Candida albicans hydrocephalus

Central nervous system (CNS) candida infections are often associated with a poor prognosis. Typically, CNS candidiasis presents as meningitis or microabscesses.

Argersinger et al. report a patient with a challenging presentation of a CNS candidiasis as a discrete, large cauda equina abscess. The patient initially presented with ventriculomegaly due to fourth ventricular outflow obstruction and a cauda equina mass. The patient was treated with a ventriculoperitoneal shunt and underwent a lumbar laminectomy for exploration of the lumbar lesion. An intradural abscess was encountered during surgery. Fungal wet mount revealed fungal elements and polymerase chain reaction confirmed the presence of Candida albicans. The patient did not have any known predisposition to fungal infections; therefore, the authors performed whole-exome sequencing using peripheral blood mononuclear cell DNA. They found heterozygous missense variants in the following genes: colony-stimulating factor 2 (CSF2) and Ras protein-specific guanine nucleotide-releasing factor 1 (RASGRF1)-genes that have been specifically associated with protection from CNS candidiasis via caspase recruitment domain-containing protein 9 (CARD9) signaling, and phospholipase C gamma 2 (PLCG2)-a lectin receptor involved in candidiasis. The authors' experience suggests that C. albicans can present as a cauda equina abscess. Hydrocephalus, a result of diffuse arachnoiditis, is a potential complication from intradural fungal abscesses 1)


a 24 year old man with profound chronic hydrocephalus found to have a cauda equina abscess composed of Candida albicans. Prior literature reveals a paucity of central nervous system candidiasis. In these previously reported cases, there was evidence of local invasion of surrounding structures; however, this case is a sentinel report of a fungal abscess without evidence of local structural invasion. The patient's course was complicated by clinical and radiographic worsening to cauda equina syndrome, requiring emergent surgical decompression, despite appropriate antifungal treatment. This case illustrates the diagnostic challenge of this rare entity and the need for close follow up with this patient population 2).


A unique case of Candida albicans ventriculitis and hydrocephalus in the absence of any evidence of systemic candidiasis or immunosuppression is reported. Initial treatment with CSF shunting and intravenous antimycotic therapy appeared to have eradicated the infection. Recurrence occurred 5 months after discharge and this was treated by intravenous and intrathecal antifungal in addition to removal of the shunt system, external ventricular drainage and then replacement of the shunt. A concomitant pyogenic brain abscess responded to burrhole aspiration and antibiotics. The role of mannan antigen monitoring is discussed. 3).


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1)
Argersinger DP, Natkha VP, Shepard MJ, Thomas AA, Oler AJ, Williamson PR, Chittiboina P, Heiss JD. Intradural cauda equina Candida abscess presenting with hydrocephalus: case report. J Neurosurg Spine. 2019 Aug 30:1-4. doi: 10.3171/2019.6.SPINE19271. Epub ahead of print. PMID: 31470401; PMCID: PMC7339489.
2)
Decker BM, Noyes CD, Ramundo MB, Thomas AA. Fungal cauda equina lesion with delayed cord compression and treatment response. Clin Neurol Neurosurg. 2018 Nov;174:185-186. doi: 10.1016/j.clineuro.2018.09.029. Epub 2018 Sep 21. PMID: 30261476.
3)
Jamjoom A, al-Abedeen Jamjoom Z, al-Hedaithy S, Jamali A, Naim-Ur-Rahman, Malabarey T. Ventriculitis and hydrocephalus caused by Candida albicans successfully treated by antimycotic therapy and cerebrospinal fluid shunting. Br J Neurosurg. 1992;6(5):501-4. doi: 10.3109/02688699208995043. PMID: 1333229.
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