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CASE REPORT  
Year : 2014  |  Volume : 57  |  Issue : 2  |  Page : 332-334
Central nervous system norcardiosis with granulomatous pachymeningitis and osteomyelitis of skull vault


1 Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India
2 Department of Neuroimaging and Interventional Radiology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India
3 Department of Neuropathology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India

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Date of Web Publication19-Jun-2014
 

   Abstract 

A 34-year-old immunocompetent man weighing 95 kg was operated for a small left parietal scalp swelling in the year 2002. He was well until 2008, when he developed chronic diffuse headache, vomiting and drowsiness. The left parietal dura and overlying vault biopsy showed evidence of granulomatous pachymeningitis with osteomyelitis secondary to nocardiosis. He had responded well to inadequate antibiotic therapy. After a dormant period of 3 years, there was recrudescence of severe raised intracranial tension symptoms in 2011. Magnetic resonance imaging showed diffuse pachymeningeal thickening mainly involving the occipital dura, posterior falx, and tentorium cerebelli. In addition, well-defined small nodules with hypointense signals on both T1- and T2-weighted images were seen in occipital lobes. Patient was treated with three drug regime with good recovery at 3 months follow-up. This is a rare case of central nervous system nocardiosis with skull vault osteomyelitis and a protracted clinical course.

Keywords: Cerebral norcardiosis, magnetic resonance imaging, osteomyelitis, pachymeningitis

How to cite this article:
Nalini A, Saini J, Mahadevan A. Central nervous system norcardiosis with granulomatous pachymeningitis and osteomyelitis of skull vault. Indian J Pathol Microbiol 2014;57:332-4

How to cite this URL:
Nalini A, Saini J, Mahadevan A. Central nervous system norcardiosis with granulomatous pachymeningitis and osteomyelitis of skull vault. Indian J Pathol Microbiol [serial online] 2014 [cited 2022 Jan 20];57:332-4. Available from: https://www.ijpmonline.org/text.asp?2014/57/2/332/134735



   Introduction Top


Nocardia spp. infections of brain are extremely rare. [1] Factors leading to a good outcome are: early diagnosis, institution of appropriate antimicrobial therapy, lack of underlying systemic disease, and limited disease. [2] Most of the reported cases are in immunocompromised individuals, however, reports of infection in immmunocompetent individuals also exist. [3] Usual clinical course described is an acute or subacute illness. We present the unusually protracted clinical course of Nocardia asteroides in an immunocompetent patient with brain infection and skull vault osteomyelitis.


   Case report Top


A 34-year-old man, weighing 95 kg, had a history of a small left parietal scalp swelling in the year 2002. This was operated without any complications, and patient was asymptomatic until 2008, when he developed chronic diffuse headache, vomiting, and drowsiness. A left parietal dura and overlying vault biopsy revealed granulomatous pachymeningitis with osteomyelitis secondary to nocardiosis. Patient had received oral medications for 5 months and symptoms of raised intracranial pressure had subsided. No records are available regarding the treatment received. In June 2011, patient was evaluated at our institute, with 5 months history of right motor seizures with secondary generalization, progressively increasing intolerable headache, blurred vision, recurrent vomiting, mild unsteadiness of gait and numbness over left half of the face and scalp for 3 months. Examination revealed a conscious patient with papilledema. No other neurological deficits. Blood biochemistry was normal. HIV serology was nonreactive. Lumbar puncture was not performed as magnetic resonance imaging (MRI) showed evidence of coning. Patient was treated as a case of serious nocardial infection with three drug regime of intravenous carbapenem (Imipenem) at 2 g/day for 8 weeks, trimethoprim/sulfamethoxazole at 15 mg/kg/day for 8 weeks, followed by oral trimethoprim medication and intravenous amikacin of 750 mg/day for 8 weeks, followed by same dos for a total period of 12 months. At 3 months follow-up, patient had good recovery with no neurological symptoms and had returned for work. MRI showed diffuse pachymeningeal thickening mainly involving occipital dura, posterior falx, and tentorium cerebelli. Well defined small nodules with hypointense signals on both T1- and T2-weighted (T1-W and T2-W) images and significant edema were seen in the occipital lobes [Figure 1]a and b]. Thickened dura and lesions in occipital lobes showed evidence of susceptibility on gradient images [Figure 1]c]. Diffusion imaging revealed no evidence of diffusion restriction. Contrast (Gd-DTPA) revealed strong pachymeningeal enhancement involving posterior falx, tentorium cerebelli and peripherally enhancing parenchymal lesions in occipital lobes [Figure 1]d]. X-ray chest was normal. Ultrasound of the abdomen did not reveal any abnormalities.
Figure 1: (a) Axial T1-weighted (T1-W) image-grossly thickened occipital bone and overlying scalp tissue. Occipital parenchyma shows hypointense signal intensity. (b) Axial T2-W images show thickened hypointense occipital bone, scalp soft tissue, underlying thickened dura. Bilateral occipital parenchyma shows abundant edema and hypointense lesions abutting the dura. (c) Gradient images reveal mild susceptibility in parenchymal and occipital dura. (d) Post contrast axial T1-W reveals marked enhancement of dura, occipital bones and scalp soft tissue. Parenchymal lesions also show thick peripheral enhancement

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Histology revealed dense chronic inflammatory cell collections comprising lymphocytes and plump histiocytes expanding the dura [Figure 2]a], infiltrating the galea aponeurotica as well as filling the marrow spaces destroying skull bone [Figure 2]c]. Within the inflammatory centre, bluish colonies were seen surrounded by multinucleate giant cells [Figure 2]b], which revealed tightly packed slender branching hyphae highlighted by periodic acid-Schiff [Figure 2]c and d] and methenamine silver stains [Figure 2]e] morphologically resembling Nocardia spp. Gram's stain was variable and modified acid fast stain revealed organisms to be weakly acid fast [Figure 2]c, inset], features highly suggestive of Nocardia spp.
Figure 2: The dura revealed dense pachymeningitis (a) with bluish colonies enclosed within the exudate (a, arrow) surrounded by multinucleate giant cells. (b) Similar colonies are seen within marrow spaces of skull bone (c) eliciting osteomyelitis. The colonies are intensely periodic acid– Schiff (PAS) positive (c and d) and methenamine silver highlights slender, branching thin filaments (d) that are weakly acid fast (c, inset) morphologically resembling Nocardia spp. (a: H and E, ×Obj.10, b: H and E, ×Obj.20, c: PAS, ×Obj.5, c, inset: 1% AFB ×Obj.40, d: PAS ×Obj.40, e: Methenamine silver ×Obj.100)

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   Discussion Top


0Nocardia is a Gram-positive actinomycete found in the environment. It causes a wide spectrum of diseases in humans depending on immune status of the host. Nocardiosis is chiefly an opportunistic infection either localized or disseminated. [1] Nocardia asteroides accounts for 80% of cases of noncutaneous invasive disease and most cases of systemic or central nervous system (CNS) disease. Primary infection is the most commonly acquired through respiratory tract. Subsequent hematogenous spread may lead to involvement of CNS and other tissues. CNS nocardiosis may present as meningitis, diffuse cerebral infiltration without localized lesions, granulomata with giant cells, or cerebral abscesses. Although they usually present as a single lesion, approximately one-third of those affected have multiple brain lesions. [1]

CNS infection can run its course without fever or leukocytosis, which hinders clinical suspicion of the infection. Brain abscesses and occasionally meningitis typically occurs after dissemination. In immunocompetent individuals, direct skin inoculation can result in skin and soft-tissue infections. Presentations of this form of nocardiosis include mycetoma, sporotrichoid infections and occasionally osteomyelitis. [1]

Our patient had presented with severe form of CNS involvement with pachymeningitis, granulomatous lesions and skull vault osteomyelitis and a long asymptomatic period even without optimal antibiotic treatment. Computed tomography findings in cases of nocardial brain infection are most commonly single or multiple ring-enhancing lesions with surrounding perilesional edema. [4] MRI also reveal multiple conglomerated ring-enhancing lesions with edema. [5] In our case, MRI showed peripherally enhancing lesions with marked surrounding edema and lesions appeared hypointense on T2-W and gradient MRI. There was no central necrosis or abscess formation. Diffusion weighted imaging also did not reveal restricted diffusion. These findings closely mimic tuberculomas and fungal granuloma on MRI. In addition, presence of hypertrophic pachymeningitis and osteomyelitis on imaging along with a prolonged clinical course supports the diagnosis of tuberculosis which is a highly prevalent infection in our part of the world. Nocardia infection has usually been associated with immunocompromised states such as underlying malignancy, immunosuppressive therapy, alcoholism, pulmonary disease, and diabetes. However in our case, no underlying predisposing factor could be identified. Possible portal of entry could be through skin as patient has a scalp wound. Although common predisposing factors for Nocardia infection were not present, other rare causes of impaired immunity could not be ruled out. In a large review of 243 patients of Nocardia infection no case of osteomyelitis was observed. [6] Osteomyelitis is a rare manifestation of Nocardia infection and a few case reports of Nocardia osteomyelitis have been published in the literature. Bone infection is either a result of direct spread of disease from the overlying soft tissue infection or is due to hematogenous spread of the organism. In our case, skull vault osteomyelitis and underlying meningeal and brain infection may be secondary to overlying soft tissue infection.

Other interesting facet of our case was the protracted course of illness. Usually Nocardia infection is considered to have high mortality and morbidity. However, our case highlights the possibility of long disease duration in an immunocompetent patient and in our patient, the response was highly satisfactory.

 
   References Top

1.Ambrosioni J, Lew D, Garbino J. Nocardiosis: Updated clinical review and experience at a tertiary center. Infection 2010;38:89-97.  Back to cited text no. 1
    
2.Byrne E, Brophy BP, Perrett LV. Nocardia cerebral abscess: New concepts in diagnosis, management, and prognosis. J Neurol Neurosurg Psychiatry 1979;42:1038-45.  Back to cited text no. 2
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3.Bross JE, Gordon G. Nocardial meningitis: Case reports and review. Rev Infect Dis 1991;13:160-5.  Back to cited text no. 3
    
4.Whelan MA, Stern J, deNapoli RA. The computed tomographic spectrum of intracranial mycosis: Correlation with histopathology. Radiology 1981;141:703-7.  Back to cited text no. 4
[PUBMED]    
5.LeBlang SD, Whiteman ML, Post MJ, Uttamchandani RB, Bell MD, Smirniotopolous JG. CNS Nocardia in AIDS patients: CT and MRI with pathologic correlation. J Comput Assist Tomogr 1995;19:15-22.  Back to cited text no. 5
    
6.Palmer DL, Harvey RL, Wheeler JK. Diagnostic and therapeutic considerations in Nocardia asteroides infection. Medicine (Baltimore) 1974;53:391-401.  Back to cited text no. 6
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Correspondence Address:
Atchayaram Nalini
Departments of Neurology, National Institute of Mental Health and Neurosciences, Neuroscience Faculty Block, Hosur Road, Bengaluru - 560 029, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.134735

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    Figures

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