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Table of Contents    
LETTER TO EDITOR
Year : 2018  |  Volume : 66  |  Issue : 4  |  Page : 1181-1183

Spinal cryptoccoma mimicking a spinal cord tumor complicated by cryptococcal meningitis in an immunocompetent patient


1 Department of Neurology, Kuala Lumpur Hospital, Jalan Pahang, Kuala Lumpur, Malaysia
2 Division of Neurology, Department of Medicine, Faculty of Medicine, University Malaya, Jalan Universiti, Kuala Lumpur, Malaysia
3 Department of Pathology, Kuala Lumpur Hospital, Jalan Pahang, Kuala Lumpur, Malaysia
4 Department of Radiology, Kuala Lumpur Hospital, Jalan Pahang, Kuala Lumpur, Malaysia

Date of Web Publication18-Jul-2018

Correspondence Address:
Dr. S Viswanathan
Department of Neurology, Kuala Lumpur Hospital, Jalan Pahang, 50560, Kuala Lumpur
Malaysia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.237012

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How to cite this article:
Carol L, Tai MLS, Yusoff S M, Rose N, Rafia M H, Viswanathan S. Spinal cryptoccoma mimicking a spinal cord tumor complicated by cryptococcal meningitis in an immunocompetent patient. Neurol India 2018;66:1181-3

How to cite this URL:
Carol L, Tai MLS, Yusoff S M, Rose N, Rafia M H, Viswanathan S. Spinal cryptoccoma mimicking a spinal cord tumor complicated by cryptococcal meningitis in an immunocompetent patient. Neurol India [serial online] 2018 [cited 2018 Aug 17];66:1181-3. Available from: http://www.neurologyindia.com/text.asp?2018/66/4/1181/237012




Sir,

A 19-year old immunocompetent healthy young man presented with acute onset symptoms suggestive of a spinal cord lesion. An urgent magnetic resonance imaging (MRI) of the whole spine revealed a lesion that raised the suspicion of a spinal cord tumor, possibly an ependymoma [Figure 1]a and [Figure 1]b.
Figure 1: (a) Sagittal T2 weighted (w) MRI of the spinal cord showing a ring-like intramedullary lesion within the thoracic spinal cord. The lesion had a central hypointensity with surrounding cord oedema (arrow). (b) Axial T1 W MRI with contrast of the spinal cord showing a ring enhancing lesion within the thoracic cord

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A laminectomy was done 48 hours later. The biopsy report was consistent with chronic granulomatous inflammation secondary to Cryptococcus neoformans infection [Figure 2]a,[Figure 2]b,[Figure 2]c,[Figure 2]d. A thorough immunological workup proved that the patient was immunocompetent.
Figure 2: Histopathology of spinal cord lesion, (a-d) showing Gomori methenamine silver (GMS) and periodic acid Schiff stains (PAS) (a and b); and hematoxylin and eosin stain at ×400 and × 600 power (c and d) of the spinal cord lesion showing (arrows) granulomas, multinucleated giant cells and encapsulated organisms (thickened membranes) positive for PAS and GMS stains

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Nevertheless, antifungal therapy alongside steroid therapy was promptly started. Despite active antifungal therapy, he deteriorated and expired due to nosocomial infection.

The acute onset presentation of an intramedullary spinal cord cryptococcoma is a rare initial presentation of cryptococcal infection. Here, we describe an unusual case of a young immunocompetent patient with a solitary spinal cord cryptococcoma that subsequently led to cryptococcal meningitis, that occurred in the absence of a cerebral cryptococcoma. Prior to this episode, he did not have headache, vomiting, and seizures to suggest a central nervous system infection or raised intracranial pressure. This immunocompetent patient presented acutely with sudden onset of lower limb weakness as a result of the spinal cryptococcoma.

Infection with C. gattii presents with granulomatous inflammatory response in immunocompetent people,[1] resulting in a chronic disease; the patients remain at a risk of developing an intracerebral mass lesions (e.g., a cryptococcoma).[2] In comparison, C. neoformans has a tendency to cause infection in predominantly immunodeficient patients, leading to acute meningitis. In this case, the patient developed C. neoformans of the spinal cord despite being immunocompetent and a young man. The patient progressed to have cryptococcal meningitis despite institution of antifungal therapy and surgical intervention.

The intramedullary lesions of spinal cryptococcosis are described as cryptococcoma.[3] Intramedullary spinal cryptococcoma are less common compared to intradural and other types of extradural lesions.[3] In the literature, there have been a few reports of spinal intramedullary cryptococcoma. In these rare reports, the majority of the patients presented with paraparesis and pyramidal signs in the lower limbs with a sensory level in the thoracolumbar region.[3] In some cases, similar to our case, the lesion was initially thought to be a tumor. Most of them were diagnosed after a biopsy.[3] The cryptococcoma on MRI spine appeared as hyperintense lesions with edema and a ring enhancement, similar to the appearance in our patient. Most patients developed the lesions in the thoracic or upper lumbar regions. The survival rate for these patients, if the lesion was limited to the spine, was high.[3],[4] The incidence of spinal cryptococcoma with cerebral meningitis is not well documented. However, Stein et al., reported 4 patients with mycotic spinal arachnoiditis and meningitis due to cryptococcal infection. Three of these patients developed acute fungal meningitis postoperatively and 2 out of 3 of them died, which is an outcome similar to our case. None of the patients had cryptococcoma of the spine. Therefore, mortality is higher when cerebral involvement is present.[5]

Treatment in our patient involved surgical intervention for removal of the tumor followed by intravenous administration of antifungal regiment; however, it is worth discussing possible alternative treatment options. As intravenous amphotericin B has poor central nervous system penetration,[6] perhaps a lumbar puncture catheter drainage with an intrathecal injection of amphotericin B might have proven to be effective. In a trial conducted by Yuchong et al., all 14 patients survived with no evidence of a relapse during a 1-year follow-up with intrathecal injection of amphotericin B or with a low-dose systemic therapy.[7] Therefore, this technique should be considered as it appears to be effective and safe.

The role of steroid was considered in this case, due to the presence of mass effect, to help in reducing the swelling caused by the tumor residing in the spinal region, which was causing significant neurological deficit. So far, there is no strong evidence showing the risks and benefits of steroid usage in severe central nervous system cryptococcal disease. However, there are multiple case reports showing therapeutic success and good neurologic outcomes with the use of adjunctive steroids among non-HIV infected individuals with severe central nervous system cryptococcal infection.[8] Nonetheless, more randomized control trials are needed to provide better evidence.

Although rare, a spinal cryptococcoma should be thought of when MRI shows hyperintense lesions with ring enhancement involving the thoracic and upper lumbar region.[4] This diagnosis needs to be entertained in young patients as this disease is potentially treatable. We need to be more vigilant when it comes to diagnosing spinal cryptococcoma. This is to highlight awareness regarding the disease, to help in improving clinical guidelines, and to offer better management for this disease.

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.
Casadevall A, Steenbergen JN, Nosanchuk JD. 'Ready made' virulence and 'dual use' virulence fators in pathogenic environmental fungi-the Cryptococcus neoformans paradigm. Curr Opin Microbiol 2003;6:332-7.  Back to cited text no. 1
    
2.
King JW. Cryptococcosis. In: Chandrasekar PH (editor). Medscape. Drugs and diseases: Infectious diseases. Available from: https://emedicine.medscape.com/article/215354-clinical. [Last accessed on 2018 Jun 17].  Back to cited text no. 2
    
3.
Inada T, Imamura H, Kawamoto M, Sekiya H, Imai Y, Tani S, et al. Cryptococcus neoformans Var. gattii meningoencephalitis with cryptococcoma in an immunocompetent patient successfully treated by surgical resection. No Shinkei Geka 2014;42:123-7.  Back to cited text no. 3
    
4.
Gültaşli NZ, Ercan K, Orhun S, Albayrak S. MRI findings of intramedullary spinal cryptococcoma. Diagn Interv Radiol 2007;13:64-7.  Back to cited text no. 4
    
5.
Stein CS, Corrado ML, Friedlander BS, Farmer P. Chronic mycotic meningitis with spinal involvement (arachnoiditis): A report of five cases. Ann Neurol 1982;11:519-24.  Back to cited text no. 5
    
6.
Schwartz S, Ruhnke M, Ribaud P, Corey L, Driscoll T, Cornely OA, et al. Improved outcome in central nervous system aspergillosis, using voriconazole treatment. Blood 2005;106:2641-5.  Back to cited text no. 6
    
7.
Yuchong C, Jianghan C, Hai W, Julin G. Lumbar puncture drainage with intrathecal injection of amphotericin B for control of cryptococcal meningitis. Mycoses. 2011;54:e248-51.  Back to cited text no. 7
    
8.
Legris T, Massad M, Purgus R, Vache-Coponat H, Ranque S, Girard N, et al. Immune reconstitution inflammatory syndrome mimicking relapsing cryptococcal meningitis in a renal transplant recipient. Transpl Infect Dis 2011;13:303-8.  Back to cited text no. 8
    


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