Neurology India, Vol. 54, No. 1, January-March, 2006, pp. 102-103
Letter To Editor
Cerebral phaeohyphomycosis presenting as an intraventricular mass
G. Samson Sujit Kumar, Dugar Manish, Chacko Geeta
Departments of Neurological Sciences, Christian Medical College and Hospital, Vellore, Tamilnadu
Code Number: ni06030
Phaeohyphomycoses are subcutaneous and systemic infections caused by dematiaceous fungi with dark walled hyphae due to the presence of melanin in the cell wall. Cerebral phaeohyphomycosis presents most commonly as a cerebral abscess and Cladophialophora bantiana accounts for the majority of the reported cases thus far.
A 20-year-old woman presented with headache, vomiting and progressive weakness of right upper and lower limbs for three months. Physical examination revealed bilateral papilloedema, right sided hemiparesis and spastic gait. Magnetic resonance imaging (MRI) of the brain showed an enhancing lesion in the left lateral ventricle causing dilatation of the ipsilateral lateral ventricle. The choroid plexus of the ipsilateral ventricle was thickened [Figure - 1]. She underwent a left frontal craniotomy and subtotal excision of the mass. At surgery the mass was intraventricular and was attached to the choroid plexus and had papillary projections. Anteriorly the mass was adherent to the caudate nucleus and the thalamostriate vein. The histopathological examination showed a circumscribed nodule in the choroid plexus consisting of several discrete necrotising granulomata composed of multinucleated giant cells, histiocytes and lymphocytes. Pigmented branching hyphae were seen within the necrotic centers of the granulomata and within some of the giant cells [Figure - 2]. The biopsy was reported as necrotising granulomatous inflammation consistent with phaeohyphomycosis. Species identification was not possible, as cultures had not been submitted. She was advised systemic antifungal treatment with amphotericin B, but was discharged at request on oral ketoconazole. Two weeks later she presented with symptoms of raised intracranial pressure. A computed tomogram of the brain showed an increase in the dilatation of the left lateral ventricle. She underwent an emergency left ventriculoperitoneal shunt. CSF fungal and mycobacterial cultures done at this point were negative. She was started on intravenous Amphotericin B and a cumulative dose of 1.5 gms was given. She was discharged on a six-month course of oral itraconazole.
Cerebral phaeohyphomycosis was first reported in 1952 by Binford and since then several cases have been reported. Apart from Cladophialophora bantiana other agents responsible are Exserohilum spp, Exophiala jeanselmei, Ramichloridium mackenziei, Fonsecaea pedrosoi, Ochroconis gallopavum, Chaetomium globosum and Bipolaris spicifera.,, Cladophialophora bantiana is neurotropic in nature and may cause brain abscess in both normal and immunosuppressed patients. The portal of entry is not well established. The possible sources include trauma, iatrogenic, contiguous spread from paranasal sinuses and hematogenous dissemination. To the best of our knowledge there are no reports of an intraventricular mass lesion. Fontana-Mason stain for histologic identification is only indicated early on in the disease process as the hyphae may not appear pigmented leading to false identification and under reporting. Treatment must be individualized with surgical resection and optimal antifungal therapy. The most appropriate drug therapy is not clearly defined. Varied results are reported with systemic administration of miconazole, potassium iodide, ketoconazole, amphotericinB, flucytosine, fluconazole and itraconazole. Itraconazole is the preferred antifungal agent for phaeohyphomycosis. Relapses after therapy with other drugs, specifically ketoconazole and amphotericin B, have been treated successfully with oral itraconazole.
In conclusion this is a case of cerebral phaeohyphomycosis presenting at an unusual site. The feasibility of surgical resection should be considered in all patients with cerebral phaeohyphomycosis and duration of antifungal treatment must be individualized using radiological evidence of resolution. Infection due to phaeohyphomycosis must be considered in fungal granulomatous inflammations.
Copyright 2006 - Neurology India
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