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Abstract

The Mucorales is the largest order of zygomycete fungi comprising ubiquitous, mostly saprophytic organisms. However, once invade, the fungus shows angiotropic and neurotropic predilection. These fungi infect humans through spore inhalation. Within tissue, they grow as nonseptate moulds and have right-angle branching and irregular, non-parallel cell walls. The most prevalent agent of mucormycosis is Rhizopus species.

The rhinocerebral form of mucormycosis may occur in immunocompetent hosts, but there is a distinct predilection for diabetics. Other risk factors for mucormycosis include ketoacidosis, steroid use, neutropenia, renal failure and intravenous drug use. Direct intracranial extension from the sinuses occurs with Mucorales infection, producing characteristic lesions at the inferior frontal lobes adjacent to the posterior sinuses, usually with enhancement and reduced diffusion. Perineural invasion is a common finding in rhino-orbito-cerebral mucormycosis (ROCM), as are angioinvasion and infarcts. ROCM develops when inhaled spores infect the paranasal sinuses and extend into the orbits, optic nerves, oral cavity and cranium and invades the intracranial vasculature. Central nervous system infection almost always involves the frontal lobes. Morphological features on histopathologic examination suggest the etiology. Specific identification of the etiologic agent requires isolation of the organism by culture.

Surgical debridement of devitalized tissue, antifungal chemotherapy, correction of impaired immunity and controlling blood glucose levels are the treatment strategies for mucormycosis. Mucorales appear to be susceptible to amphotericin B and are generally not susceptible to the triazoles and echinocandins. Among the extended-spectrum triazoles, posaconazole appears to be active against most of the species.

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Abbreviations

ABLC:

AmB lipid complex

AIDS:

Acquired immunodeficiency syndrome

Als:

Agglutinin-like sequence

AmB:

Amphotericin B

BHB:

β-hydroxy butyrate

BHI:

Brain heart infusion

CAS:

Caspofungin

CNS:

Central nervous system

CotH:

Coating

CSF:

Cerebrospinal fluid

CT:

Computed tomography

DC:

Dendritic cell

ECMM:

European Confederation of Medical Mycology

EUCAST:

European Committee on Antimicrobial Susceptibility Testing

G-CSF:

Granulocyte colony-stimulating factor

GM-CSF:

Granulocyte-macrophage colony-stimulating factor

GMS:

Grocott methenamine silver

GRP:

Glucose-regulated protein

HBO:

Hyperbaric oxygen

H&E:

Haematoxylin and eosin

HIV:

Human immunodeficiency virus

IFN:

Interferon

IL:

Interleukin

ILC:

Innate lymphocyte

ISA:

Isavuconazole

ISHAM:

International Society for Human and Animal Mycology

ITS:

Internal transcribed spacer

iv:

Intravenous

KOH:

Potassium hydroxide

LAmB:

Liposomal AmB

MALDI-TOF:

Matrix-assisted laser desorption/ionization time-of-flight

MHC:

Major histocompatibility complex

MIC:

Minimal inhibitory concentration

MNC:

Mononuclear cell

MRI:

Magnetic resonance imaging

NK:

Natural killer

NO:

Nitric oxide

PAS:

Periodic acid-Schiff

PCR:

Polymerase chain reaction

PDGF:

Platelet-derived growth factor

PL:

Pectin lyase

PMN:

Polymorphonuclear neutrophil

PRR:

Pathogen recognition receptor/Pattern recognition receptor

PSZ:

Posaconazole

RANTES:

Regulated on activation, normal T cell expressed and secreted

RCM:

Rhinocerebral mucormycosis

ROCM:

Rhino-orbito-cerebral mucormycosis

SIG:

Special Interest Group

TLR:

Toll-like receptor

TNF:

Tumour necrosis factor

Th 1:

T-helper type 1

VRZ:

Voriconazole

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Kantarcioglu, A.S. (2019). Mucormycosis. In: Turgut, M., Challa, S., Akhaddar, A. (eds) Fungal Infections of the Central Nervous System. Springer, Cham. https://doi.org/10.1007/978-3-030-06088-6_10

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