ABSTRACT
Garcin's Syndrome is a rare pathology involving multiple cranial nerves in the setting of invasive mucormycosis, usually in immunocompromised patients. Owing to its extremely high mortality rate, clinician should have a high suspicion for diagnosis. This article presents a rare case of Rhino-Orbito-Cerebral Mucormycosis with Garcin's syndrome in a 33-years old male along with a discussion of previously reported cases. The case is discussed in light of scant contemporary literature on the cited subject. A thorough search using the keywords Garcin's Syndrome, Invasive Mucormycosis, Rhino-orbital, Rhino-cerebral mucormycosis, was conducted on Pubmed/MEDLINE, Google scholar, LILACS, medRxiv and Google. A total of six reported cases found in international literature published between 2000 and 2020 were reviewed and analyzed. Garcin's Syndrome is associated with a high mortality rate. In our review, of the total seven patients, only three survived, bringing the collective mortality to 42.85%. Patients had multiple cranial nerve involvement leading to long term sequelae. Our case showed the unique finding of VIII nerve involvement. Early intervention helped in patient survival and better functional outcome. This literature review highlights the distinct clinical nature of the presentation of disease and the importance of prompt diagnosis and early management in the reversal of complications in an otherwise potentially fatal disease.
ABSTRACT
Background: One of the largest outbreaks of rhinosinocerebral mucormycosis (RSCM) occurred in India close to the second wave of the SARS-CoV-2 infection. RSCM is a rare infection caused by several fungal species occurring in immunocompromised subjects. Mucor shows a high propensity to invade the central nervous system. There have been limited studies, mostly isolated case reports, on the neurological manifestations of RSCM. The outbreak of mucormycosis infection was thus the most opportune to study the neurological manifestations and cranial nerve involvement in mucormycosis in greater depths. Aim of the study: The purpose of the study was to investigate and review the involvement of cranial nerves in a series of cases of rhinosinocerebral mucormycosis associated with the novel coronavirus disease caused by SARS-CoV-2. Results: It was a retrospective cross-sectional study of seven patients who were undergoing treatment of RSCM with a recent history of coronavirus disease caused by SARS-CoV-2 infection within the last 3 months. Patients with cranial nerve involvement were identified by magnetic resonance imaging (MRI) at a single institution. Demographic details of the patients, clinical presentation, imaging, microbiological and pathological findings were recorded. All subjects had two or more cranial nerves affected by fungal infection. The most commonly involved cranial nerve was found to be the optic nerve followed by the trigeminal nerve and its branches. We document three cases with extensive involvement of the inferior alveolar branch of the mandibular division of the trigeminal nerve (V3), a previously unreported finding. In one case, in addition to the second and fifth cranial nerves, the third, fourth, sixth, seventh, eighth, and twelfth cranial nerves were involved without any sensory or motor long tract involvement, suggestive of Garcin syndrome secondary to intracranial abscesses and skull base osteomyelitis due to invasive fungal infection. This case is of rare occurrence in the literature, and our study provides one such example. Conclusion: Cranial nerve involvement in patients of mucormycosis tends to have a poor prognosis, both cosmetic and functional. Radical surgeries and aggressive medical management is needed in such cases to improve the outcome.