Abstract

Permanent visual loss is a devastating yet preventable complication of cryptococcal meningitis. Early and aggressive management of cerebrospinal fluid pressure in conjunction with antifungal therapy is required. Historically, the mechanisms of visual loss in cryptococcal meningitis have included optic neuritis and papilloedema. Hence, the basis of visual loss therapy has been steroid therapy and intracranial pressure lowering without clear guidelines. With the use of high-resolution magnetic resonance imaging of the optic nerve, an additional mechanism has emerged, namely an optic nerve sheath compartment syndrome (ONSCS) caused by severely elevated intracranial pressure and fungal loading in the peri-optic space. An improved understanding of these mechanisms and recognition of the important role played by raised intracranial pressure allows for more targeted treatment measures and better outcomes. In the present case series of 90 HIV co-infected patients with cryptococcal meningitis, we present the clinical and electrophysiological manifestations of Cryptococcus-induced visual loss and review the mechanisms involved.

Highlights

  • Meningitis owing to Cryptococcus neoformans remains a frequent human immunodeficiency virus (HIV)- associated opportunistic infection even in developing countries with effective antiretroviral therapy (ART) rollout programmes.[1]

  • Visual loss occurred at any stage of the illness and occurred frequently before starting drug therapy

  • Rex’s landmark article in 1993 of Cryptococcusinduced visual loss suggested two main mechanisms: an early and sudden visual loss owing to optic neuritis, and a late and gradual visual loss owing to papilloedema

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Summary

Introduction

Meningitis owing to Cryptococcus neoformans remains a frequent human immunodeficiency virus (HIV)- associated opportunistic infection even in developing countries with effective antiretroviral therapy (ART) rollout programmes.[1] This is largely a result of failure of HIV testing by individuals with risky sexual behaviour, and late presentation for and poor compliance with ART. Effective antifungal therapy (amphotericin B, flucytosine and fluconazole) is not readily available in most developing countries.[2] Mortality remains high and contributes up to 20% of HIV-related deaths.[1] Complications in survivors are severe, with visual loss being the most disabling, yet are potentially preventable and reversible. In the following case series, we evaluated 90 patients with culture-confirmed CM. Their results and a discussion of the mechanisms implicated in Cryptococcus-induced visual loss are discussed. An illustrative case of the optic nerve sheath compartment syndrome (ONSCS) as a putative mechanism is presented in the discussion

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