosis and treatment was emphasized.

Aspergillosis infection in neurosurgical practice.

Fungal infections including those due to aspergillus are rare in neurosurgical practice despite their possible inclusion in many differential diagnoses. Recently, these diseases have been diagnosed with increasing frequency, principally as opportunistic infections in patients undergoing treatment for diseases resulting in immune compromise. The epidemiology is poorly understood as mycoses are not notifiable diseases. We have recently been involved in the care of seven patients with aspergillosis between 1988 and 1991. Its presentation, with abscess formation, granulomas, the rhinocerebral form, meningitis, hydrocephalus and vascular involvement, is varied. The majority of cases were seen in immunocompromised patients following haematogenous dissemination from a pulmonary or gastro-intestinal focus. Direct spread from sinus infection has also been seen. The prognosis is poor despite modern antifungal treatment, which in part reflects the primary underlying condition.