. however , cerebral phaeohyphomycosis ( cp ) caused by darkly pigmented fungi appears to be a common exception to this rule because about one - half of this fungal infection occurred in patients with no underlying disease or risk factors . cp is a very rare cause of brain abscess , but is often a fatal disease regardless of immune status14,17,23 ) . the authors illustrate a 75-year - old , immunocompetent male patient who had a single brain abscess from dematiaceous fungi . to the authors ' knowledge , this is the first case of cp in korea . a 75-year - old male , a resident of a rural area and a farmer by occupation , visited our outpatient clinic with the symptoms of poor cognition and memory decline over 2 weeks . he denied any history of fever , headache , blurred vision , vomiting or seizure . there were no laboratory abnormalities including leukocytosis or c - reactive protein rising . upon the neurologic examination , he was conscious and there were no neurologic deficits except intermittent expressive dysphasia and disorientation . brain magnetic resonance imaging ( mri ) was performed because of suspicion of some type of dementia . it showed a 20 mm sized nodular enhancing mass with peritumoral edema in the left frontal lobe . high - grade glioma or metastatic tumor was initially presumed based on his age and progressive symptoms . the lesion appeared to be white and took the form of a relatively hard mass with a clear boundary , permitting radical excision of the mass ( fig . . septated hyphae and melanin pigments were confirmed at fontana - masson stain consistent with cp ( fig . the patient was started on intravenous amphotericin b at a dose of 68 mg daily . after 10 days , he was switched to 270 mg of intravenous voriconazole twice a day because of the elevation of serum creatinine . he took the injection for 8 weeks , followed by oral voriconazole 200 mg twice a day for 2 months . a follow - up brain mri 3 weeks after surgical excision demonstrated a significant resolution of the edema . ongoing resolution of the lesion was found on the latest follow - up mri ( fig . he showed dramatic improvement in his symptoms including disorientation and memory disturbance after completion of surgery and antifungal therapy . cp is a rare infection caused by darkly pigmented fungi , namely dematiaceous fungi23 ) . dematiaceous fungi represent a group of filamentous molds that contain melanin pigment in their cell walls3,6,14,17 ) . rhinocladiella mackenziei ( formerly ramichloridium mackenziei ) is the second most common cause of cp , which is exclusively endemic in the middle east area17,22 ) . because of this , occupational predisposition has been reported in agricultural workers , especially farmers due to risk of soil exposure17,23 ) . cp commonly occurs in the second and third decades of life with male predominance , except rhinocladiella mackenziei which affects adults with a median age of 62 years without male predominance3,13,23 ) . the most unique characteristic of cp is its occurrence irrelevant to the immune status of the host15,17,19,23 ) . even though immunodeficiency may play a role as a risk factor , there are many reports of this infection in immunocompetent individuals similar to the patient in this report12,15,17,19,25 ) . the portal entry to brain is unclear , although several possible routes have been suggested , such as hematogenous dissemination of inhaled spores or accidental skin inoculation as well as direct extension from adjacent paranasal sinuses or ears2,6,10,14,15,19,22,23 ) . pathogenesis of cp is associated with the presence of melanin as a virulence factor that provides advantages in evading host defense and crossing the blood - brain barrier by binding to hydrolytic enzyme14,16,17 ) . clinical spectrum of phaeohyphomycosis was listed as a variable , ranging from solitary subcutaneous nodules to a life - threatening infection5,11,16,17,18 ) . in the central nervous system ( cns ) about 70 - 80% of cases typically manifest as a single brain abscess particularly on the frontal lobe ( 52% ) like in our case , while multiple brain abscesses can be seen in immunocompromised patients3,6,17,19 ) . the diagnosis of cp can be difficult because dematiaceous fungi are often considered contaminants when identified in culture . furthermore , the pathogen can not always be cultured and isolated from the serum or cerebrospinal fluid ( csf)12,21,24 ) . no molecular techniques are available to speedily identify these fungi even to the genus level17 ) . only the tissue examination can be useful to identify irregularly swollen hyphae with yeast - like structure and to confirm the presence of dematiaceous hyphae in melanin - specific fontana - masson stain14,17 ) . unfortunately in this case , fungus was not identified in the culture of surgical specimen , therefore , the species that causes cp could not be detected . meanwhile , the brain mri reveals a ring - enhancing lesion with a low - attenuation core , suggesting the presence of necrosis or pus10,19 ) . in cases where high - grade glioma or metastasis is mimicked by irregular and variably contrast - enhancing lesions , imaging findings of this patient were more suggestive of a glioma than an abscess , because nodular heterogeneity on contrast injection mimicked the images seen in high - grade tumors . consequently , surgical biopsy is essential for the diagnosis of cp . because of the rarity of the cases complete excision of brain lesions may provide better results than simple aspiration unless the lesion is multiple or is located within the eloquent area of the brain3,17 ) . antifungal agents are generally used in combination of amphotericin b , 5-flucytosine and itraconazole because it is associated with improved survival rates3,14,17,20 ) . voriconazole can be used as alternative to itraconazole because of its good penetration into both csf and brain tissue17,22 ) . duration of taking the medications is still unknown because most reported patients expired during treatment except a few survivors who received voriconazole for about 12 months8,19 ) . in addition , posaconazole may be a potent drug when pathogen is rhinocladiella mackenziei1,3,8 ) . in this case , amphotericin b has fatal side effects such as nephrotoxicity , therefore , close observation on kidney function is needed . mortality rate approaches 100% in untreated patients , while that of treated cases as high as 65% to 73% despite the aggressive treatment6,9,10,17,19,23 ) . interestingly , mortality rate did not differ significantly between immunocompromised and immunocompetent patients ( 75% vs. 71%)12,17 ) . fortunately , the patient reported here had a good response to surgery and chemotherapy and showed fine recovery without any sequela . solitary lesion and the good general condition of the patient , together with an aggressive therapeutic approach , are therefore inferred to contribute to a favorable outcome . further studies are necessary to find more potentially useful antifungal regimen for these refractory infections and to investigate more detailed pathophysiology and prognostic factors to increase the survival rate . cp is rare disease , but challenging one with high mortality rate , particularly when the cns is affected . as shown in this report , complete resection and adequate antifungal therapy are the most recommended modality for patients with cp - related abscess to this time .
cerebral phaeohyphomycosis ( cp ) is a very rare but serious form of central nervous system fungal infection that is caused by dematiaceous fungi . 
 it is commonly associated with poor prognosis irrespective of the immune status of the patient . in this study , the authors describe the first case of cp in korea that occurred in a 75-year - old man without immunodeficiency and showed favorable outcome after surgical excision and antifungal therapy . 
 in addition , the authors herein review the literature regarding characteristics of this rare clinical entity with previously reported cases .