Title : A rare case of cerebral phaeohypomycosis - Tissue biopsy to the rescue
Abstract:
Primary cerebral phaeohyphomycosis is a rare infection caused by brown-black pigmented fungi, namely dematiaceous fungi that can cause infrequent but devastating CNS infection due to their recognised neurotropism. Cladophialophora bantiana is recognised as the most common cause of cerebral phaeohyphomycosis. While intracerebral fungal abscesses have been historically associated with immunocompromised patients, C bantiana has a predilection for immunocompetent hosts.
Successful treatment depends on accurate microbiological and histopathological diagnosis and initiation of targeted antifungal treatment. We hereby report a case of 64 year old male, known hypertensive, chronic smoker, who presented with complaints of bilateral lower limb pain with blackish discoloration of left foot and left foot drop for 1 month. Patient also had breathlessness for 4 days. On examination, He was hemodynamically stable. Dorsalis pedis and posterior tibial pulsations were absent in left lower limb. He was planned for trans femoral embolectomy based on doppler findings which showed thrombotic occlusion of all major vessels.
He then developed lower respiratory tract infection due to which the procedure was deferred. On investigations, he was found to have a hemoglobin of 21.1g/dL with PCV of 65.7. Workup for thrombophilia showed JAK-2 mutation positive.
Patient underwent phlebotomy for the same. Patient had an episode of dizziness and headache following which MRI brain was done and it showed space occupying lesion in left cerebellum with haemorrhage. PET CT whole body was done which revealed FDG avid pleural based nodular lesion in the left lung along with findings consistent with MRI brain.
Biopsy of lung nodule revealed necrotizing granulomatous inflammation and gram stain, KOH, cultures were negative. Anti tubercular treatment was started. Repeated blood cultures were sterile. During course of hospital stay, patient developed sudden onset altered sensorium with involuntary movements of right upper limb and dysphasia following which he was intubated for airway protection. CT brain showed left cerebellar lesion with mass effect for which steroids were added.
Patient’s sensorium did not improve and MRI brain was repeated which showed increase in size of lesion with multiple cerebellar abscesses in the posterior fossa region, most likely pyogenic or fungal and likely vasculitic subacute infarcts in the left side of the pons and right inferior cerebellar region. Trans Esophageal Echo showed no evidence of vegetation. Steroids/Anti tubercular drugs were discontinued and Amphotericin B was added to the treatment. Autoimmune work up was normal. Brain biopsy was done which showed growth of Cladophilophora bantiana. Echinocandin was added. Despite treatment, patient succumbed to illness after 50 days of treatment.
