Invasive aspergillosis presenting with a painless complete ophthalmoplegia

Objective

We report on a patient who presented with complete ophthalmoplegia as a first symptom of fatal invasive aspergillosis.

Case report

We present the clinical course of an immunocompromised 68-year-old man with unclear painless loss of vision, ptosis and ophthalmoplegia of the right eye lasting for 10 days which was referred from an ophthalmological outpatient clinic. He had a history of kidney transplantation after renal failure, diabetes mellitus II, and coronary heart disease. He was on immunsuppressive therapy with tacrolimus, mycophenolate and prednisolone since renal transplantation. The initial cranial magnetic resonance imaging (MRI) was unremarkable. The additional thin section computed tomography (CT) of the paranasal sinuses was suspicious for bone erosion of the superior wall of the right sphenoidal sinus. Initial laboratory testing and cerebrospinal fluid were without pathological findings. When the patient showed signs of generalised sepsis he was transferred to the intensive care unit. Due to fulminant sepsis it was not possible to perform the planned biopsy. The patient died a few days later of cardio-respiratory insufficiency. The consecutive autopsy revealed invasive aspergillosis of paranasal sinuses with infiltration into the orbita and intracranial spreading into sinus cavernosus. Autopsy also showed aspergillus pneumonia.

Discussion

Invasive aspergillosis of the paranasal sinuses and the skull base is most often a lethal condition in immunocompromised patients. Therefore, rapid diagnostics applying radiological (CT and MRI) and surgical procedures (biopsy) and immediate ignition of antimycotic treatment can be life-saving.     

Cerebral aspergillosis as a cerebral vascular accident

Cerebral aspergillosis is one of the most common mycotic infections in the central nervous system causing different clinical features such as brain abscess, granuloma, meningitis, and encephalitis. Cerebral aspergillosis, however, may lead to a cerebral vascular accident such as intracranial hemorrhage or cerebral infarction. In this report, we present two patients with cerebral aspergillosis accompanied by intracranial hemorrhage. A total of 124 reported cases of cerebral aspergillosis are reviewed to ascertain the pathogenesis of the associated vascular lesion. The first patient was a 9-year-old girl, who developed drowsiness with a headache during the medical treatment for acute myelocytic leukemia. CT disclosed subarachnoid and intraventricular hemorrhage. The autopsy revealed that the aspergillus arteritis was the cause of repeated hemorrhage. The second patient was a 15-year-old boy with allergic purpura and renal failure, who suddenly developed a stupor with convulsive seizure. CT disclosed an intracerebral hemorrhage in the right parieto-occipital area. The patient gradually deteriorated and died in spite of the surgical removal of the hematoma. The autopsy revealed that the hemorrhage was caused by the aspergillus arteritis. Cerebral aspergillosis has two routes of infection to the central nervous system: hematogenous dissemination from the distant site (usually the lung) and direct extension from the contiguous site (usually the paranasal sinuses or orbit). The primary mechanism of neuropathology is different between these two types. Primary cerebral arteritis is most often seen in patients with the former type, whereas primary basal meningitis occurs in the latter. The incidence of clinico-pathological features is different between hematogenous dissemination type and direct extension type.(ABSTRACT TRUNCATED AT 250 WORDS)     

Aspergillus sinusitis complicated with meningitis and multiple cerebral infarctions in immunocompetent patient

This is a case of a 33-year-old male complaining of severe headache, neck pain, photophobia, vomiting and high-grade fever of several days. He had history of nasal polyp removal and recurrent sinusitis in the last 8 years. On examination: conscious with glasco coma scale (GCS) 15/15 and normal limbs strength but with positive Babinski sign. For further observation, he was admitted and full work-up was done. Even though full empirical antibiotics were started, there was no immediate improvement and he deteriorated dramatically developing ocular deficit, hydrocephalus and lower level of consciousness with multiple infarctions found at different areas in brain. After that point, a decompressive craniectomy was done, and multiple antibiotics and antifungal medications were prescribed. However, he deteriorated to GCS 3/15; cardiopulmonary resuscitations were not successful, as he demised next day. It shall be noted that aspergillosis can lead to difficult complications, so diagnosis and treatments should not be delayed.

Fungal infections of the paranasal sinuses are uncommon and usually occur in individuals who are immunocompromised. However, recently, the occurrence of fungal sinusitis has increased in the immunocompetent population. This case report is for young man who is none-smoker and none-alcoholic immunocompetent patient who complained of severe headache and neck pain of several days; he was exposed to a highly invasive aspergillus fungal infection that showed drastic invasive complications even with aggressive medical and surgical interventions.     

Relevant imaging findings of cerebral aspergillosis on MRI: a retrospective case-based study in immunocompromised patients

The aim of the study was to assess neuroimaging patterns of cerebral aspergillosis with magnetic resonance imaging (1.5 T). The clinical and imaging data of nine patients were reviewed. Patients were included in the study if the diagnosis of aspergillosis was confirmed by either biopsy, autopsy, aspergillus antigen determination and/or neuroradiological and clinical response to specific treatment. Four patients had single or multiple abscesses presenting as ring-enhancing lesions on T1-weighted images, hypointensity of the ring on T2-weighted MR images and low to high signal intensity on diffusion-weighted imaging. Four patients had single or multiple infarctions affecting all compartments of the brain with hyperintensities on T2-weighted images in three of four patients, irregular parenchymal contrast enhancement in all patients and hemorrhagic transformation of the infarcted parenchyma in one patient. Diffusion-weighted images were positive in all ischemic areas. One patient with paranasal sinusitis developed a mycotic aneurysm of the internal carotid artery. Cerebral aspergillosis presents three principal neuroimaging findings: areas consistent with infarction; ring lesions consistent with abscess formation following infarction; and dural or vascular infiltration originating from paranasal sinusitis or orbital infiltration. Recognition of these patterns in cerebral aspergillosis may lead to more timely and effective diagnosis and treatment.     

Localized invasive intracranial aspergillosis with multiple cranial nerve failure -- case report and review of the literature

Contrary to the more frequent hematogenously spread cerebral aspergillosis, localized invasive intracranial aspergillosis is a fungal infection that can also occur in patients who are not severely immunosuppressed. This illness can be effectively treated in some of these patients by early and rigorous therapy. Localized invasion of the fungus, generally from one of the nasal sinuses, causes intracranial growth mainly along the base of the skull and larger vessels,where fibrous, granulomatous tissue develops. This generally leads to damage of the cranial nerves (primarily I-VI) as well as localized pain syndromes. We report on the clinical course documented by MRI of a patient with localized invasive intracranial aspergillosis who had multiple failure of cranial nerves following surgery for an aspergilloma of the maxillary sinus. Clinical course, imaging findings, and treatment of the illness are discussed with a review of the relevant literature.     

Invasive aspergillosis in a patient with MELAS syndrome

Invasive infection with the opportunistic fungus Aspergillus fumigatus predominantly affects people with impaired cell mediated immunity. The case of a 31 year old woman with no identified cause for immunosuppression who presented with severe refractory aspergillosis of the paranasal sinuses is reported. She subsequently developed clinical and molecular evidence of mitochondrial encephalomyopathy with lactic acidosis and stroke-like events (MELAS) syndrome. It is proposed that MELAS syndrome may represent an unusual risk factor for the development of invasive aspergillosis and mechanisms are supported by which mitochondrial dysfunction may predispose to this.     

Aqueductal stenosis and hydrocephalus in an infant due to aspergillus infection

Aqueductal stenosis is a common cause of hydrocephalus during infancy. We report on an infant born with aplasia cutis congenita at the scalp vertex and hypoplastic left heart syndrome developing systemic aspergillosis after cardiac surgery. The infant died at the age of 76 days despite systemic antimycotic therapy with a combination of flucytosine and amphotericin B. Therapy started at post-operative day 17 and was also applied intrathecally. Post-mortem examination revealed meningitis, multiple brain aspergillomas and microabscesses with focal ependymitis, focal bronchopneumonia, and necrotizing enterocolitis. One of the brain aspergillomas was located close to the aqueduct causing an aqueductal stenosis and an obstructive hydrocephalus. Histologically, aspergillus hyphae could only be detected in the aspergilloma of the aqueduct. To the best of our knowledge, this is the first reported case of an aqueductal stenosis caused by an aspergilloma.     

Aspergillosis of the central nervous system

Aspergillosis of the central nervous system is rare. The spread to the CNS is usually from the lungs and uncommonly from paranasal sinuses. Four cases of CNS aspergillosis with spread of infection from the paranasal sinuses are described. Two patients had meningitis, one intracranial granuloma and one presented with subarachnoid haemorrhage resulting from a fungal mycotic aneurysm. Difficulties in establishing the diagnosis are discussed.     

Rapidly Progressive Rhino-orbito-cerebral Mucormycosis Complicated with Unilateral Internal Carotid Artery Occlusion: A Case Report

Rhinocerebral mucormycosis is an acute fulminant opportunistic fungal infection usually seen in diabetic or immunocompromised patients. The fungi that cause mucormycosis inoculate the nasal mucosa and may spread to the paranasal sinuses, orbit, and brain. Our patient initially presented with mild ethmoid sinusitis. At that time, brain MRI and contrast-enhanced MR angiography were grossly normal. However, aggravation of sinusitis with extension to the right orbit and anterior cranial fossa rapidly developed within two months. Moreover, an occlusion of the right internal carotid artery was combined. We report a case of a pathologically-proven rhino-orbital-cerebral mucormycosis with serial follow-up imaging for over one year.     

Embolic and thrombotic changes in numerous CNS vessels in the course of aspergillosis

A case of aspergillous encephalitis in a diabetic patient with acute sinusitis is presented. MRI investigation revealed characteristic changes in the brain and skull bones, including these of face and skull base. Despite a radical surgery of the sinuses and early treatment with Amphotericin B, the disease progressed rapidly and was fatal. Post-mortem neuropathological examination confirmed the diagnosis of encephalitis caused by aspergillosis.     

Combined transfrontal and endonasal endoscopic surgery of epidural abscess following frontal sinusitis. A case report

A large epidural abscess secondary to frontal sinusitis in a previously healthy 19-year-old man was successfully treated with a small eyebrow incision using combined transfrontal and endonasal endoscopic technique. The abscess was resolved with concomitant pneumatization of the paranasal sinuses. The present case illustrates the promising use of endoscopy in the merging fields of neurosurgery and otorhinolaryngology.     

Value of visually evoked potentials in lesions of the optic nerve caused by a mucocele

Mucoceles originating in accessory paranasal sinuses with orbit involvement are quite rare. The authors describe two cases of mucoceles, revealed by CT-Scan, the first in the ethmoid-sphenoidal-maxillary sinuses and the other in the ethmoid-frontal sinuses respectively. In both cases Visual Evoked Potentials were most important for assessing optic nerve involvement. They were also useful to check post surgical recovery. These case reports emphasize that early diagnosis of mucoceles involving the optic nerve is very important because prompt surgical treatment may lead to recovery of visual disturbances.     

Treatment of primary aspergilloma of the central nervous system in a diabetic immunocompetent patient with surgical resection and voriconazole: a case report and review of the literature

Fungal infections of the central nervous system (CNS) are uncommon and occur mainly in immunocompromised patients. We describe a case of central nervous system aspergilloma without any evidence of systemic or paranasal foci in a diabetic but otherwise immunocompetent 71-year-old female treated successfully with surgical resection and medical therapy with voriconazole. Magnetic resonance imaging (MRI) after 6 months of voriconazole showed improvement and no evidence of residual or recurrent disease. Given its good CNS penetration, voriconazole along with surgical resection appears to be promising in treatment of these infections. Our case also demonstrates the importance of surgical intervention in the diagnosis and management of these atypical cases.     

Epidural and pericranial abscesses complicating frontal sinusitis in a comatose child

Most complications of sinusitis have a significant risk of severe morbidity and possibly mortality. Therefore a keen awareness of the subtle signs and symptoms of bacterial spread beyond the paranasal sinuses must be maintained. This increased awareness is especially important in mentally retarded and psychomotor handicapped children because they can develop a rapidly fatal course despite the absence of any warning signs.     

Combined transfrontal and endonasal endoscopic surgery of epidural abscess following frontal sinusitis. A case report

Abstract

A large epidural abscess secondary to frontal sinusitis in a previously healthy 79-year-old man was successfully treated with a small eyebrow incision using combined transfrontal and endonasal endoscopictechnique. The abscess was resolved with concomitant pneumatization of the paranasal sinuses. Thepresent case illustrates the promising use of endoscopy in the merging fields of neurosurgery and otorhinolaryngology. [Neural Res 1999; 21: 229-232]     

Successful treatment of sellar aspergillus abscess

A sellar aspergillus abscess is a rare fungal infection of the central nervous system (CNS). A retrospective analysis of three patients with sellar aspergillus abscess was conducted from 2006 to 2008. Data were retrieved from patient records at our hospital. Clinical findings, pathological data and final outcomes were reviewed and analysed. All patients underwent transsphenoidal surgery with the operating microscope and histopathologic examination revealed aspergillosis in all cases. Postoperatively, all patients received medical treatment with voriconazole and caspofungin. During the 3-6-month follow-up period, the patients were symptom free with no recurrences. Therefore, sellar aspergillus abscess should be included in the differential diagnosis of a sellar mass. Early and correct diagnosis via surgery can improve the prognosis. A combination of surgical resection and antifungal therapy has a good outcome. The importance of early treatment for sellar aspergillus abscesses is emphasised.     

Bilateral optic neuritis in wegener granulomatosis.

A 32-year-old patient with recurrent sinusitis had severe visual loss from optic neuropathy. Imaging revealed severe bone destruction and soft tissue densities of the paranasal sinuses and enhancement of the dura of the frontal sinuses, optic canals, and superior orbital fissures bilaterally. Endoscopic sinusectomy with biopsy showed granulomatous vasculitis compatible with Wegener granulomatosis (WG). The patient was treated with intravenous and oral corticosteroids and oral cyclophosphamide that led to rapid and dramatic visual recovery. This case draws attention to the fact that optic neuritis may be an early inflammatory manifestation of WG and that rapid diagnosis and aggressive anti-inflammatory treatment is critical before inflammation of arteries leads to infarction and irreversible visual loss.     

Invasive aspergillosis of the sphenoid sinus and paralysis of the 6th nerve]

A case of aspergillosis of the sphenoid sinus manifested as an isolated sixth nerve palsy occurred in a 74-year old diabetic woman who had no complaints of headache or symptoms suggestive of sinusitis. A CT scan demonstrated a large mass occupying the sphenoid and ethmoid sinuses extending posteriorly to the clivus. There was a calcific density within the opacified sinus and bony erosion of the sphenoid walls and the sella turcica. The patient underwent a sublabial transseptal sphenoidotomy with removal of necrotic material and debridement of the surrounding tissue. Histologic examination revealed granulation tissue with chronic inflammatory cells and abundant dichotomously branching hyphae. Postoperatively the patient was given amphotericin B and 5-fluorocytosine. Three months later the sixth nerve palsy had completely cleared and the patient had no other complaint. Sphenoid sinus aspergillosis is a rare disease and may have variable clinical manifestations according to involvement of different structures located closely to the sinus. Our patient developed an isolated sixth nerve palsy which was at onset considered to be caused by diabetes. Computerized tomography scans disclosed abnormalities strongly indicative of invasive aspergillosis. It illustrates the need of appropriate work-up in cases of an isolated sixth nerve palsy even in patients with diabetes or other risk factors.     

Isolated central nervous system aspergillosis in the acquired immunodeficiency syndrome.

Aspergillus infection involving the central nervous system are unusual, but should be included in the differential diagnosis in patients with the acquired immunodeficiency syndrome and neurologic signs and symptoms. Of the few reported AIDS cases with central nervous system aspergillosis, the majority have had focal brain abscesses. We report an atypical case that presented as a basal meningitis with pontine infarction secondary to invasive Aspergillus sinusitis.     

Management of subdural empyema: a series of 24 cases.

Twenty four cases of subdural empyema are reviewed. The overall mortality was 17%, 18 patients were managed by burr hole and five by craniotomy or craniectomy. Antibiotic therapy was commenced once pus had been evacuated. Infection of the paranasal and mastoid sinuses was the commonest aetiological factor. Aspiration of pus through burr holes is the recommended surgical procedure with low mortality and morbidity, when combined with early diagnosis and aggressive chemotherapy.