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Table of Contents    
LETTERS TO EDITOR
Year : 2019  |  Volume : 67  |  Issue : 5  |  Page : 1374-1375

Primary Angiitis of the Central Nervous System Presenting with Microhemorrhages on Gradient Echo Imaging


1 Department of Neurology and Rehabilitation Medicine, University of Cincinnati, Cincinnati, Ohio, USA
2 Department of Neurology, University of Kentucky, Lexington, Kentucky, USA
3 Department of Clinical Genomics, Mayo Clinic, Phoenix, Arizona, USA
4 Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA

Date of Web Publication19-Nov-2019

Correspondence Address:
Dr. Brian D Moseley
Department of Neurology and Rehabilitation Medicine, University of Cincinnati, Cincinnati, Ohio
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.271267

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How to cite this article:
Moseley BD, Smith JH, Dhamija RR, Jones Jr LK. Primary Angiitis of the Central Nervous System Presenting with Microhemorrhages on Gradient Echo Imaging. Neurol India 2019;67:1374-5

How to cite this URL:
Moseley BD, Smith JH, Dhamija RR, Jones Jr LK. Primary Angiitis of the Central Nervous System Presenting with Microhemorrhages on Gradient Echo Imaging. Neurol India [serial online] 2019 [cited 2019 Dec 8];67:1374-5. Available from: http://www.neurologyindia.com/text.asp?2019/67/5/1374/271267


Sir,

We present a case which demonstrates that the earliest imaging finding suggestive of primary angiitis of the central nervous system (PACNS) may be subcortical microhemorrhages on gradient echo (GRE) imaging.

A 52-year-old gentleman presented with episodic headaches, rotatory vertigo, blurred vision, and semantic paraphasia. Magnetic resonance imaging (MRI) of the brain with gadolinium revealed confluent T2 signal hyperintensities in the right frontotemporal and left temporal subcortical white matter and a subacute-chronic left cerebellar hemorrhage [Figure 1]a, [Figure 1]b, [Figure 1]c. GRE revealed punctate foci of decreased signal throughout the right frontotemporal and left temporal regions in addition to the left cerebellar hemorrhage [Figure 1]d and [Figure 1]e. MR angiography was normal.
Figure 1: MRI findings in a patient with PACNS. (a) Axial MRI (FLAIR) demonstrating abnormal T2 signal hyperintensity primarily in the right frontotemporal region. (b) Axial MRI (T2) demonstrating abnormal signal hyperintensity in the left cerebellum, compatible with a subacute hemorrhage. (c) Axial MRI (T1 with contrast) demonstrating small punctuate and linear zones of contrast enhancement corresponding to the areas of right frontotemporal T2 signal hyperintensity. (d) Axial MRI (gradient echo sequencing) demonstrating punctate foci of decreased signal in the right frontotemporal and left temporal regions consistent with hemorrhages. (e) Axial MRI (gradient echo sequencing) demonstrating subacute hemorrhage in the left cerebellum

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Serologic studies (complete blood count, electrolytes, sedimentation rate, lactate dehydrogenase, angiotensin -converting enzyme, myeloperoxidase, proteinase 3, antiphospholipid, and paraneoplastic antibodies) were normal. Cerebrospinal fluid (CSF) examination revealed a normal glucose, protein, and total nucleated cell count. CSF cytology and flow cytometry revealed polyclonal lymphocytosis but no malignancy. Varicella and other herpes virus polymerase chain reactions from CSF were negative. Four vessel cerebral angiogram was normal.

The patient underwent biopsy of the right frontal lobe signal abnormality. Immunohistochemical staining revealed small lymphocytic collections of perivascular, predominantly CD3 positive T-lymphocytes with focal granulomatous features involving the white matter [Figure 2]a, [Figure 2]b, [Figure 2]cxs. A beta-amyloid stain was negative [Figure 2]d.
Figure 2: Histologic findings in a patient with PACNS. (a) Right frontal brain biopsy demonstrating chronic perivascular lymphocytic inflammation. (b) with focal granulomatous features involving the white matter, (c) positive CD3 staining, (d) and negative beta amyloid staining, consistent with PACNS

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Given the pathologic findings, the diagnosis of PACNS was established. The patient was treated with five days of intravenous methylprednisolone (1 g daily), followed by an oral prednisone taper. The patient reported no further episodes of vertigo, blurred vision, or dysphasia.

In our case, the earliest imaging findings most suggestive of PACNS were subcortical microhemorrhages on GRE. Intracerebral hemorrhage is known to occur in PACNS.[1] Cortical and subcortical signal hypointensities on GRE consistent with petechial hemorrhages help to distinguish PACNS radiographically from many of its mimickers. This is particularly true for cerebral lymphoma, where GRE hypointensities have only been reported in the setting of tumor hemorrhage.[2] Although GRE findings of microhemorrhage can be common and observed in amyloid angiopathy, such hemorrhages are typically larger than those seen in PACNS.[3]

At present, biopsy is the gold standard for the diagnosis of PACNS. However, the potential use of GRE to distinguish PACNS earlier from its mimickers is important, as timely initiation of corticosteroids with or without cyclophosphamide is needed to prevent significant morbidity and mortality. Other steroid-sparing agents such as azathioprine, methotrexate, and mycophenolate mofetil may be useful for chronic treatment.[3]

Acknowledgements

The authors would like to acknowledge Dr. Caterina Giannini for her assistance in photographing and interpreting the histologic findings in this case. The authors have no additional acknowledgements.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Calabrese LH, Furlan AJ, Gragg LA, Ropos TJ. Primary angiitis of the central nervous system: Diagnostic criteria and clinical approach. Cleve Clin J Med 1992;59:293-306.  Back to cited text no. 1
    
2.
Erdag N, Bhorade RM, Alberico RA, Yousuf N, Patel MR. Primary lymphoma of the central nervous system: Typical and atypical CT and MR imaging appearances. AJR Am J Roentgenol 2001;176:1319-26.  Back to cited text no. 2
    
3.
Arias M, Osorio X, Dapena D, Arias-Rivas S, Vázquez F. Recurrent leukoencephalopathy with microhemorrhages: Gradient-echo MRI study diagnostic value in CNS primary angiitis. Mult Scler 2008;14:1139-41.  Back to cited text no. 3
    


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