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Neuroaspergillosis Leading to Blindness in a Patient with Multiple Myeloma

Butler, Sean DO; Malone, Richard J. DO

American Journal of Physical Medicine & Rehabilitation: January 2010 - Volume 89 - Issue 1 - p 87
doi: 10.1097/PHM.0b013e3181c1ed72
Visual Vignette

From the JFK/Johnson Rehabilitation Institute, Robert Wood Johnson Medical School-UMDNJ, Edison, New Jersey.

Financial disclosure statements have been obtained, and no conflicts of interest have been reported by the authors or by any individuals in control of the content of this article.

All correspondence and requests for reprints should be addressed to Sean Butler, DO, JFK/Johnson Rehabilitation Institute, Robert Wood Johnson Medical School-UMDNJ, Edison, New Jersey.

A 54-yr-old man presented to a major university hospital with right eye vision loss and was found to have fungal sinusitis/meningitis. He then underwent endoscopic sinus surgery for debridement and ethmoidectomy and was placed on antifungal agents. He returned to the hospital 2 months later with 1 wk of left eye vision loss. Computed tomography and magnetic resonance imaging at that time revealed findings most consistent with right frontal cerebritis. Four days later he had transnasal approach to the anterior skull base lesion with debridement and repair of the skull bone defect. During the procedure, a subfrontal collection was drained and sent for culture. He was then diagnosed with invasive neuroaspergillosis. It is believed that the patient's optic nerve was invaded by the fungus, leaving him with severe visual deficits. He was sent to acute inpatient rehabilitation on intravenous caspofungin and oral voriconazole. The patient was declared legally blind.

This patient was originally diagnosed with multiple myeloma in 1999 and underwent chemotherapy followed by stem cell transplant and a second treatment with chemotherapy.

The magnetic resonance imaging shown was obtained during the patient's rehabilitation stay because of increasing headache (Fig. 1). It shows an extra-axial abscess overlying a transphenoidal defect with extension into the left subfrontal lobe with diffuse edema. He was started on topiramate, etodolac, and oxycodone/acetaminophen as needed for headache, which seemed to help but did not completely alleviate the pain. His neurosurgeon believed that the patient would not benefit from additional surgery. His rehabilitation stay was further complicated by an extensive right lower limb deep vein thrombosis, for which he received anticoagulation with low-molecular-weight heparin and eventually had an inferior vena cava filter placed, hyponatremia secondary to inappropriate antidiuretic hormone hypersecretion, hypokalemia, hypocalcemia, and Clostridium difficile colitis.



Some of the rehabilitation challenges besides the above-mentioned medical complications were that the patient had to learn compensatory techniques for his new-onset bilateral blindness. Tactile cues for feeding and dressing were taught, as well as cane sweep for walking. He had loss of balance backward with no attempt to correct himself. Difficulty arose when discharging the patient on anticoagulation because warfarin and voriconazole interact, potentially doubling the effect of warfarin on the International Normalized Ratio. It was decided to discharge the patient home on low-molecular-weight heparin instead of starting warfarin to avoid said risk.

Invasion of the brain tissue by Aspergillus is an extremely rare occurrence. It is highly resistant to treatment with antifungals and can become fatal quickly. This fungus usually makes its way into the central nervous system via the respiratory tract.1 There must be a high index of suspicion for neuroaspergillosis in immunocompromised patients with mental status changes, seizures, or focal neurologic findings, because there is a high mortality rate if it is not treated promptly. These patients are at risk for direct extension of sinonasal disease as well as hematogenous spread from other locations of Aspergillus infection.2

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1.Turgut M, Ozsunar Y, Oncü S, et al: Invasive fungal granuloma of the brain caused by Aspergillus fumigatus: A case report and review of the literature. Surg Neurol 2008;69:169–74
2.DeLone NR, Goldstein RA, Petermann G, et al: Disseminated aspergillosis involving the brain: Distribution and imaging characteristics. Am J Neuroradiol 1999;20:1597–604
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