A Rare Case of Rhinocerebral Mucormycosis Presenting as Garcin Syndrome and Acute Ischemic Stroke.

BACKGROUND: Mucormycosis is a rare fungal infection affecting immunocompromised patients, with the rhinocerebral variant as the most common presentation.1 Garcin syndrome is the progressive involvement of the cranial nerves resulting in total unilateral paralysis of cranial nerves, absence of sensory or motor deficits, and not associated with features of raised intracranial pressure.2 We report a case of invasive rhinocerebral mucormycosis presenting as Garcin syndrome and acute ischemic stroke. HOW TO CITE THIS ARTICLE: Nagendra V, Thakkar KD, Prasad Hrishi A, Prathapadas U. A Rare Case of Rhinocerebral Mucormycosis Presenting as Garcin Syndrome and Acute Ischemic Stroke. Indian J Crit Care Med 2020;24(11):1137-1138.

Garcin syndrome caused by sphenoid bone metastasis of lung cancer: a case study.

BACKGROUND: Garcin syndrome, which consists of unilateral palsies of almost all cranial nerves without either sensory or motor long-tract disturbances or intracranial hypertension, can be caused by malignant tumors at the skull base. The case of a patient with lung cancer that metastasized to the sphenoid bone and resulted in Garcin syndrome is presented. CASE PRESENTATION: A 76-year-old woman was diagnosed as having non-small cell lung cancer with pericardial and diaphragmatic infiltration, cT4N1M0, stage 3A. The left lower lobectomy with concomitant resection of the pericardium and diaphragm was performed. The pathological diagnosis was pleomorphic carcinoma, pT2bN0M0, stage 1B. She was then followed in the surgery clinic, and 2 months after surgery, she visited an emergency room complaining of headache and diplopia. Neurological examination showed the left IV, V1, and VI cranial nerve palsies. Metastatic tumor with bone destruction was found in the left sphenoid sinus on head computed tomography (CT) and contrast magnetic resonance imaging (MRI), and she was diagnosed with Garcin syndrome caused by sphenoid bone metastasis of lung cancer. Irradiation was performed as palliative treatment, but her neurological findings did not improve. Her general condition gradually worsened, and she died 5 months after surgery. CONCLUSIONS: Bone metastasis of lung cancer occurs frequently, but sphenoid bone metastasis is extremely rare. In this case report, Garcin syndrome caused by lung cancer is discussed in the context of the few previous reports.

Skull base osteomyelitis: A case report of Garcin syndrome due to mucormycosis in COVID pandemic.

Garcin syndrome is characterized by progressive unilateral multiple cranial nerve palsy without the presence of intracranial hypertension. In this case, we present a patient who experienced lower cranial nerve (CN 9-12) involvement attributed to post-mucormycosis osteomyelitis of the skull base. The osteomyelitis resulting from mucormycosis led to the development of Garcin syndrome, which manifested as progressive paralysis of the cranial nerves. It is important to recognize this rare complication and consider it in the differential diagnosis when evaluating patients with lower cranial nerve palsy following mucormycosis-related skull base osteomyelitis.

Garcin syndrome in a case of acquired immunodeficiency syndrome.

In this study, we report a parapharyngeal diffuse large B-cell lymphoma in a human immunodeficiency virus (HIV) infected patient which had caused the patient to suffer from Garcin syndrome.

Gracin's Syndrome, a Rare Clinical Challenge in the Setting of Invasive Mucormycosis: A Systematic Review.

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.

Garcin Syndrome Due to Meningeal Carcinomatosis from Gastric Cancer.

A 58-year-old woman presented to our hospital with complaints of dysphagia. Esophagogastroduodenoscopy showed an esophagogastric junction tumor with multiple duodenal intramural metastases, and computed tomography showed peritoneal metastasis. In the middle of her fourth cycle of chemotherapy, she displayed symptoms of a left-sided multi-cranial nerve palsy. She was diagnosed with Garcin syndrome caused by meningeal carcinomatosis from gastric cancer based on the results of gadolinium-enhanced brain magnetic resonance imaging and cytology of the cerebrospinal fluid. It is important not to overlook meningeal irritation symptoms or paralysis of cranial nerves and to consider the possibility of Garcin syndrome caused by meningeal carcinomatosis.

Surgical management of primary Ewing's sarcoma of the petroclival bone extend into the sphenoid sinus: A case report and review of literatures.

BACKGROUND: Ewing's sarcoma (ES) is a malignancy that arises from bones or soft tissue, characterized by primitive small and round blue cells. Primary ES typically occurs in the long bones, vertebrae, or pelvis, and is extremely rare in the skull base. CASE DESCRIPTION: A 14-year-old girl presented with posterior cervical pain and dysfunction of multiple cranial nerves (CNs). Radiological investigation revealed a solid mass of the petroclival bone extending into the sphenoid sinus. The patient underwent endoscopic transsphenoidal surgery for diagnosis of the pathology, and partial resection was safely achieved. Histopathological, genetic, and radiological examinations confirmed the diagnosis of primary ES. Subsequently, the patient underwent adjuvant chemotherapy and radiotherapy following which the clinical symptoms resolved. Complete response was achieved after multimodal treatment. Twenty months after treatment, the patient remains in remission without recurrence or metastatic disease. Primary ES of the petroclival bone has been reported in only three cases in the literature. As seen in the present case, dysfunction of multiple CNs is the most common manifestation of petroclival ES. Diagnosis should be confirmed by histopathological and genetic examinations considering the nonspecific clinical symptoms and radiological features. CONCLUSION: Multimodal treatment, including surgery, chemotherapy, and radiotherapy, can result in favorable outcomes. Clinicians should consider safe resection during surgical management to prevent complications that can delay postoperative multimodal treatment.

Relapsing Long-Lasting Garcin Syndrome Revealing Skull Base Diffuse B Cell Lymphoma: The Diagnosis through the "Hartel's Route".

The Garcin syndrome is a rare condition characterized by multiple unilateral cranial nerve palsy, without neither long-tract involvement nor intracranial hypertension. Non-Hodgkin lymphoma is a systemic malignant disease that localizes in a minority of cases in the central nervous system. We report a case of Garcin syndrome that revealed a diffuse large B cell lymphoma (DLBCL) located in the skull base and in the right kidney. We reached the diagnosis by mean of a nonstandard, mini-invasive, transforamen ovale biopsy of the intracranial lesion (Hartel's route). The nature of the renal mass was determined ex juvantibus. The patient responded to the polichemotherapy with a complete regression of the intracranial lesion and of the renal mass evaluated by computed tomography and total body positron emission tomography scans. We, therefore, confirmed the DLBCL location in the right kidney. Over 4 years of follow-up, the patient has showed a complete remission of the disease. In this report, we emphasize the importance of biopsy in case of Garcin syndrome.

[Atypical skull base osteomyelitis suspected of spreading inflammation from the ear canal with unilateral multiple cranial neuropathy and cerebral infarctions].

A 76-year-old man, who had undergone surgery for esophageal cancer in 2010, presented to our hospital in April 2017 complaining of prolonged slight fever, loss of appetite, and dysphagia. Initial evaluation revealed a paralyzed left vocal cord, slight muscle weakness of the extremities, left facial paralysis, hoarseness, left sternocleidomastoid and trapezius muscle weakness, tongue deviation to the left, and left hypacusia-suggesting a diagnosis of Garcin's syndrome. Laboratory tests revealed increased white blood cells and C-reactive protein. Cerebrospinal fluid (CSF) analysis showed mild pleocytosis (predominantly polymorphonuclear cells), elevated protein, and low CSF/plasma glucose ratio. CT showed mild clival erosion, with no evidence of carcinoma recurrence. Brain contrast-enhanced MRI showed abnormal clival marrow, enhanced soft tissue and dura matter from the clivus to the atlantoaxial joint, enhanced soft tissue around the left ear canal, multiple cerebral infarctions in the left watershed zones, and left internal carotid stenosis. There was excessive ear wax and inflammation of the left external acoustic meatus but no otorrhea or otalgia. On the basis of his overall presentation, he was diagnosed with atypical skull base osteomyelitis due to external otitis. He was treated with antibiotic treatment that included ceftazidime for the Pseudomonas aeruginosa detected on bacterial cultures. He did not respond to treatment and died approximately 4 months later. Skull base osteomyelitis is thus an important differential diagnosis candidate after finding unilateral, multiple cranial neuropathy, underscoring the importance of prompt treatment when suspected.

The "black evil" affecting patients with diabetes: a case of rhino orbito cerebral mucormycosis causing Garcin syndrome.

Mucormycosis is a life-threatening infection affecting patients with diabetes. It is an angioinvasive disease often resistant to treatment with a debilitating course and high mortality. Here, we report a case of a 45 year old woman with type 2 diabetes mellitus who presented to us with history of right-sided ptosis and facial palsy, and subsequently developed loss of vision and palatal palsy. She was in diabetic ketoacidosis. Nervous system examination revealed involvement of right second, third, fourth, sixth, seventh, ninth, and tenth cranial nerves, suggestive of Garcin syndrome. The hard palate had been eroded with formation of black eschar. Computed tomography of paranasal sinuses revealed right maxillary and ethmoid sinusitis, with spread of inflammation to infratemporal fossa and parapharynygeal neck spaces. Debridement of sinus mucosa was done, and culture of the same yielded growth of rhizopus species. Histopathological examination of the tissue showed angioinvasion and fungal hyphae suggestive of mucormycosis. She was treated with amphotericin B, posaconazole, and periodic nasal sinus debridement, but her general condition worsened after 8 weeks due to secondary sepsis and she succumbed to death.

Looks like Tuberculous Meningitis, But Not: A Case of Rhinocerebral Mucormycosis with Garcin Syndrome.

Rhinocerebral mucomycosis (RCM) as an emerging opportunistic, angioinvasive, and devastating fungi infection with high mortality is difficult to be diagnosed early because of the lack of specific clinical features or manifestations. Garcin syndrome is more often caused by skull base and rhinopharyngeal tumors or metastases, and basal meningitis. We reported that an aged diabetic man, involved nearly all cranial nerves (Garcin syndrome), who was at first suspected to be suffered from tuberculous meningitis, ultimately developed typically progressing RCM. Diagnosis was made to find the presence of mucormycosis in the infected tissue by biopsy.

A case of chondrosarcoma presenting as Garcin syndorme

Garcin's syndrome is defined as showing total unilateral cranial nerve palsies (at least more than 7 ipsilateral cranial nerve palsies), without either sensory or motor long tract disturbance, and without intracranial hypertension. The present report describes an 8-year-old child who had swelling on the right postauricular area and ptosis on the same side which developed before 2 months ago. Neurologic examination disclosed complete unilateral cranial nerve palsies on the right side. Brain MRI revealed a bulky hypervascular well-enhancing solid tumor, involving the right temporal bone, middle cranial fossa, cerebellopontine angle, infratemporal and pterygopalatine fossae, and parapharyngeal space. A biopsy was performed, and its finding showed a chondrosarcoma.