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1/6. Acute isolated cerebral mucormycosis in a patient with high grade non-Hodgkins lymphoma.

    A 57-year-old female in complete remission of grade IV non-Hodgkin lymphoma whilst on intensive chemotherapy, suddenly developed unilateral hemispheric stroke with a fatal outcome in 3 days. She was apyrexial and had received antifungal prophylaxis during her treatment. Post-mortem examination showed complete thrombosis of the internal carotid artery leading to infarction in the territory of the middle and anterior cerebral arteries. Microscopic examination of the brain showed involvement of intra-cranial vessel walls and brain parenchyma by mucormyces, with no evidence of systemic mucormycosis. Isolated cerebral mucormycosis is a rare occurrence, more commonly found in intravenous drug abusers, but can occur in patients with haematological malignancy.
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2/6. Cerebral mucormycosis: proton MR spectroscopy and MR imaging.

    Proton magnetic resonance spectroscopy (MRS) was integrated with magnetic resonance imaging (MRI) in the evaluation of a case of cerebral mucormycosis. MRS showed markedly elevated lactate, depleted N-acetyl aspartate and metabolite resonances attributable to succinate and acetate. The spectroscopy profile is essentially similar to that of bacterial abscess but without the commonly seen resonances of the amino acids valine, leucine and isoleucine. Our extensive literature review did not yield any reports of MRS findings on cerebral mucormycosis. MRS prospectively limited the differential diagnoses given the otherwise nonspecific and complex MR imaging findings in our immunosuppressed patient.
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keywords = mucormycosis
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3/6. Management of CNS mucormycosis in the pediatric patient.

    Rhinocerebral mucormycosis (RM) is a rare, rapidly progressive disorder caused by fungi from the Mucoraceae family. With extensive central nervous system involvement, this disease is uniformly fatal within weeks. mucormycosis normally presents in poorly controlled diabetics, intravenous drug abusers and immunocompromised patients. Many have advocated radical surgical resection (i.e. exenteration of the cavernous sinus with carotid sacrifice and en bloc resection) with administration of amphotericin b. We present a case of mucormycosis involving the paranasal sinuses and cranial base in a pediatric patient who experienced long-term survival with a more limited resection. We also present a review of the relevant literature. A 14-year-old diabetic male presented with RM with involvement of the bilateral frontal lobes, right basal ganglia and temporal lobe. Additionally, there was involvement of the sphenoid sinus and right cavernous sinus with extension into the posterior fossa along the course of the trigeminal nerve and encasement with narrowing of the right carotid artery. The patient underwent sinus endoscopy with debridement of necrotic fungal tissue and bone. This was followed by craniotomy with evacuation of bifrontal, right temporal and basal ganglia abscesses in such a way that all abscess cavities communicated. An Ommaya reservoir was placed into the largest cavity. The patient continued to receive intrathecal and intravenous antibiotics as well as hyperbaric oxygen therapy. The patient was clinically and radiographically free of disease 1 year after diagnosis. While invasive RM is generally a fatal disease, this rare disorder can be treated successfully without radical resection, particularly if multimodality treatment options are implemented.
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keywords = mucormycosis
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4/6. Improving outcomes in rhinocerebral mucormycosis--early diagnostic pointers and prognostic factors.

    Rhinocerebral mucormycosis is an uncommon, rapidly progressive, highly fatal sinus infection, usually occurring in immunocompromised hosts. We describe our clinical experience with nine such consecutive cases managed at our centre, with emphasis on identifying early diagnostic and prognostic features. Perinasal cellulitis/paraesthesia was the most frequent early clinical sign of disease, being evident in at least six cases. Periorbital oedema, mucopurulent rhinorrhoea and nasal crusting were the other early manifestations. Concurrent computed tomography (CT) scan at this initial stage however revealed only minimal mucosal thickening of the sinuses in all four cases wherein it was done. Intracranial extension as evident on CT was the only adverse prognostic sign (p<0.05). The present report highlights the importance of early diagnosis and prompt institution of antifungal chemotherapy in ensuring a favourable outcome in rhinocerebral mucormycosis. However, initial CT is frequently near-normal and biopsy time-consuming and often not feasible. To optimize early diagnosis therefore, the clinician should be highly alert to certain subtle clinical signs, in the appropriate setting of an immunocompromised patient.
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5/6. Carotid artery occlusion due to fulminant rhinocerebral mucormycosis.

    mucormycosis is a rare disorder with a potentially lethal course caused by an opportunistic fungus of the Phycomycetes family. Rhinocerebral mucormycosis (RCM) is an acute fulminant form of invasive fungal sinusitis occurring principally in individuals who are immunologically or metabolically compromised. This form originates from one of the paranasal sinuses and may spread to the orbit or the intracranial space either by direct extension or using the unique capability of spreading along vascular walls, leaving the bony structure intact. Carotid artery occlusion is a complication of RCM. Because of the rapidity with which this disease progresses, prompt and aggressive therapy is essential. The morbidity and mortality of this disease are directly related to the length of time before diagnosis and treatment. The diagnosis is difficult because invasive mucormycosis may be present with no mucosal changes and a normal sinus CT scan. It is a histological diagnosis. Treatment of RCM includes early diagnosis, correction of underlying conditions, early and radical surgical debridement, and lipid formulation of amphotericin b. Multiple surgeries are often necessary for adequate debridement. We report a case of carotid artery occlusion due to RCM and discuss the literature on its early signs and symptoms, pathophysiology, and treatment options.
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6/6. mucormycosis presenting with painful ophthalmoplegia.

    mucormycosis is a rare fungal infection that can involve the sino-orbito-cerebral region. Sino-orbito-cerebral mucormycosis is most common in patients who are immunocompromised or have diabetes mellitus, severe malnutrition or burns. This condition can be fatal if it is not diagnosed early and treated aggressively. This article presents 4 cases of mucormycosis, including 2 with orbital apex syndrome, 1 with cavernous sinus syndrome, and 1 with multiple cranial nerve involvement. All of the patients were presented with painful ophthalmoplegia. The predisposing factors for mucormycosis included diabetes mellitus (three patients) and chronic leukemia (one patient). In all cases, mucormycosis was diagnosed by examining endoscopic sinus drainage material and was treated with surgical debridement and amphotericin b. Two patients with central nervous system involvement died. The others have survived, but still exhibiting various neurologic abnormalities after aggressive treatment. patients with mucormycosis rarely present with orbital apex syndrome. The possibility of mucormycosis should be investigated in any patient with painful ophthalmoplegia, and prompt otorhinolaryngologic examination is recommended to ensure rapid diagnosis and treatment.
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keywords = mucormycosis
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