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proptosis, ptosis and periorbital cellulitis. Paralysis of the third, fourth and sixth cranial nerves and visual loss due to central retinal artery occlusion is not uncommon. The fifth and seventh cranial nerves may be involved. The most serious complication of rhinocerebral mycosis is cerebral vasculitis due to direct vascular involvement. [4] Rhinocerebral mycosis is a rapidly progressive and fatal opportunistic infection, predominantly affecting individuals in an immunocompromised state. Occurrence in immunocompetent individuals is not uncommon. [5] It carries a high residual morbidity and mortality due to the angioinvasive property of fungi, causing vascular occlusion and extensive tissue necrosis. Impaired delivery of the antifungal drugs to the site of infection because of vascular thrombosis and limited aggressive surgery because of the complex anatomy of the rhinoorbitocerebral region cautions for early diagnosis and aggressive management in these patients. There are many case reports / series of rhinocerebral mycosis. The mortality in rhinocerebral mycosis ranged from 15-82% in various series. [6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16] The predictors of poor outcome were intracranial extension of the disease, [7],[12] delayed diagnosis [9] and hemiplegia as a neurological complication. [11] Aggressive surgical therapy, with repeated debridement in combination with intravenous amphotericin B can lead to high rate of cure with long-term survival rate. [10],[15]Medical records of 46 consecutive patients with a diagnosis of rhinocerebral mycosis admitted to the Neurology department from January 1997 to December 2005 were analyzed retrospectively. All the patients had histopathalogical evidence of fungal infection. Functional endoscopic excision biopsy using 0 degree and 30 degree endoscopes was done. Tissues were fixed in buffered formalin and processed for paraffin embedding. Hematoxylin and Eosin stained sections were examined for tissue reaction. Fungal morphology was identified by Periodic acid Schiff (PAS) and Gomori's methenamine silver (GMS) and other appropriate special stains. 'Broad non-septate hyaline ribbon-like hyphae with irregular branching' was the basis for identification as Zygomycetes and narrow hyphae with distinctive septa, parallel walls and acute angle dichotomous branching, for Aspergillus . Melanized fungi were identified by the presence of thin branching, septate hyphae with melanin pigment. The melanin-specific Masson-Fontana stain was used for confirmation. Culture reports were obtained whenever tissue was submitted for culture. The demographic data, predisposing illness, clinical features, radiological (MRI/CT) findings, treatment details (medical and/or surgical) and outcomes were recorded.
Univariate analysis with odds ratio (OR) was employed in data analysis. Chi-square test was used for P value and P value of < 0.05 was taken as significant.There were 46 patients with a diagnosis of rhinocerebral mycosis. Eight patients did not continue the treatment and were lost to follow-up. Hence the results were analyzed in 38 patients. Age ranged from 7-82 (mean 48.68) years; 30 (79%) were males. All the patients presented with multiple symptoms and signs. Craniofacial pain was the most common initial presenting symptom, noted in 29 (76.3%) patients. The other symptoms and signs were fever, vomiting, seizures, cranial nerve palsies, altered sensorium, meningitis and focal neurological deficits [Table - 1]. At admission significant anemia (hemoglobin of <8 gm/dl) was noted in 15 (39.5%), leukocytosis in 12 (31.6%) and elevated erythrocyte sedimentation rate (ESR) in 22 (57.9%) patients.Rhinoorbital involvement with facial edema, proptosis, chemosis and ophthalmoplegia were the commonest findings noted in 26 (68.4%) patients. Rhinoorbitocerebral involvement with focal neurological deficits was seen in 10 (31.6%) patients. Sinocranial involvement with focal neurological deficits due to brain abscess was noted in two patients with mycosis due to melanized fungi. The third, fourth and sixth cranial nerves were involved in all the patients with rhinoorbital involvement and optic nerve in 30 (79%) [Table - 1] patients. Two patients had bilateral rhinoorbital involvement.Immunocompromised state was noted in 24 (63.2%); 23 had diabetes mellitus (DM) and one patient of Wegener's granulomatosis was on immunosuppressive therapy. One patient with DM had renal transplantation with hepatitis C and B virus infection and one patient with DM had HIV-1 infection. Seven patients with DM were in diabetic ketoacidosis (DKA), 12 had hyperglycemia and four were euglycemic at the time of admission. One patient had denovo DM with DKA. There were 14
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