Journal of Neuro-Ophthalmology, June 2004, Volume 24, Issue 2

Concurrent Sino-Orbital Aspergillosis and Cerebral Nocardiosis

A 79-year-old man with myelodysplastic syndrome developed a right optic neuropathy with optic disc edema and intractable periocular pain, one month after undergoing removal of a gangrenous gallbladder. Although results of a temporal artery biopsy were negative, he was treated with prednisone for presumed temporal arteritis. Attempts at tapering the prednisone dose led to recurrence of periocular pain. On neuro-ophthalmologic evaluation six months after the prednisone treatment was begun, he had developed right fourth and sixth cranial nerve palsies, and magnetic resonance imaging demonstrated a right orbital apex mass. Trans-sphenoidal biopsy revealed Aspergillus fumigatus. During treatment of aspergillosis, the patient developed a left hemiparesis. Magnetic resonance imaging disclosed multiple ring-enhancing cerebral masses. Biopsy revealed Nocardia asteroides. The patient was successfully treated for both infections with recovery of neurologic function except for the right optic neuropathy. Although immunocompromised patients are known to be subject to multiple infections, this may be the first reported case of concurrent sino-orbital aspergillosis and cerebral nocardiosis.

PHOTO ESSAY Concurrent Sino- Orbital Aspergillosis and Cerebral Nocardiosis Liselotte Pieroth, MD, Jacqueline M. S. Winterkorn, MD, PhD, Hermann Schubert, MD, William S. Millar MD, MS, and Michael Kazim, MD FIG. 1. A. T1- weighted coronal enhanced magnetic reso­nance imaging scan shows an enhancing mass in the right orbital apex, optic foramen, and adjacent anterior clinoid process. B. Biopsy through the sphenoid sinus revealed a filamentous fungus composed of branching hyphae consis­tent with Aspergillus fumigatus ( Gomori methenamine silver stain, original magnification X40). From the Departments of Ophthalmology ( LP, HS, MK) and Radiol­ogy ( WSM), The New York Presbyterian Hospital- Columbia University Medical College, and the Departments of Ophthalmology and Neurology and Neuroscience ( JMSW), The New York Presbyterian Hospital- Weill Medical College of Cornell University, New York, New York. Presented at the 33rd Frank B. Walsh Meeting of the North American Neuro- Ophfhalmology Society, Rancho Mirage, CA, April 21, 2001. Address correspondence to Jacqueline M. S. Winterkorn, MD, PhD, Department of Neurology and Neuroscience, The New York Presbyterian Hospital- Weill Medical College of Cornell University, 525 East 68th Street, New York, New York 10021; E- mail: jmswinte@ aol. com FIG. 2. A. T1- weighted coronal enhanced magnetic reso­nance imaging scan shows a ring- enhancing lesion in the right frontal cortex. B. Biopsy of the lesion revealed colonies of thin filamentous, branched bacteria consistent with No-cardia asteroides ( Gomori methenamine silver stain, original magnification X40). Abstract: A 79- year- old man with myelodysplastic syn­drome developed a right optic neuropathy with optic disc edema and intractable periocular pain, one month after un­dergoing removal of a gangrenous gallbladder. Although results of a temporal artery biopsy were negative, he was treated with prednisone for presumed temporal arteritis. At­tempts at tapering the prednisone dose led to recurrence of periocular pain. On neuro- ophthalmologic evaluation six months after the prednisone treatment was begun, he had developed right fourth and sixth cranial nerve palsies, and J Neuro- Ophthalmol, Vol. 24, No. 2, 2004 135 JNeuro- Ophthalmol, Vol. 24, No. 2, 2004 Pieroth et al. magnetic resonance imaging demonstrated a right orbital apex mass. Trans- sphenoidal biopsy revealed Aspergillus fumigatus. During treatment of aspergillosis, the patient de­veloped a left hemiparesis. Magnetic resonance imaging disclosed multiple ring- enhancing cerebral masses. Biopsy revealed Nocardia asteroides. The patient was successfully treated for both infections with recovery of neurologic function except for the right optic neuropathy. Although immunocompromised patients are known to be subject to multiple infections, this may be the first reported case of concurrent sino- orbital aspergillosis and cerebral nocar­diosis. { J Neuro- Ophthalmol 2004; 24: 135- 137) A 79- year- old man suddenly lost vision in his right eye and developed right periocular pain one month after he underwent cholecystectomy for a gangrenous but culture-negative gall bladder. Visual acuity was hand movements OD, 20/ 25 OS. The right optic disc was swollen, the left optic disc was normal. These findings suggested anterior ischemic optic neuropathy. He had previously been diag­nosed with a myelodysplastic disorder characterized by anemia and thrombocytopenia. Westergren erythrocyte sedimentation rate was 86 mm/ h. No brain imaging was performed. Despite negative findings on temporal artery bi­opsy, he received a presumptive diagnosis of temporal ar­teritis and was treated with 60 mg/ d prednisone, which eliminated his pain. Each time the prednisone was tapered to 40 mg, pain recurred, so the dose was maintained at 60 mg/ d. Six months after commencement of prednisone treat­ment, he was referred for neuro- ophthalmologic evaluation. Visual acuity was no light perception OD, 20/ 40 OS. Right fourth and sixth cranial nerve palsies were observed, local­izing the lesion to the right orbital apex. Magnetic reso­nance imaging ( MRI) showed an enhancing mass in the right orbital apex, optic foramen, and adjacent anterior cli-noid, with a component in the sphenoid sinus ( Fig. 1A). Lumbar puncture opening pressure was 140 mm H20, with a normal cell count and normal protein and glucose values. Fungal and bacterial cultures of cerebrospinal fluid and blood were negative. Endoscopic biopsy of the mass was performed by the trans- sphenoidal approach. Gomori me-thenamine silver stain revealed the septate hyphae of As­pergillus fumigatus ( Fig. IB). The patient was treated for invasive aspergillosis with 250 mg intravenous liposomal amphotericin daily. Vision did not recover. Three months later, he developed a left hemiplegia. MRI revealed multiple new ring- en­hancing lesions in the cerebrum ( Fig. 2A). Biopsy of a right frontal cortex lesion disclosed Nocardia asteroides ( Fig. 2B). The patient was treated with 200 mg intravenous minocycline and 500 mg intravenous imipenem daily. Six months later, his neurologic status had normalized, apart from a persisting right optic neuropathy. A repeat MRI scan six months after treatment showed no cere­bral lesions. However, the orbital mass persisted. He died of pneumonia two years after the onset of the optic neur­opathy. Immunocompromised patients are particularly sus­ceptible to infections of the paranasal sinuses, yet multiple infections have rarely been reported. Our patient, immuno­compromised by a myelodysplastic disorder, developed invasive aspergillosis originating in the right sphenoid sinus and extending into the orbital apex and cavernous si­nus, causing optic neuropathy and ophthalmoplegia. Hema­togenous spread of invasive aspergillosis from the gastro­intestinal tract has also been reported ( 1,2). Optic neuropathy resulting from invasive aspergillosis may ini­tially manifest as optic neuritis or ischemic optic neuropa­thy ( 3- 5). In the present case, the swollen disc led to the misdiagnosis of temporal arteritis. The optic neuropathy of aspergillosis may initially improve with corticosteroid treatment, but ultimately the organisms proliferate and cause further damage, as occurred in our case ( 5,6). Invasive aspergillosis can occlude cerebral vessels ( 7,8) and cause cerebral infarcts, but our patient's hemiplegia was due to a second infection by N. asteroides. N asteroides is an aerobic, gram- positive, fila­mentous bacterium with a branching growth pattern. Ring- enhancement on MRI is characteristic of nocar-dial infection. Although concomitant pulmonary infections occur commonly, dual infections have been documented in only a few immunocompromised patients ( 9- 12). Concur­rent cerebral infection has occurred from toxoplasmosis and cytomegalovirus or cryptococcus, and cerebral as­pergillosis with candidiasis. To our knowledge, the case presented in the current report is the first reported case of sino- orbital aspergillosis concurrent with cerebral nocar­diosis. REFERENCES 1. RinaldiMG. Invasive aspergillosis. RevInfectDis 1983; 5: 1061- 77. 2. Hedges TR, Leung LS. Parasellar and orbital apex syndrome caused by aspergillosis. Neurology 1976; 26: 117- 20. 3. Segawa H, Takahashi H, Shimizu H, et al. Aspergillosis of the optic nerve: report of a case. No To Shinkei 1973; 25: 77- 82. 4. Weinstein JM, Morris GL, ZuRhein GM, et al. 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