|Year : 2017 | Volume
| Issue : 1 | Page : 69-71
Primary CNS vasculitis masquerading as glioblastoma: A case report and review
Pelluru Pavan Kumar1, Alugolu Rajesh1, Rukmini Mrudula Kandadai2, Aniruddh Kumar Purohit1, Challa Sundaram3
1 Department of Neurosurgery, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad, Telangana, India
2 Department of Neurology, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad, Telangana, India
3 Department of Pathology, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad, Telangana, India
|Date of Web Publication||17-Mar-2017|
Department of Neurosurgery, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad, Telangana
Source of Support: None, Conflict of Interest: None
Isolated angitis of the central nervous system (IACNS)/primary angitis of central nervous system vasculitis (PACNS) is an uncommon vascular disease, sparingly presenting as an isolated inflammatory lesion on magnetic resonance imaging (MRI). The disease usually manifests as a long-drawn and progressive ischemic event. Delay in diagnosis due to focal nature of the lesion also contributes to the poor prognosis as the dismal natural history and immunosuppressive therapy. To date, only a few cases with tumor-like isolated angitis of CNS have been reported with clear and definitive diagnostic workup.
Keywords: Mimicking glioma, primary angitis, tumor-like lesion, vasculitis
|How to cite this article:|
Kumar PP, Rajesh A, Kandadai RM, Purohit AK, Sundaram C. Primary CNS vasculitis masquerading as glioblastoma: A case report and review. Asian J Neurosurg 2017;12:69-71
|How to cite this URL:|
Kumar PP, Rajesh A, Kandadai RM, Purohit AK, Sundaram C. Primary CNS vasculitis masquerading as glioblastoma: A case report and review. Asian J Neurosurg [serial online] 2017 [cited 2021 Apr 20];12:69-71. Available from: https://www.asianjns.org/text.asp?2017/12/1/69/145187
| Introduction|| |
Isolated angitis of the central nervous system (IACNS)/primary angitis of central nervous system (PACNS) represents a rare and poorly understood form of vascular inflammatory disease restricted to the brain and spinal cord. An average annual incidence rate of 2.4 cases per 1,000,000 persons per year has been reported. Histopathology usually reveals granulomatous inflammation affecting arterioles and small arteries of the parenchyma or leptomeninges. Nonspecific clinical manifestations and various imaging findings often lead to an incorrect or delayed diagnosis and treatment, particularly for an extremely rare form of tumor-like lesion. We herein present a case of IACNS presenting as mass lesion and raised intracranial tension.
| Case Report|| |
A 56-year-old male, known hypertensive and diabetic on regular treatment presented to us with sudden onset of behavioral changes for the past 10 days, which gradually progressed to urinary incontinence and difficulty in walking for about 5 days. There was no history of headache or vomiting. On examination, the patient was conscious; Mini-Mental State Examination (MMSE) was 24/30, with lack of spontaneity of speech with perseveration and spastic gait. All his routine investigations were within normal limits. Magnetic Resonance (MR) Imaging showed a hypointense to iso-intense lesion in the left frontal lobe, involving both gray and white mater on T1W [Figure 1]a, which was hyper-intense on T2W, with disproportionate edema. T1W Gadolinium contrast study showed ring and central enhancement of the lesion. Abnormal enhancement of adjacent vessels and the falx was also noted
|Figure 1: (a) MRI, T1W image showing hypointense lesion in the left frontal lobe. (b and c) showing peripheral enhancement on contrast administration|
Click here to view
[Figure 1]b and [Figure 1]c. Single voxel MR spectroscopy showed increased lipid peak. Multi-voxel spectroscopy showed reduced N-acetylaspartate (NAA) and increased choline peak, with increased choline/creatinine ratio adjacent to lesion, which is suggestive of infiltration [Figure 2]. A provisional diagnosis of glioblastoma was made and patient was taken up for left frontal craniotomy and gross total excision of the lesion was done. Peroperatively, the lesion was grayish, soft to firm with blood clots. No clear gliotic plane was noted. Gross total excision of the lesion was performed.
|Figure 2: MR spectroscopy showing increased choline creatinine ratio and increased choline NAA ratio which is suggestive of glioma|
Click here to view
The biopsy showed brain with overlying meninges. There was perivascular and transmural infiltrate of lymphonuclear cells with focal perivascular granulomas involving small and medium-sized vessels. Infarction and focal haemorrhages in the adjacent brain parenchyma, along with gliosis were observed. The leptomeninges showed mild lymphomononuclear infiltrate. Stains for tuberculous bacilli and fungi were negative [Figure 3].
|Figure 3: The vessels showing perivascular infiltrate of lymphocytes and neutrophils causing wall destruction. H and E, ×100|
Click here to view
MR angiography done postoperatively showed focal narrowing of the vessels in the left frontal region, suggestive of vasculitis [Figure 4]. MR venogram was normal. Systemic evaluation for secondary vasculitis with erythrocyte sedimentation rate (ESR), cANCA, pANCA, retroviral assays, fungal and bacterial blood cultures were negative. Bilateral carotid Doppler showed no significant hemodynamic abnormality.
|Figure 4: MR angiography in the postoperative period shows focal narrowing of vessels in the left frontal region, indicating vasculitis|
Click here to view
Monotherapy with steroids was initiated immediately after establishing the diagnosis with intravenous prednisolone 1000 mg/day for 3 days, 500 mg/day for 3 days, 250 mg/day for 3 days, 125 mg/day for 3 days, followed by oral prednisolone 60 mg/day. Six weeks after surgery, the patient's neurological symptoms gradually improved and there was no relapse of symptoms at 18 months follow-up.
| Discussion|| |
IACNS/PACNS is a rare form of angitis limited to the central nervous system. PACNS currently is a diagnosis of exclusion, due to nonspecific symptoms and signs. There are no readily available non-invasive diagnostic tests.
Calabrese and Mallek  proposed diagnostic criteria for IACNS, which include the following:
- Unexplained neurologic deficit after thorough clinical and laboratory evaluation;
- arteritic process demonstrated by cerebral angiogram and/or histopathologic examination in CNS; and
- absent evidence of systemic vasculitis.
Clinical onset of IACNS is usually subacute, but it can have acute onset with rapid progression within a few days or weeks. The most frequent clinical manifestations at presentation are headache, altered cognition, hemiparesis, or persistent neurological deficit or stroke. First presentation as a subarachnoid hemorrhage has been reported too. Less common complaints are aphasia, transient ischemic attack, ataxia, dysphasia, nausea or vomiting, loss of memory, seizure, or a psychiatric disorder. Duna et al. have described the occurrence of mass-like presentation of PACNS in about 15% of their cases (25 of 168) with predominantly focal symptoms (84%), followed by headaches (64%), altered sensorium, and cognitive decline (56%).
MRI findings are highly variable and nonspecific, ranging from multiple irregular white matter changes to intracerebral haemorrhages, with sensitivity ranging from 50% to 100% and with a 100% specificity in biopsy-proven cases. You et al. had described ill-defined lesion with disproportionate edema, striped hemorrhages, along with abnormal enhancement of adjacent vessel. Panchal et al. described marked elevation of glutamine and glutamate peaks as a marker of inflammatory pathology in the CNS on MR spectroscopy (MRS), along with markedly elevated lipid and N-Acetyl- Aspartate (NAA) peaks and absent lactate peaks, differentiating them from aggressive neoplasms. Their reported case had normal choline/creatinine ratio, indicating against a neoplastic etiology. Xiao et al. reported elevated choline/creatinine ratio and reduced NAA peaks in their case similar to the present case, indicating towards a neoplastic etiology. There was a peak noted at around 2 ppm, which could be attributed to glutamine/glutamate, which is more commonly seen in inflammatory pathologies than aggressive neoplastic lesions. Role of diffusion-weighted MR imaging has been described for detection of lesions, which shows hyperintense lesions with heterogeneous average diffusion coefficient (ADC) values suggestive of coexistence of cytotoxic and vasogenic edema.,
Cerebral angiography, once considered as a gold standard, is not without pitfalls, with a sensitivity between 27% and 90% and a false-negativity rate of 30-40%., 3, ,, Brain and meningeal biopsy, though gold standard for the diagnosis, is positive only in 66-75% cases due to focality of the disease. The preferred site for the brain and meningeal biopsy is the temporal lobe of non-dominant side in areas with longitudinally arranged surface vessels.
Treatment regimens for cerebral vasculitis include induction with high-dose steroids (methylprednisolone), coupled with or without cyclophosphamide. Maintenance therapy includes low-dose steroids/pulsed cyclophosphamide/methotrexate. In the present case, we were able to achieve and maintain remission with steroids alone.
| Conclusion|| |
Isolated central nervous system vasculitis still poses a challenge for diagnosis and treatment. MRS showing a glutamine peak seems to be a promising sign requiring further evaluation.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Salvarani C, Brown RD Jr, Calamia KT, Christianson TJ, Weigand SD, Miller DV, et al
. Primary central nervous system vasculitis: Analysis of 101 patients. Ann Neurol 2007;62:442-51.
MacLaren K, Gillespie J, Shrestha S, Neary D, Ballardie FW. Primary angitis of the central nervous system: Emerging variants. Q J Med 2005;98:643-54.
Calabrese LH, Mallek JA. Primary angitis of the central nervous system. Report of 8 new cases, review of the literature, and proposal for diagnostic criteria. Medicine 1988;67:20-39.
Kumar R, Wijdicks EF, Brown RD Jr, Parisi JE, Hammond CA. Isolated angitis of the CNS presenting as subarachnoid haemorrhage. J Neurol Neurosurg Psychiatry 1997;62:649-51.
Hocaoglu C, Tan M. Isolated angiitis of the central nervous system: A case presented with atypical psychiatric symptoms. Prog Neuropsychopharmacol Biol Psychiatry 2005;29:627-31.
Duna GF, George T, Rybicki L, Calabrese LH. Primary angiitis of the central nervous system; an analysis of unusual presentation. Arthritis Rheum 1995;38:S340-S1122.
Siva A. Vasculitis of the nervous system. J Neurol 2001;248:451-68.
You G, Yan W, Zhang W, Shaowu L, Guilin L, Jiang T. Isolated angiitis of central nervous system with tumor-like lesion, mimicking brain malignant glioma: A case report and review of the literature. World J Surg Oncol 2011;9:97.
Panchal NJ, Niku S, Imbesi SG. Lymphocytic vasculitis mimicking aggressive multifocal cerebral neoplasm: MR imaging and MR spectroscopic appearance. AJNR Am J Neuroradiol 2005:26;642-5.
Xiao-lin YU, Ai-fen LIU, Lin MA, Chun-zhu YAN, Yu-ying ZHAO, Pei-yan SHAN. Primary angiitis of the central nervous system: A case report. Chin Med J 2011;124: 2782-5.
Moritani T, Hiwatashi A, ShrierDA, Wang HZ, NumaguchiY, Westesson PL. CNS vasculitis and vasculopathy: Efficacy and usefulness of diffusion-weighted echo planar MR imaging. Clin Imaging 2004:28;261-70.
Lee SY, Chu K, Park KI, Jeong SW, Yoon BW. Diffusion- weighted MR findings in isolated angiitis of the central nervous system (IACNS). Acta Neurol Scand 2003;108:346-51.
Duna G, Calabrese L. Limitations in the diagnostic modalities in the diagnosis of primary angitis of the central nervous system (PACNS). J Rheumatol 1995; 22:662-669.
Chu CT, Gray L, Goldstein LB, Hulette CM. Diagnosis of intracranial vasculitis: A multi-disciplinary approach. J Neuropathol Exp Neurol 1998; 57:30-38.
Alrawi A, Trobe JD, Blaivas M, Musch DC. Brain biopsy in primary angitis of the central nervous system. Neurology 1999; 53: 858-60.
Calabrese LH, Duna GF, Lie JT. Vasculitis in the central nervous system. Arthritis Rheum 1997;40:1189-1201.
Moore PM. Diagnosis and management of isolated angitis of the central nervous system. Neurology 1989; 39:167-73.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
|This article has been cited by|
||Central Nervous System Systemic Lupus Erythematosus (CNS-SLE) Vasculitis Mimicking Lewy Body Dementia: A Case Report Emphasizing the Role of Imaging With an Analysis of 33 Comparable Cases From the Scientific Literature
| ||Peter Abraham,Ian Neel,Steven Bishay,Daniel D. Sewell |
| ||Journal of Geriatric Psychiatry and Neurology. 2021; 34(2): 128 |
|[Pubmed] | [DOI]|
||“When should primary angiitis of the central nervous system (PACNS) be suspected?”: literature review and proposal of a preliminary screening algorithm
| ||Sarti Cristina,Picchioni Antonella,Telese Roberta,Pasi Marco,Failli Ylenia,Pracucci Giovanni,Cammelli Daniele,Inzitari Domenico |
| ||Neurological Sciences. 2020; |
|[Pubmed] | [DOI]|
||Tumefactive Primary Central Nervous System Vasculitis: Imaging Findings of a Rare and Underrecognized Neuroinflammatory Disease
| ||S. Suthiphosuwan,A. Bharatha,C.C.-T. Hsu,A.W. Lin,J.A. Maloney,D.G. Munoz,C.A. Palmer,A.G. Osborn |
| ||American Journal of Neuroradiology. 2020; |
|[Pubmed] | [DOI]|