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Table of Contents    
LETTER TO EDITOR
Year : 2020  |  Volume : 68  |  Issue : 1  |  Page : 202-203

A Rare Case Report of Aspergillosis of CNS Presenting as a Clival Mass Causing SAH from a Mycotic Aneurysm in an Immunocompetent Patient


1 Department of Neurosurgery, Sree Chitra Tirunal Institute for Medical Science and Technology, Trivandrum, Kerala, India
2 Department of Pathology, Sree Chitra Tirunal Institute for Medical Science and Technology, Trivandrum, Kerala, India

Date of Web Publication28-Feb-2020

Correspondence Address:
Dr. Prakash Nair
Department of Neurosurgery, Sree Chitra Tirunal Institute for Medical Science and Technology, Trivandrum - 695 011, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.279654

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How to cite this article:
Vaidya P, Nair P, Abraham M, Rajalakshmi P. A Rare Case Report of Aspergillosis of CNS Presenting as a Clival Mass Causing SAH from a Mycotic Aneurysm in an Immunocompetent Patient. Neurol India 2020;68:202-3

How to cite this URL:
Vaidya P, Nair P, Abraham M, Rajalakshmi P. A Rare Case Report of Aspergillosis of CNS Presenting as a Clival Mass Causing SAH from a Mycotic Aneurysm in an Immunocompetent Patient. Neurol India [serial online] 2020 [cited 2020 Mar 31];68:202-3. Available from: http://www.neurologyindia.com/text.asp?2020/68/1/202/279654




Sir,

Fungi are considered to be low virulence organisms that generally cause opportunistic infections when the host's immunity is lowered. Aspergillosis remains the most commonly reported fungal infection of the central nervous system in India and other tropical countries.[1] The fungus usually enters the CNS by contiguous infection of the paranasal sinuses.[1] Although more common in immunosuppressed patients, aspergillosis of the CNS in immunocompetent patients can be life-threatening.[2] One of the most lethal sequelae of CNS aspergillus infection is the formation of intracranial aneurysms. It has been reported less than 15 times since it was first described in 1968.[3] Subarachnoid hemorrhage (SAH) from a rupture of intracranial aneurysm caused by an Aspergillus infection has a high mortality rate.[4] Due to its high fatality, the early diagnosis and prompt treatment of cases with CNS aspergillus infection is crucial.

A 22-year-old male patient with no co-morbidities presented with a 6-month history of headache and diplopia. On examination, he was conscious, alert and afebrile. He had no clinical lateralizing signs, signs of meningitis, nor symptoms of elevated intracranial pressure (ICP). MRI showed an irregular mass measuring 2.6 × 2.8 × 2.7 cm, involving the clivus, sella and the dorsum sella with significant bone erosion, the mass was seen encasing the cavernous ICA bilaterally [Figure 1]a. MR Angiogram did not reveal any intracranial aneurysm or areas of infarction. He was advised endoscopic endonasal surgery for the clival mass, which he declined. Two weeks later, he returned to hospital with fever, severe headache and two episodes of seizures. On examination, his GCS was 8/15. CT scan showed evidence of subarachnoid hemorrhage (SAH) and intraventricular hemorrhage (IVH) [Figure 1]b. MRI showed diffusion restriction in right posterior cerebral artery (PCA) territory and the left thalamus, MRA showed a saccular aneurysm directed laterally from the distal basilar artery measuring 6.2 × 5.1 mm [Figure 1]c. Another aneurysm was seen in the distal right M1 segment of the middle cerebral artery (MCA) measuring 4.2 × 2.9 mm directed inferiorly [Figure 1]d. An endoscopic transnasal biopsy showed chronically inflamed respiratory mucosa with ill-defined epithelioid cell granuloma and foreign body giant cells. Entrapped within the granulomatous response were many septate fungal hyphae that were slender with parallel edges and acute angle branch points. These features were highlighted by Grocott's methenamine silver and periodic acid-Schiff stains and were suggestive of Aspergillus species [Figure 2]. Cerebrospinal fluid analysis showed evidence of meningitis. The patient received treatment for fungal meningitis; however, he succumbed to overwhelming infection.
Figure 1: (a) Sagittal contrast enhanced MRI showing irregular heterogenous mass involving clivus. Pre-pontine hyperintensity due to extension of infection to the prepontine cistern is seen. (b) CT image showing SAH and IVH with hydrocephalus. (c) MRA showing distal basilar saccular aneurysm directed laterally. (d) MRA showing right M1 segment aneurysm directed inferiorly

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Figure 2: Respiratory mucosa showing multiple ill-defined epithelioid cell granuloma and foreign body giant cell response (a and b) elicited around fungal hyphae with morphological features suggestive of Aspergillus species (c). [(a and b): Haematoxylin and Eosin, (c): Grocott's Methenamine Silver; Magnification = Scale bar]

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Fungal spores are inhaled into the bronco-pulmonary system or paranasal sinuses. Most fungal infections of the respiratory passages are localized; however, invasive infection is typically characterized by extension of hyphae through the sinus mucosa and walls, followed by invasive spread into contiguous structures. This is seen especially for certain fungi like Aspergillus and Mucor. Involvement of the sphenoid gives access to multiple passages for intracranial extension. Intracranial complications following invasive Aspergillus sinusitis may include meningitis, fungal brain abscess, cavernous sinus thrombosis, skull base osteomyelitis, stroke, and, rarely, as was seen in our patient, cerebral vascular aneurysms.[5]

The angioinvasive nature of Aspergillus can be attributed to its ability produce elastase, which in turn causes damage to the internal elastic membrane of intracranial vessels and contributes to compromising the structural integrity of vessels. Fungal aneurysms are characteristically fusiform in shape and tend to involve longer and more proximal segments of the intracranial vessels.[6] The mortality rate for intracranial aspergillosis is extremely high.[7] Prompt recognition coupled with endoscopic biopsy for identifying the pathology, allows early initiation of treatment. The presence of T2W hypointense lesions with the presence of areas of restricted diffusion on DWI within the lesion, a decrease in perfusion on perfusion-weighted imaging, and areas of hemorrhage are characteristic of fungal infection. In our case, the lesion was confined to the sphenoid sinus, with destruction of the clivus, on T2W the lesion was hyperintense with no diffusion restriction. CSF analysis showed an increase in white cell count (CSF-Total Count-300, polymorphs 40% lymphocyte 60%) and an increase in protein content (CSF protein 124mg%), CSF culture results did not grow any microorganism or fungi. In addition, imaging did not show involvement of any another sinus, nor did the patient reveal any complaints suggestive of sinonasal fungal infection. The patient was immunocompetent and did not have any history of diabetes mellitus. All the above-mentioned details demonstrate a highly atypical presentation of a sinonasal aspergillus infection.

Good outcomes can only be obtained with early diagnosis, prompt biopsy, and antifungal therapy (preferably with voriconazole and liposomal amphotericin B).

The current case report suggests that the mycotic aneurysm was a result of the direct extension of fungal sinusitis to the intracranial circulation in an immunocompetent patient. Aneurysm and SAH are devastating complications of invasive aspergillosis, especially in immunocompetent patients. Intracranial fungal infection is a challenging diagnosis which requires early detection. In this case report we propose that in tropical countries, aspergillosis infection must be kept as a differential diagnosis of a clival mass, a high index of suspicion is needed to initiate early biopsy and definitive medical treatment.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Sundaram C, Umabala P, Laxmi V, Purohit AK, Prasad VS, Panigrahi M, et al. Pathology of fungal infections of the central nervous system: 17 Years' experience from Southern India. Histopathology 2006;49:396-405.  Back to cited text no. 1
    
2.
Kim DG, Hong SC, Kim HJ, Chi JG, Han MH, Choi KS, et al. Cerebral aspergillosis in immunologically competent patients. Surg Neurol 1993;40:326-31.  Back to cited text no. 2
    
3.
Dubey A, Patwardhan RV, Sampth S, Santosh V, Kolluri S, Nanda A. Intracranial fungal granuloma: Analysis of 40 patients and review of the literature. Surg Neurol 2005;63:254-60.  Back to cited text no. 3
    
4.
Ahsan H, Ajmal F, Saleem MF, Sonawala AB. Cerebral fungal infection with mycotic aneurysm of basilar artery and subarachnoid haemorrhage. Singapore Med J 2009;50:e22-5.  Back to cited text no. 4
    
5.
Hurst RW, Judkins A, Bolger W, Chu A, Loevner LA. Mycotic aneurysm and cerebral infarction resulting from fungal sinusitis: Imaging and pathologic correlation. Am J Neuroradiol 2001;22:858-63.  Back to cited text no. 5
    
6.
Laurencikas E, Sandstedt B, Söderman M. Intrathecal aspergillosis and fusiform arterial aneurysms in an immunocompromised child: A clinico-pathological case report. Child's Nerv Syst 2006;22:1497-501.  Back to cited text no. 6
    
7.
Wilson WR, Hawrych A, Olan W. Rapid development of bilateral internal carotid artery aneurysm from sphenoid sinus aspergillosis. Skull Base Surg 1998;8:211-4.  Back to cited text no. 7
    


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